A syndrome complex composed of three conditions which represent clinical variants of the same disease process: STRIATONIGRAL DEGENERATION; SHY-DRAGER SYNDROME; and the sporadic form of OLIVOPONTOCEREBELLAR ATROPHIES. Clinical features include autonomic, cerebellar, and basal ganglia dysfunction. Pathologic examination reveals atrophy of the basal ganglia, cerebellum, pons, and medulla, with prominent loss of autonomic neurons in the brain stem and spinal cord. (From Adams et al., Principles of Neurology, 6th ed, p1076; Baillieres Clin Neurol 1997 Apr;6(1):187-204; Med Clin North Am 1999 Mar;83(2):381-92)
A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS, PATHOLOGIC; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085)
A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord. This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with ORTHOSTASIS and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p536)
A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7)
A synuclein that is a major component of LEWY BODIES that plays a role in neurodegeneration and neuroprotection.
Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.
Diseases of the parasympathetic or sympathetic divisions of the AUTONOMIC NERVOUS SYSTEM; which has components located in the CENTRAL NERVOUS SYSTEM and PERIPHERAL NERVOUS SYSTEM. Autonomic dysfunction may be associated with HYPOTHALAMIC DISEASES; BRAIN STEM disorders; SPINAL CORD DISEASES; and PERIPHERAL NERVOUS SYSTEM DISEASES. Manifestations include impairments of vegetative functions including the maintenance of BLOOD PRESSURE; HEART RATE; pupil function; SWEATING; REPRODUCTIVE AND URINARY PHYSIOLOGY; and DIGESTION.
A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75)
The largest and most lateral of the BASAL GANGLIA lying between the lateral medullary lamina of the GLOBUS PALLIDUS and the EXTERNAL CAPSULE. It is part of the neostriatum and forms part of the LENTIFORM NUCLEUS along with the GLOBUS PALLIDUS.
A family of homologous proteins of low MOLECULAR WEIGHT that are predominately expressed in the BRAIN and that have been implicated in a variety of human diseases. They were originally isolated from CHOLINERGIC FIBERS of TORPEDO.
A significant drop in BLOOD PRESSURE after assuming a standing position. Orthostatic hypotension is a finding, and defined as a 20-mm Hg decrease in systolic pressure or a 10-mm Hg decrease in diastolic pressure 3 minutes after the person has risen from supine to standing. Symptoms generally include DIZZINESS, blurred vision, and SYNCOPE.
Incoordination of voluntary movements that occur as a manifestation of CEREBELLAR DISEASES. Characteristic features include a tendency for limb movements to overshoot or undershoot a target (dysmetria), a tremor that occurs during attempted movements (intention TREMOR), impaired force and rhythm of diadochokinesis (rapidly alternating movements), and GAIT ATAXIA. (From Adams et al., Principles of Neurology, 6th ed, p90)
A generic term for any circumscribed mass of foreign (e.g., lead or viruses) or metabolically inactive materials (e.g., ceroid or MALLORY BODIES), within the cytoplasm or nucleus of a cell. Inclusion bodies are in cells infected with certain filtrable viruses, observed especially in nerve, epithelial, or endothelial cells. (Stedman, 25th ed)
A neurodegenerative disease characterized by dementia, mild parkinsonism, and fluctuations in attention and alertness. The neuropsychiatric manifestations tend to precede the onset of bradykinesia, MUSCLE RIGIDITY, and other extrapyramidal signs. DELUSIONS and visual HALLUCINATIONS are relatively frequent in this condition. Histologic examination reveals LEWY BODIES in the CEREBRAL CORTEX and BRAIN STEM. SENILE PLAQUES and other pathologic features characteristic of ALZHEIMER DISEASE may also be present. (From Neurology 1997;48:376-380; Neurology 1996;47:1113-1124)
An ethanolamine derivative that is an adrenergic alpha-1 agonist. It is used as a vasoconstrictor agent in the treatment of HYPOTENSION.
Antimicrobial peptides of 45-47 amino acids and typically with four disulfide bridges. They are found in PLANTS. Type-V thionins lack the C-terminal nonapeptide. This should not be confused with thionine.
Methods and procedures for the diagnosis of diseases of the nervous system, central and peripheral, or demonstration of neurologic function or dysfunction.
A sporadic neurodegenerative disease with onset in middle-age characterized clinically by Parkinsonian features (e.g., MUSCLE RIGIDITY; HYPOKINESIA; stooped posture) and HYPOTENSION. This condition is considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Pathologic features include a prominent loss of neurons in the zona compacta of the SUBSTANTIA NIGRA and PUTAMEN. (From Adams et al., Principles of Neurology, 6th ed, p1075-6)
A group of disorders which feature impaired motor control characterized by bradykinesia, MUSCLE RIGIDITY; TREMOR; and postural instability. Parkinsonian diseases are generally divided into primary parkinsonism (see PARKINSON DISEASE), secondary parkinsonism (see PARKINSON DISEASE, SECONDARY) and inherited forms. These conditions are associated with dysfunction of dopaminergic or closely related motor integration neuronal pathways in the BASAL GANGLIA.
Conditions which feature clinical manifestations resembling primary Parkinson disease that are caused by a known or suspected condition. Examples include parkinsonism caused by vascular injury, drugs, trauma, toxin exposure, neoplasms, infections and degenerative or hereditary conditions. Clinical features may include bradykinesia, rigidity, parkinsonian gait, and masked facies. In general, tremor is less prominent in secondary parkinsonism than in the primary form. (From Joynt, Clinical Neurology, 1998, Ch38, pp39-42)
A degenerative disease of the AUTONOMIC NERVOUS SYSTEM that is characterized by idiopathic ORTHOSTATIC HYPOTENSION and a greatly reduced level of CATECHOLAMINES. No other neurological deficits are present.
Congenital or acquired paralysis of one or both VOCAL CORDS. This condition is caused by defects in the CENTRAL NERVOUS SYSTEM, the VAGUS NERVE and branches of LARYNGEAL NERVES. Common symptoms are VOICE DISORDERS including HOARSENESS or APHONIA.
Intracytoplasmic, eosinophilic, round to elongated inclusions found in vacuoles of injured or fragmented neurons. The presence of Lewy bodies is the histological marker of the degenerative changes in LEWY BODY DISEASE and PARKINSON DISEASE but they may be seen in other neurological conditions. They are typically found in the substantia nigra and locus coeruleus but they are also seen in the basal forebrain, hypothalamic nuclei, and neocortex.
A pupillary abnormality characterized by a poor pupillary light reaction, reduced accommodation, iris sector palsies, an enhanced pupillary response to near effort that results in a prolonged, "tonic" constriction, and slow pupillary redilation. This condition is associated with injury to the postganglionic parasympathetic innervation to the pupil. (From Miller et al., Clinical Neuro-Ophthalmology, 4th ed, pp492-500)
A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. Sporadic and inherited subtypes occur. Inheritance patterns include autosomal dominant, autosomal recessive, and X-linked.
A class of large neuroglial (macroglial) cells in the central nervous system. Oligodendroglia may be called interfascicular, perivascular, or perineuronal (not the same as SATELLITE CELLS, PERINEURONAL of GANGLIA) according to their location. They form the insulating MYELIN SHEATH of axons in the central nervous system.
Uncrossed tracts of motor nerves from the brain to the anterior horns of the spinal cord, involved in reflexes, locomotion, complex movements, and postural control.
Impairment of the ability to coordinate the movements required for normal ambulation (WALKING) which may result from impairments of motor function or sensory feedback. This condition may be associated with BRAIN DISEASES (including CEREBELLAR DISEASES and BASAL GANGLIA DISEASES); SPINAL CORD DISEASES; or PERIPHERAL NERVOUS SYSTEM DISEASES.
Branches of the vagus (tenth cranial) nerve. The recurrent laryngeal nerves originate more caudally than the superior laryngeal nerves and follow different paths on the right and left sides. They carry efferents to all muscles of the larynx except the cricothyroid and carry sensory and autonomic fibers to the laryngeal, pharyngeal, tracheal, and cardiac regions.
The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.
Diseases of the SWEAT GLANDS.
Derangement in size and number of muscle fibers occurring with aging, reduction in blood supply, or following immobilization, prolonged weightlessness, malnutrition, and particularly in denervation.
A disorder characterized by episodes of vigorous and often violent motor activity during REM sleep (SLEEP, REM). The affected individual may inflict self injury or harm others, and is difficult to awaken from this condition. Episodes are usually followed by a vivid recollection of a dream that is consistent with the aggressive behavior. This condition primarily affects adult males. (From Adams et al., Principles of Neurology, 6th ed, p393)
The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA.
Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures.
The naturally occurring form of DIHYDROXYPHENYLALANINE and the immediate precursor of DOPAMINE. Unlike dopamine itself, it can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to DOPAMINE. It is used for the treatment of PARKINSONIAN DISORDERS and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system.
Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.
The ENTERIC NERVOUS SYSTEM; PARASYMPATHETIC NERVOUS SYSTEM; and SYMPATHETIC NERVOUS SYSTEM taken together. Generally speaking, the autonomic nervous system regulates the internal environment during both peaceful activity and physical or emotional stress. Autonomic activity is controlled and integrated by the CENTRAL NERVOUS SYSTEM, especially the HYPOTHALAMUS and the SOLITARY NUCLEUS, which receive information relayed from VISCERAL AFFERENTS.
Examination, therapy or surgery of the interior of the larynx performed with a specially designed endoscope.
Large subcortical nuclear masses derived from the telencephalon and located in the basal regions of the cerebral hemispheres.
A phenethylamine found in EPHEDRA SINICA. PSEUDOEPHEDRINE is an isomer. It is an alpha- and beta-adrenergic agonist that may also enhance release of norepinephrine. It has been used for asthma, heart failure, rhinitis, and urinary incontinence, and for its central nervous system stimulatory effects in the treatment of narcolepsy and depression. It has become less extensively used with the advent of more selective agonists.
Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.
The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.
A beta-hydroxylated derivative of phenylalanine. The D-form of dihydroxyphenylalanine has less physiologic activity than the L-form and is commonly used experimentally to determine whether the pharmacological effects of LEVODOPA are stereospecific.
Agents used in the treatment of Parkinson's disease. The most commonly used drugs act on the dopaminergic system in the striatum and basal ganglia or are centrally acting muscarinic antagonists.
Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or PERIPHERAL NERVE DISEASES. Motor ataxia may be associated with CEREBELLAR DISEASES; CEREBRAL CORTEX diseases; THALAMIC DISEASES; BASAL GANGLIA DISEASES; injury to the RED NUCLEUS; and other conditions.
The body region lying between the genital area and the ANUS on the surface of the trunk, and to the shallow compartment lying deep to this area that is inferior to the PELVIC DIAPHRAGM. The surface area is between the VULVA and the anus in the female, and between the SCROTUM and the anus in the male.
'Nerve tissue proteins' are specialized proteins found within the nervous system's biological tissue, including neurofilaments, neuronal cytoskeletal proteins, and neural cell adhesion molecules, which facilitate structural support, intracellular communication, and synaptic connectivity essential for proper neurological function.
The front part of the hindbrain (RHOMBENCEPHALON) that lies between the MEDULLA and the midbrain (MESENCEPHALON) ventral to the cerebellum. It is composed of two parts, the dorsal and the ventral. The pons serves as a relay station for neural pathways between the CEREBELLUM to the CEREBRUM.
The mechanical laws of fluid dynamics as they apply to urine transport.
Recording of the changes in electric potential of muscle by means of surface or needle electrodes.
Amides of salicylic acid.
Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system.
Elongated gray mass of the neostriatum located adjacent to the lateral ventricle of the brain.
The black substance in the ventral midbrain or the nucleus of cells containing the black substance. These cells produce DOPAMINE, an important neurotransmitter in regulation of the sensorimotor system and mood. The dark colored MELANIN is a by-product of dopamine synthesis.
The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions.

EMG responses to free fall in elderly subjects and akinetic rigid patients. (1/215)

OBJECTIVES: The EMG startle response to free fall was studied in young and old normal subjects, patients with absent vestibular function, and patients with akinetic-rigid syndromes. The aim was to detect any derangement in this early phase of the "landing response" in patient groups with a tendency to fall. In normal subjects the characteristics of a voluntary muscle contraction (tibialis anterior) was also compared when evoked by a non-startling sound and by the free fall startle. METHODS: Subjects lay supine on a couch which was unexpectedly released into free fall. Latencies of multiple surface EMG recordings to the onset of free fall, detected by a head mounted linear accelerometer, were measured. RESULTS AND CONCLUSIONS: (1) EMG responses in younger normal subjects occurred at: sternomastoid 54 ms, abdominals 69 ms, quadriceps 78 ms, deltoid 80 ms, and tibialis anterior 85 ms. This pattern of muscle activation, which is not a simple rostrocaudal progression, may be temporally/spatially organised in the startle brainstem centres. (2) Voluntary tibialis EMG activation was earlier and stronger in response to a startling stimulus (fall) than in response to a non-startling stimulus (sound). This suggests that the startle response can be regarded as a reticular mechanism enhancing motor responsiveness. (3) Elderly subjects showed similar activation sequences but delayed by about 20 ms. This delay is more than can be accounted for by slowing of central and peripheral motor conduction, therefore suggesting age dependent delay in central processing. (4) Avestibular patients had normal latencies indicating that the free fall startle can be elicited by non-vestibular inputs. (5) Latencies in patients with idiopathic Parkinson's disease were normal whereas responses were earlier in patients with multiple system atrophy (MSA) and delayed or absent in patients with Steele-Richardson-Olszewski (SRO) syndrome. The findings in this patient group suggest: (1) lack of dopaminergic influence on the timing of the startle response, (2) concurrent cerebellar involvement in MSA may cause startle disinhibition, and (3) extensive reticular damage in SRO severely interferes with the response to free fall.  (+info)

Affective symptoms in multiple system atrophy and Parkinson's disease: response to levodopa therapy. (2/215)

The objective was to determine the extent to which psychiatric disturbances (especially mood disorders) generally considered poor prognostic factors, are present in patients with striatonigral (SND) type multiple system atrophy (MSA) compared with patients with idiopathic Parkinson's disease (IPD). The Hamilton depression scale (HAM-D), brief psychiatric rating scale (BPRS), and Unified Parkinson's disease rating scale (UPDRS) were administered to clinically probable non-demented patients with SND-type MSA and patients with IPD matched for age and motor disability, at baseline and after receiving levodopa. At baseline total HAM-D score was greater in patients with IPD. Overall, BPRS score did not differ between the two groups; however, patients with IPD scored higher on anxiety items of the BPRS, and patients with MSA had higher scores on the item indicating blunted affect. After levodopa, both groups improved significantly in UPDRS and HAM-D total scores (just significant for patients with MSA). Patients with IPD improved significantly in total BPRS score but patients with MSA did not. At baseline patients with IPD were more depressed and anxious than patients with MSA who, by contrast, showed blunted affect. After levodopa, depression and anxiety of patients with IPD improved significantly whereas the affective detachment of patients with MSA did not change. Major neuronal loss in the caudate and ventral striatum, which are part of the lateral orbitofrontal and limbic circuits, may be responsible for the blunted affect not responsive to levodopa therapy found in patients with MSA.  (+info)

Multiple system atrophy. (3/215)

Multiple system atrophy is a neurological disorder that has gone unrecognized for too long due to its involvement across multiple regions of the central nervous system. This disorder is finally being unveiled through increased reporting in the scientific literature. Further research will enhance our understanding of this disease and lead to more effective treatment regimens as well as an improved quality of life for patients with MSA.  (+info)

The genetics of disorders with synuclein pathology and parkinsonism. (4/215)

Despite being considered the archetypal non-genetic neurological disorder, genetic analysis of Parkinson's disease has shown that there are at least three genetic loci. Furthermore, these analyses have suggested that the phenotype of the pathogenic loci is wider than simple Parkinson's disease and may include Lewy body dementia and some forms of essential tremor. Identification of alpha-synuclein as the first of the loci involved in Parkinson's disease and the identification of this protein in pathological deposits in other disorders has led to the suggestion that it may share pathogenic mechanisms with multiple system atrophy, Alzheimer's disease and prion disease and that these mechanisms are related to a synuclein pathway to cell death. Finally, genetic analysis of the synuclein diseases and the tau diseases may indicate that this synuclein pathway is an alternative to the tau pathway to cell death.  (+info)

Nocturnal decrease in vasopressin secretion into plasma in patients with multiple system atrophy. (5/215)

To determine whether the nocturnal decrease in arginine vasopressin (AVP) secretion into the plasma, found in a patient with multiple system atrophy (MSA) reported previously, is a usual finding in MSA, the plasma AVP concentrations in 13 patients with MSA were measured every 4 hours during a 24 hour period. The plasma AVP concentrations in these patients showed significant daily variations and were the lowest during the night. This finding indicates that patients with MSA often exhibit nocturnal decrease in AVP secretion into the plasma. The results suggest the possibility that the system responsible for the daily variations in AVP secretion is involved in MSA.  (+info)

Widespread alterations of alpha-synuclein in multiple system atrophy. (6/215)

Glial cytoplasmic inclusions (GCI) are the hallmark of multiple system atrophy (MSA), a rare movement disorder frequently associated with autonomic dysfunction. In this study of 21 cases of MSA, GCI were consistently immunoreactive for alpha-synuclein and double-immunostained for ubiquitin and oligodendroglial markers, but not glial fibrillary acidic protein. No statistically significant difference was found in the density of GCI in various brain regions in the two forms of MSA, striatonigral degeneration (SND) and olivopontocerebellar atrophy (OPCA). Postmortem brain samples from 9 cases of MSA were fractionated according to solubility in buffer, Triton-X 100, sodium dodecyl sulfate (SDS), and formic acid, and alpha-synuclein immunoreactivity was measured in Western blots. Total alpha-synuclein immunoreactivity was increased in MSA compared to controls, with no statistically significant difference between SND and OPCA. Most of the increase was due to alpha-synuclein in SDS fractions. In controls this fraction had little or no immunoreactivity. In 7 cases and 4 controls correlations were investigated between quantitative neuropathology and biochemical properties of alpha-synuclein. Surprisingly, the amount of SDS-soluble alpha-synuclein correlated poorly with the number of GCI in adjacent sections. Furthermore, areas with few or no GCI unexpectedly had abundant SDS-soluble alpha-synuclein. These findings provide evidence that modifications of alpha-synuclein in MSA may be more widespread than obvious histopathology. Moreover, these alterations may constitute a biochemical signature for the synucleinopathies.  (+info)

Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation? (7/215)

OBJECTIVES: Urinary dysfunction and orthostatic hypotension are the prominent autonomic features in multiple system atrophy (MSA). A detailed questionnaire was given and autonomic function tests were performed in 121 patients with MSA concerning both urinary and cardiovascular systems. METHODS: Replies to the questionnaire on autonomic symptoms were obtained from 121 patients including three clinical variants; olivopontocerebellar atrophy (OPCA) type in 48, striatonigral degeneration (SND) type in 17, and Shy-Drager type in 56. Urodynamic studies comprised measurement of postmicturition residuals, EMG cystometry, and bethanechol injection. Cardiovascular tests included head up tilt test, measurement of supine plasma noradrenaline (norepinephrine,NA), measurement of R-R variability (CV R-R), and intravenous infusions of NA and isoproterenol. RESULTS: Urinary symptoms (96%) were found to be more common than orthostatic symptoms (43%) (p<0.01) in patients with MSA, particularly with OPCA (p<0.01) and SND (p<0.01) types. In 53 patients with both urinary and orthostatic symptoms, patients who had urinary symptoms first (48%) were more common than those who had orthostatic symptoms first (29%), and there were patients who developed both symptoms simultaneously (23%). Post-micturition residuals were noted in 74% of the patients. EMG cystometry showed detrusor hyperreflexia in 56%, low compliance in 31%, atonic curve in 5%, detrusor-sphincter dyssynergia in 45%, and neurogenic sphincter EMG in 74%. The cystometric curve tended to change from hyperreflexia to low compliance, then atonic curve in repeated tests. Bethanechol injection showed denervation supersensitivity of the bladder in 19%. Cardiovascular tests showed orthostatic hypotension below -30 mm Hg in 41%, low CV R-R below 1.5 in 57%, supine plasma NA below 100 pg/ml in 28%, and denervation supersensitivity of the vessels (alpha in 73%; beta2 in 60%) and of the heart (beta1 in 62%). CONCLUSION: It is likely that urinary dysfunction is more common and often an earlier manifestation than orthostatic hypotension in patients with MSA, although subclinical cardiovascular abnormalities appear in the early stage of the disease. The responsible sites seem to be central and peripheral for both dysfunctions.  (+info)

Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. (8/215)

Dentatorubral and pallidoluysian atrophy (DRPLA) is a member of a family of progressive neurodegenerative diseases caused by polyglutamine repeat expansion. Transgenic mice expressing full-length human atrophin-1 with 65 consecutive glutamines exhibit ataxia, tremors, abnormal movements, seizures, and premature death. These mice accumulate atrophin-1 immunoreactivity and inclusion bodies in the nuclei of multiple populations of neurons. Subcellular fractionation revealed 120 kDa nuclear fragments of mutant atrophin-1, whose abundance increased with age and phenotypic severity. Brains of DRPLA patients contained apparently identical 120 kDa nuclear fragments. By contrast, mice overexpressing atrophin-1 with 26 glutamines were phenotypically normal and did not accumulate the 120 kDa fragments. We conclude that the evolution of neuropathology in DRPLA involves proteolytic processing of mutant atrophin-1 and nuclear accumulation of truncated fragments.  (+info)

Multiple System Atrophy (MSA) is a rare, progressive neurodegenerative disorder that affects multiple systems in the body. It is characterized by a combination of symptoms including Parkinsonism (such as stiffness, slowness of movement, and tremors), cerebellar ataxia (lack of muscle coordination), autonomic dysfunction (problems with the autonomic nervous system which controls involuntary actions like heart rate, blood pressure, sweating, and digestion), and pyramidal signs (abnormalities in the corticospinal tracts that control voluntary movements).

The disorder is caused by the degeneration of nerve cells in various parts of the brain and spinal cord, leading to a loss of function in these areas. The exact cause of MSA is unknown, but it is thought to involve a combination of genetic and environmental factors. There is currently no cure for MSA, and treatment is focused on managing symptoms and improving quality of life.

Olivopontocerebellar atrophies (OPCA) are a group of rare, progressive neurodegenerative disorders that primarily affect the cerebellum, olive (inferior olivary nucleus), and pons in the brainstem. The condition is characterized by degeneration and atrophy of these specific areas, leading to various neurological symptoms.

The term "olivopontocerebellar atrophies" encompasses several subtypes, including:

1. Hereditary spastic paraplegia with cerebellar ataxia (SPG/ATA) - Autosomal dominant or recessive inheritance pattern.
2. Hereditary dentatorubral-pallidoluysian atrophy (DRPLA) - Autosomal dominant inheritance pattern.
3. Idiopathic OPCA - No known genetic cause, possibly related to environmental factors or spontaneous mutations.

Symptoms of olivopontocerebellar atrophies may include:

* Progressive cerebellar ataxia (gait and limb incoordination)
* Dysarthria (slurred speech)
* Oculomotor abnormalities (nystagmus, gaze palsy)
* Spasticity (stiffness and rigidity of muscles)
* Dysphagia (difficulty swallowing)
* Tremors or dystonia (involuntary muscle contractions)

Diagnosis typically involves a combination of clinical examination, neuroimaging studies (MRI), genetic testing, and exclusion of other possible causes. Currently, there is no cure for olivopontocerebellar atrophies, but supportive care can help manage symptoms and improve quality of life.

Shy-Drager syndrome (SDS) is a rare and progressive neurodegenerative disorder that affects the autonomic nervous system (ANS). The ANS controls involuntary bodily functions such as heart rate, blood pressure, sweating, digestion, and pupil dilation. SDS is also known as multiple system atrophy with orthostatic hypotension or Bradbury-Eggleston syndrome.

SDS is characterized by a combination of symptoms related to the dysfunction of the autonomic nervous system, including:

1. Orthostatic hypotension (a sudden drop in blood pressure upon standing)
2. Autonomic failure (manifesting as erectile dysfunction, urinary retention or incontinence, and gastrointestinal disturbances)
3. Parkinsonian features (tremors, rigidity, bradykinesia, and postural instability)
4. Respiratory abnormalities (breathing difficulties, especially during sleep)
5. Ocular symptoms (abnormal pupil dilation and convergence insufficiency)
6. Smooth muscle atrophy (leading to reduced bladder capacity and gastrointestinal motility issues)

The underlying cause of Shy-Drager syndrome is the degeneration of nerve cells in specific areas of the brain, particularly within the autonomic nervous system centers. The exact etiology remains unclear; however, it is believed to involve a combination of genetic and environmental factors. There is no known cure for SDS, and treatment primarily focuses on managing symptoms and improving quality of life.

Progressive Supranuclear Palsy (PSP) is a rare neurological disorder characterized by the progressive degeneration of brain cells that regulate movement, thoughts, behavior, and eye movements. The term "supranuclear" refers to the location of the damage in the brain, specifically above the level of the "nuclei" which are clusters of nerve cells that control voluntary movements.

The most common early symptom of PSP is a loss of balance and difficulty coordinating eye movements, particularly vertical gaze. Other symptoms may include stiffness or rigidity of muscles, slowness of movement, difficulty swallowing, changes in speech and writing, and cognitive decline leading to dementia.

PSP typically affects people over the age of 60, and its progression can vary from person to person. Currently, there is no cure for PSP, and treatment is focused on managing symptoms and maintaining quality of life.

Alpha-synuclein is a protein that is primarily found in neurons (nerve cells) in the brain. It is encoded by the SNCA gene and is abundantly expressed in presynaptic terminals, where it is believed to play a role in the regulation of neurotransmitter release.

In certain neurological disorders, including Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy, alpha-synuclein can form aggregates known as Lewy bodies and Lewy neurites. These aggregates are a pathological hallmark of these diseases and are believed to contribute to the death of nerve cells, leading to the symptoms associated with these disorders.

The precise function of alpha-synuclein is not fully understood, but it is thought to be involved in various cellular processes such as maintaining the structure of the presynaptic terminal, regulating synaptic vesicle trafficking and neurotransmitter release, and protecting neurons from stress.

Atrophy is a medical term that refers to the decrease in size and wasting of an organ or tissue due to the disappearance of cells, shrinkage of cells, or decreased number of cells. This process can be caused by various factors such as disuse, aging, degeneration, injury, or disease.

For example, if a muscle is immobilized for an extended period, it may undergo atrophy due to lack of use. Similarly, certain medical conditions like diabetes, cancer, and heart failure can lead to the wasting away of various tissues and organs in the body.

Atrophy can also occur as a result of natural aging processes, leading to decreased muscle mass and strength in older adults. In general, atrophy is characterized by a decrease in the volume or weight of an organ or tissue, which can have significant impacts on its function and overall health.

The Autonomic Nervous System (ANS) is a part of the nervous system that controls involuntary actions, such as heart rate, digestion, respiratory rate, pupillary response, urination, and sexual arousal. It consists of two subdivisions: the sympathetic and parasympathetic nervous systems, which generally have opposing effects and maintain homeostasis in the body.

Autonomic Nervous System Diseases (also known as Autonomic Disorders or Autonomic Neuropathies) refer to a group of conditions that affect the functioning of the autonomic nervous system. These diseases can cause damage to the nerves that control automatic functions, leading to various symptoms and complications.

Autonomic Nervous System Diseases can be classified into two main categories:

1. Primary Autonomic Nervous System Disorders: These are conditions that primarily affect the autonomic nervous system without any underlying cause. Examples include:
* Pure Autonomic Failure (PAF): A rare disorder characterized by progressive loss of autonomic nerve function, leading to symptoms such as orthostatic hypotension, urinary retention, and constipation.
* Multiple System Atrophy (MSA): A degenerative neurological disorder that affects both the autonomic nervous system and movement coordination. Symptoms may include orthostatic hypotension, urinary incontinence, sexual dysfunction, and Parkinsonian features like stiffness and slowness of movements.
* Autonomic Neuropathy associated with Parkinson's Disease: Some individuals with Parkinson's disease develop autonomic symptoms such as orthostatic hypotension, constipation, and urinary dysfunction due to the degeneration of autonomic nerves.
2. Secondary Autonomic Nervous System Disorders: These are conditions that affect the autonomic nervous system as a result of an underlying cause or disease. Examples include:
* Diabetic Autonomic Neuropathy: A complication of diabetes mellitus that affects the autonomic nerves, leading to symptoms such as orthostatic hypotension, gastroparesis (delayed gastric emptying), and sexual dysfunction.
* Autoimmune-mediated Autonomic Neuropathies: Conditions like Guillain-Barré syndrome or autoimmune autonomic ganglionopathy can cause autonomic symptoms due to the immune system attacking the autonomic nerves.
* Infectious Autonomic Neuropathies: Certain infections, such as HIV or Lyme disease, can lead to autonomic dysfunction as a result of nerve damage.
* Toxin-induced Autonomic Neuropathy: Exposure to certain toxins, like heavy metals or organophosphate pesticides, can cause autonomic neuropathy.

Autonomic nervous system disorders can significantly impact a person's quality of life and daily functioning. Proper diagnosis and management are crucial for improving symptoms and preventing complications. Treatment options may include lifestyle modifications, medications, and in some cases, devices or surgical interventions.

Parkinson's disease is a progressive neurodegenerative disorder that affects movement. It is characterized by the death of dopamine-producing cells in the brain, specifically in an area called the substantia nigra. The loss of these cells leads to a decrease in dopamine levels, which results in the motor symptoms associated with Parkinson's disease. These symptoms can include tremors at rest, stiffness or rigidity of the limbs and trunk, bradykinesia (slowness of movement), and postural instability (impaired balance and coordination). In addition to these motor symptoms, non-motor symptoms such as cognitive impairment, depression, anxiety, and sleep disturbances are also common in people with Parkinson's disease. The exact cause of Parkinson's disease is unknown, but it is thought to be a combination of genetic and environmental factors. There is currently no cure for Parkinson's disease, but medications and therapies can help manage the symptoms and improve quality of life.

The putamen is a round, egg-shaped structure that is a part of the basal ganglia, located in the forebrain. It is situated laterally to the globus pallidus and medially to the internal capsule. The putamen plays a crucial role in regulating movement and is involved in various functions such as learning, motivation, and habit formation.

It receives input from the cerebral cortex via the corticostriatal pathway and sends output to the globus pallidus and substantia nigra pars reticulata, which are also part of the basal ganglia circuitry. The putamen is heavily innervated by dopaminergic neurons from the substantia nigra pars compacta, and degeneration of these neurons in Parkinson's disease leads to a significant reduction in dopamine levels in the putamen, resulting in motor dysfunction.

Synucleins are a family of small, heat-stable, water-soluble proteins that are primarily expressed in neurons. They are involved in various cellular processes such as modulating synaptic plasticity, vesicle trafficking, and neurotransmitter release. The most well-known members of this family are alpha-synuclein, beta-synuclein, and gamma-synuclein.

Abnormal accumulation and aggregation of alpha-synuclein into insoluble fibrils called Lewy bodies and Lewy neurites are hallmark features of several neurodegenerative disorders, including Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. These conditions are collectively referred to as synucleinopathies. The dysfunction and aggregation of alpha-synuclein are thought to contribute to the progressive loss of dopaminergic neurons in the substantia nigra pars compacta, a region of the brain involved in motor control, leading to the characteristic symptoms observed in these disorders.

Orthostatic hypotension is a type of low blood pressure that occurs when you stand up from a sitting or lying position. The drop in blood pressure causes a brief period of lightheadedness or dizziness, and can even cause fainting in some cases. This condition is also known as postural hypotension.

Orthostatic hypotension is caused by a rapid decrease in blood pressure when you stand up, which reduces the amount of blood that reaches your brain. Normally, when you stand up, your body compensates for this by increasing your heart rate and constricting blood vessels to maintain blood pressure. However, if these mechanisms fail or are impaired, orthostatic hypotension can occur.

Orthostatic hypotension is more common in older adults, but it can also affect younger people who have certain medical conditions or take certain medications. Some of the risk factors for orthostatic hypotension include dehydration, prolonged bed rest, pregnancy, diabetes, heart disease, Parkinson's disease, and certain neurological disorders.

If you experience symptoms of orthostatic hypotension, it is important to seek medical attention. Your healthcare provider can perform tests to determine the underlying cause of your symptoms and recommend appropriate treatment options. Treatment may include lifestyle changes, such as increasing fluid intake, avoiding alcohol and caffeine, and gradually changing positions from lying down or sitting to standing up. In some cases, medication may be necessary to manage orthostatic hypotension.

Cerebellar ataxia is a type of ataxia, which refers to a group of disorders that cause difficulties with coordination and movement. Cerebellar ataxia specifically involves the cerebellum, which is the part of the brain responsible for maintaining balance, coordinating muscle movements, and regulating speech and eye movements.

The symptoms of cerebellar ataxia may include:

* Unsteady gait or difficulty walking
* Poor coordination of limb movements
* Tremors or shakiness, especially in the hands
* Slurred or irregular speech
* Abnormal eye movements, such as nystagmus (rapid, involuntary movement of the eyes)
* Difficulty with fine motor tasks, such as writing or buttoning a shirt

Cerebellar ataxia can be caused by a variety of underlying conditions, including:

* Genetic disorders, such as spinocerebellar ataxia or Friedreich's ataxia
* Brain injury or trauma
* Stroke or brain hemorrhage
* Infections, such as meningitis or encephalitis
* Exposure to toxins, such as alcohol or certain medications
* Tumors or other growths in the brain

Treatment for cerebellar ataxia depends on the underlying cause. In some cases, there may be no cure, and treatment is focused on managing symptoms and improving quality of life. Physical therapy, occupational therapy, and speech therapy can help improve coordination, balance, and communication skills. Medications may also be used to treat specific symptoms, such as tremors or muscle spasticity. In some cases, surgery may be recommended to remove tumors or repair damage to the brain.

Inclusion bodies are abnormal, intracellular accumulations or aggregations of various misfolded proteins, protein complexes, or other materials within the cells of an organism. They can be found in various tissues and cell types and are often associated with several pathological conditions, including infectious diseases, neurodegenerative disorders, and genetic diseases.

Inclusion bodies can vary in size, shape, and location depending on the specific disease or condition. Some inclusion bodies have a characteristic appearance under the microscope, such as eosinophilic (pink) staining with hematoxylin and eosin (H&E) histological stain, while others may require specialized stains or immunohistochemical techniques to identify the specific misfolded proteins involved.

Examples of diseases associated with inclusion bodies include:

1. Infectious diseases: Some viral infections, such as HIV, hepatitis B and C, and herpes simplex virus, can lead to the formation of inclusion bodies within infected cells.
2. Neurodegenerative disorders: Several neurodegenerative diseases are characterized by the presence of inclusion bodies, including Alzheimer's disease (amyloid-beta plaques and tau tangles), Parkinson's disease (Lewy bodies), Huntington's disease (Huntingtin aggregates), and amyotrophic lateral sclerosis (TDP-43 and SOD1 inclusions).
3. Genetic diseases: Certain genetic disorders, such as Danon disease, neuronal intranuclear inclusion disease, and some lysosomal storage disorders, can also present with inclusion bodies due to the accumulation of abnormal proteins or metabolic products within cells.

The exact role of inclusion bodies in disease pathogenesis remains unclear; however, they are often associated with cellular dysfunction, oxidative stress, and increased inflammation, which can contribute to disease progression and neurodegeneration.

Lewy body disease, also known as dementia with Lewy bodies, is a type of progressive degenerative dementia that affects thinking, behavior, and movement. It's named after Dr. Friedrich Lewy, the scientist who discovered the abnormal protein deposits, called Lewy bodies, that are characteristic of this disease.

Lewy bodies are made up of a protein called alpha-synuclein and are found in the brain cells of individuals with Lewy body disease. These abnormal protein deposits are also found in people with Parkinson's disease, but they are more widespread in Lewy body disease, affecting multiple areas of the brain.

The symptoms of Lewy body disease can vary from person to person, but they often include:

* Cognitive decline, such as memory loss, confusion, and difficulty with problem-solving
* Visual hallucinations and delusions
* Parkinsonian symptoms, such as stiffness, tremors, and difficulty walking or moving
* Fluctuations in alertness and attention
* REM sleep behavior disorder, where a person acts out their dreams during sleep

Lewy body disease is a progressive condition, which means that the symptoms get worse over time. Currently, there is no cure for Lewy body disease, but medications can help manage some of the symptoms.

Midodrine is a medication that belongs to a class of drugs called vasoconstrictors. It works by narrowing the blood vessels and increasing blood pressure. The medical definition of Midodrine is:

A synthetic derivative of the imidazole compound, adrenergic agonist, which is used in the treatment of orthostatic hypotension. Midodrine is a prodrug that is rapidly metabolized to its active form, desglymidodrine, after oral administration. It selectively binds to and activates alpha-1 adrenergic receptors, causing vasoconstriction and an increase in blood pressure. The drug's effects are most pronounced on the venous side of the circulation, leading to increased venous return and cardiac output. Midodrine is typically administered orally in divided doses throughout the day, and its use is usually reserved for patients who have not responded to other treatments for orthostatic hypotension.

Thionins are a group of small, sulfur-rich, cysteine-rich proteins that are widely distributed in nature, particularly in seeds of various plants. They have been found in over 60 plant species and are known for their antimicrobial properties. Thionins are toxic to a wide range of organisms, including bacteria, fungi, and insects.

The name "thionin" comes from the high sulfur content of these proteins, which is reflected in their chemical structure. Thionins contain eight cysteine residues that form four disulfide bonds, giving them a stable and rigid structure. This structural feature allows thionins to maintain their activity under a wide range of conditions, including extreme temperatures and pH levels.

Thionins have been studied for their potential use as natural pesticides and antimicrobial agents in agriculture and medicine. However, their toxicity to non-target organisms, including mammals, has limited their practical application. Further research is needed to fully understand the mechanisms of thionin toxicity and to develop strategies for harnessing their potential benefits while minimizing their risks.

Neurological diagnostic techniques are medical tests and examinations used to identify and diagnose conditions related to the nervous system, which includes the brain, spinal cord, nerves, and muscles. These techniques can be divided into several categories:

1. Clinical Examination: A thorough physical examination, including a neurological evaluation, is often the first step in diagnosing neurological conditions. This may involve assessing a person's mental status, muscle strength, coordination, reflexes, sensation, and gait.

2. Imaging Techniques: These are used to produce detailed images of the brain and nervous system. Common imaging techniques include:

- Computed Tomography (CT): This uses X-rays to create cross-sectional images of the brain and other parts of the body.
- Magnetic Resonance Imaging (MRI): This uses a strong magnetic field and radio waves to produce detailed images of the brain and other internal structures.
- Functional MRI (fMRI): This is a type of MRI that measures brain activity by detecting changes in blood flow.
- Positron Emission Tomography (PET): This uses small amounts of radioactive material to produce detailed images of brain function.
- Single Photon Emission Computed Tomography (SPECT): This is a type of nuclear medicine imaging that uses a gamma camera and a computer to produce detailed images of brain function.

3. Electrophysiological Tests: These are used to measure the electrical activity of the brain and nervous system. Common electrophysiological tests include:

- Electroencephalography (EEG): This measures the electrical activity of the brain.
- Evoked Potentials (EPs): These measure the electrical response of the brain and nervous system to sensory stimuli, such as sound or light.
- Nerve Conduction Studies (NCS): These measure the speed and strength of nerve impulses.
- Electromyography (EMG): This measures the electrical activity of muscles.

4. Laboratory Tests: These are used to analyze blood, cerebrospinal fluid, and other bodily fluids for signs of neurological conditions. Common laboratory tests include:

- Complete Blood Count (CBC): This measures the number and type of white and red blood cells in the body.
- Blood Chemistry Tests: These measure the levels of various chemicals in the blood.
- Lumbar Puncture (Spinal Tap): This is used to collect cerebrospinal fluid for analysis.
- Genetic Testing: This is used to identify genetic mutations associated with neurological conditions.

5. Imaging Studies: These are used to produce detailed images of the brain and nervous system. Common imaging studies include:

- Magnetic Resonance Imaging (MRI): This uses a strong magnetic field and radio waves to produce detailed images of the brain and nervous system.
- Computed Tomography (CT): This uses X-rays to produce detailed images of the brain and nervous system.
- Functional MRI (fMRI): This measures changes in blood flow in the brain during cognitive tasks.
- Diffusion Tensor Imaging (DTI): This is used to assess white matter integrity in the brain.
- Magnetic Resonance Spectroscopy (MRS): This is used to measure chemical levels in the brain.

Striatonigral degeneration (SND) is a type of neurodegenerative disorder that affects the basal ganglia, specifically the striatum and the substantia nigra. It is also known as "striatonigral degeneration with olivopontocerebellar atrophy" or "multiple system atrophy-parkinsonian type (MSA-P)".

SND is characterized by the progressive loss of nerve cells in the striatum, which receives input from the cerebral cortex and sends output to the substantia nigra. This results in a decrease in the neurotransmitter dopamine, leading to symptoms similar to those seen in Parkinson's disease (PD), such as stiffness, slowness of movement, rigidity, and tremors.

However, unlike PD, SND is also associated with degeneration of the olivopontocerebellar system, which can lead to additional symptoms such as ataxia, dysarthria, and oculomotor abnormalities. The exact cause of striatonigral degeneration is unknown, but it is believed to involve a combination of genetic and environmental factors. Currently, there is no cure for the condition, and treatment is focused on managing the symptoms.

Parkinsonian disorders are a group of neurological conditions characterized by motor symptoms such as bradykinesia (slowness of movement), rigidity, resting tremor, and postural instability. These symptoms are caused by the degeneration of dopamine-producing neurons in the brain, particularly in the substantia nigra pars compacta.

The most common Parkinsonian disorder is Parkinson's disease (PD), which is a progressive neurodegenerative disorder. However, there are also several other secondary Parkinsonian disorders, including:

1. Drug-induced parkinsonism: This is caused by the use of certain medications, such as antipsychotics and metoclopramide.
2. Vascular parkinsonism: This is caused by small vessel disease in the brain, which can lead to similar symptoms as PD.
3. Dementia with Lewy bodies (DLB): This is a type of dementia that shares some features with PD, such as the presence of alpha-synuclein protein clumps called Lewy bodies.
4. Progressive supranuclear palsy (PSP): This is a rare brain disorder that affects movement, gait, and eye movements.
5. Multiple system atrophy (MSA): This is a progressive neurodegenerative disorder that affects multiple systems in the body, including the autonomic nervous system, motor system, and cerebellum.
6. Corticobasal degeneration (CBD): This is a rare neurological disorder that affects both movement and cognition.

It's important to note that while these disorders share some symptoms with PD, they have different underlying causes and may require different treatments.

Secondary Parkinson's disease, also known as acquired or symptomatic Parkinsonism, is a clinical syndrome characterized by the signs and symptoms of classic Parkinson's disease (tremor at rest, rigidity, bradykinesia, and postural instability) but caused by a known secondary cause. These causes can include various conditions such as brain injuries, infections, drugs or toxins, metabolic disorders, and vascular damage. The underlying pathology of secondary Parkinson's disease is different from that of classic Parkinson's disease, which is primarily due to the degeneration of dopamine-producing neurons in a specific area of the brain called the substantia nigra pars compacta.

Pure Autonomic Failure (PAF) is a rare neurological disorder characterized by the progressive loss of function of the autonomic nervous system, which regulates involuntary bodily functions such as heart rate, blood pressure, sweating, digestion, and bladder control. In PAF, there is no evidence of any other underlying disease or neurological condition that could explain these symptoms.

The primary feature of PAF is orthostatic hypotension, a sudden drop in blood pressure when standing up from a sitting or lying down position, which can lead to dizziness, lightheadedness, and even fainting. Other common symptoms include:

* Anhidrosis (inability to sweat) or hyperhidrosis (excessive sweating)
* Constipation or diarrhea
* Urinary incontinence or retention
* Sexual dysfunction
* Tachycardia (rapid heart rate) or bradycardia (slow heart rate)
* Difficulty regulating body temperature

The exact cause of PAF is unknown, but it is believed to be related to the degeneration of nerve cells in the autonomic nervous system. There is no cure for PAF, and treatment is focused on managing symptoms and preventing complications. This may include lifestyle changes such as increasing fluid and salt intake, wearing compression stockings, and avoiding prolonged periods of standing or sitting. Medications may also be prescribed to help regulate blood pressure, heart rate, and other autonomic functions.

Vocal cord paralysis is a medical condition characterized by the inability of one or both vocal cords to move or function properly due to nerve damage or disruption. The vocal cords are two bands of muscle located in the larynx (voice box) that vibrate to produce sound during speech, singing, and breathing. When the nerves that control the vocal cord movements are damaged or not functioning correctly, the vocal cords may become paralyzed or weakened, leading to voice changes, breathing difficulties, and other symptoms.

The causes of vocal cord paralysis can vary, including neurological disorders, trauma, tumors, surgery, or infections. The diagnosis typically involves a physical examination, including a laryngoscopy, to assess the movement and function of the vocal cords. Treatment options may include voice therapy, surgical procedures, or other interventions to improve voice quality and breathing functions.

Lewy bodies are abnormal aggregates of alpha-synuclein protein that develop in nerve cells (neurons) in the brain. They are named after Frederick Lewy, a German-American neurologist who discovered them while working with Dr. Alois Alzheimer. The presence of Lewy bodies is a hallmark feature of Lewy body dementia, which includes both Parkinson's disease dementia and dementia with Lewy bodies.

Lewy bodies can lead to the dysfunction and death of neurons in areas of the brain that control movement, cognition, and behavior. This can result in a range of symptoms, including motor impairments, cognitive decline, visual hallucinations, and mood changes. The exact role of Lewy bodies in the development and progression of these disorders is not fully understood, but they are believed to contribute to the neurodegenerative process that underlies these conditions.

A tonic pupil, also known as a "Adie's pupil," is a type of abnormal pupillary response named after Sir William John Adie, who first described it in 1932. It is characterized by an initial sluggish or absent reaction to light, followed by a slow and sustained redilation. This condition typically occurs as a result of damage to the ciliary ganglion or short ciliary nerves, which are part of the parasympathetic nervous system.

Tonic pupils can be unilateral (occurring in one eye) or bilateral (occurring in both eyes). They may be associated with other neurological symptoms such as decreased deep tendon reflexes and abnormal sweating patterns, depending on the extent of the damage to the autonomic nervous system.

It is important to note that tonic pupils can also occur as a result of various medical conditions, including viral infections, neurotoxins, trauma, or tumors. Therefore, it is essential to consult with a healthcare professional for proper evaluation and management.

Spinocerebellar degenerations (SCDs) are a group of genetic disorders that primarily affect the cerebellum, the part of the brain responsible for coordinating muscle movements, and the spinal cord. These conditions are characterized by progressive degeneration or loss of nerve cells in the cerebellum and/or spinal cord, leading to various neurological symptoms.

SCDs are often inherited in an autosomal dominant manner, meaning that only one copy of the altered gene from either parent is enough to cause the disorder. The most common type of SCD is spinocerebellar ataxia (SCA), which includes several subtypes (SCA1, SCA2, SCA3, etc.) differentiated by their genetic causes and specific clinical features.

Symptoms of spinocerebellar degenerations may include:

1. Progressive ataxia (loss of coordination and balance)
2. Dysarthria (speech difficulty)
3. Nystagmus (involuntary eye movements)
4. Oculomotor abnormalities (problems with eye movement control)
5. Tremors or other involuntary muscle movements
6. Muscle weakness and spasticity
7. Sensory disturbances, such as numbness or tingling sensations
8. Dysphagia (difficulty swallowing)
9. Cognitive impairment in some cases

The age of onset, severity, and progression of symptoms can vary significantly among different SCD subtypes and individuals. Currently, there is no cure for spinocerebellar degenerations, but various supportive treatments and therapies can help manage symptoms and improve quality of life.

Oligodendroglia are a type of neuroglial cell found in the central nervous system (CNS) of vertebrates, including humans. These cells play a crucial role in providing support and insulation to nerve fibers (axons) in the CNS, which includes the brain and spinal cord.

More specifically, oligodendroglia produce a fatty substance called myelin that wraps around axons, forming myelin sheaths. This myelination process helps to increase the speed of electrical impulse transmission (nerve impulses) along the axons, allowing for efficient communication between different neurons.

In addition to their role in myelination, oligodendroglia also contribute to the overall health and maintenance of the CNS by providing essential nutrients and supporting factors to neurons. Dysfunction or damage to oligodendroglia has been implicated in various neurological disorders, such as multiple sclerosis (MS), where demyelination of axons leads to impaired nerve function and neurodegeneration.

Extrapyramidal tracts are a part of the motor system that lies outside of the pyramidal tracts, which are responsible for controlling voluntary movements. These extrapyramidal tracts consist of several different pathways in the brain and spinal cord that work together to regulate and coordinate involuntary movements, muscle tone, and posture.

The extrapyramidal system includes structures such as the basal ganglia, cerebellum, and brainstem, and it helps to modulate and fine-tune motor activity. Disorders of the extrapyramidal tracts can result in a variety of symptoms, including rigidity, tremors, involuntary movements, and difficulty with coordination and balance.

Some common conditions that affect the extrapyramidal system include Parkinson's disease, Huntington's disease, and drug-induced movement disorders. Treatment for these conditions may involve medications that target specific components of the extrapyramidal system to help alleviate symptoms and improve function.

Gait ataxia is a type of ataxia, which refers to a lack of coordination or stability, specifically involving walking or gait. It is characterized by an unsteady, uncoordinated, and typically wide-based gait pattern. This occurs due to dysfunction in the cerebellum or its connecting pathways, responsible for maintaining balance and coordinating muscle movements.

In gait ataxia, individuals often have difficulty with controlling the rhythm and pace of their steps, tend to veer or stagger off course, and may display a reeling or stumbling motion while walking. They might also have trouble performing rapid alternating movements like quickly tapping their foot or heel. These symptoms are usually worse when the person is tired or attempting to walk in the dark.

Gait ataxia can be caused by various underlying conditions, including degenerative neurological disorders (e.g., cerebellar atrophy, multiple sclerosis), stroke, brain injury, infection (e.g., alcoholism, HIV), or exposure to certain toxins. Proper diagnosis and identification of the underlying cause are essential for effective treatment and management of gait ataxia.

The Recurrent Laryngeal Nerve (RLN) is a branch of the vagus nerve (cranial nerve X), which is a mixed sensory, motor, and autonomic nerve. The RLN has important functions in providing motor innervation to the intrinsic muscles of the larynx, except for the cricothyroid muscle, which is supplied by the external branch of the superior laryngeal nerve.

The recurrent laryngeal nerve supplies all the muscles that are responsible for adduction (bringing together) of the vocal cords, including the vocalis muscle, lateral cricoarytenoid, thyroarytenoid, and interarytenoid muscles. These muscles play a crucial role in voice production, coughing, and swallowing.

The right recurrent laryngeal nerve has a longer course than the left one. It loops around the subclavian artery in the chest before ascending to the larynx, while the left RLN hooks around the arch of the aorta. This anatomical course makes them vulnerable to injury during various surgical procedures, such as thyroidectomy and neck dissection, leading to potential voice impairment or vocal cord paralysis.

The brain is the central organ of the nervous system, responsible for receiving and processing sensory information, regulating vital functions, and controlling behavior, movement, and cognition. It is divided into several distinct regions, each with specific functions:

1. Cerebrum: The largest part of the brain, responsible for higher cognitive functions such as thinking, learning, memory, language, and perception. It is divided into two hemispheres, each controlling the opposite side of the body.
2. Cerebellum: Located at the back of the brain, it is responsible for coordinating muscle movements, maintaining balance, and fine-tuning motor skills.
3. Brainstem: Connects the cerebrum and cerebellum to the spinal cord, controlling vital functions such as breathing, heart rate, and blood pressure. It also serves as a relay center for sensory information and motor commands between the brain and the rest of the body.
4. Diencephalon: A region that includes the thalamus (a major sensory relay station) and hypothalamus (regulates hormones, temperature, hunger, thirst, and sleep).
5. Limbic system: A group of structures involved in emotional processing, memory formation, and motivation, including the hippocampus, amygdala, and cingulate gyrus.

The brain is composed of billions of interconnected neurons that communicate through electrical and chemical signals. It is protected by the skull and surrounded by three layers of membranes called meninges, as well as cerebrospinal fluid that provides cushioning and nutrients.

Sweat gland diseases are medical conditions that affect the functioning or structure of sweat glands, leading to excessive sweating (hyperhidrosis), lack of sweating (anhydrosis), or abnormal sweating (e.g., foul-smelling sweat). There are two main types of sweat glands in humans: eccrine glands, which produce a watery sweat that helps regulate body temperature, and apocrine glands, which are located in the armpits and groin and produce a thicker, milky sweat that can mix with bacteria on the skin and cause body odor.

Some examples of sweat gland diseases include:

1. Hidradenitis suppurativa: A chronic skin condition characterized by inflammation and infection of the apocrine glands, leading to the formation of abscesses, nodules, and sinus tracts.
2. Primary focal hyperhidrosis: A condition that causes excessive sweating in specific areas of the body, such as the armpits, hands, feet, or face, without any underlying medical cause.
3. Secondary generalized hyperhidrosis: Excessive sweating that affects the entire body and is caused by an underlying medical condition, such as diabetes, thyroid disease, or obesity.
4. Cystic adenoma of the axilla: A benign tumor that arises from the apocrine glands in the armpit.
5. Eccrine nevus: A rare congenital condition characterized by an increased number of eccrine glands in a localized area of the skin, leading to excessive sweating.
6. Fox-Fordyce disease: A chronic inflammatory disorder that affects the apocrine glands, causing itchy papules and pustules in the armpits and groin.
7. Pachyonychia congenita: A rare genetic disorder characterized by thickened nails, palmoplantar keratoderma, and abnormalities of the eccrine glands, leading to excessive sweating and odor production.

Muscular atrophy is a condition characterized by a decrease in the size and mass of muscles due to lack of use, disease, or injury. This occurs when there is a disruption in the balance between muscle protein synthesis and degradation, leading to a net loss of muscle proteins. There are two main types of muscular atrophy:

1. Disuse atrophy: This type of atrophy occurs when muscles are not used or are immobilized for an extended period, such as after an injury, surgery, or prolonged bed rest. In this case, the nerves that control the muscles may still be functioning properly, but the muscles themselves waste away due to lack of use.
2. Neurogenic atrophy: This type of atrophy is caused by damage to the nerves that supply the muscles, leading to muscle weakness and wasting. Conditions such as amyotrophic lateral sclerosis (ALS), spinal cord injuries, and peripheral neuropathies can cause neurogenic atrophy.

In both cases, the affected muscles may become weak, shrink in size, and lose their tone and mass. Treatment for muscular atrophy depends on the underlying cause and may include physical therapy, exercise, and medication to manage symptoms and improve muscle strength and function.

REM Sleep Behavior Disorder (RBD) is a parasomnia, which is a disorder that involves undesirable experiences or abnormal behaviors during sleep. Specifically, RBD is a type of rapid eye movement (REM) sleep parasomnia where the muscle atonia (lack of muscle tone) that normally occurs during REM sleep is absent or incomplete, allowing for the emergence of motor behaviors and vivid dreaming. These dreams can be quite intense and may result in the individual physically acting out their dreams, leading to potential harm for themselves or their bed partner. RBD can occur in isolation or as a symptom of another neurological condition.

The brainstem is the lower part of the brain that connects to the spinal cord. It consists of the midbrain, pons, and medulla oblongata. The brainstem controls many vital functions such as heart rate, breathing, and blood pressure. It also serves as a relay center for sensory and motor information between the cerebral cortex and the rest of the body. Additionally, several cranial nerves originate from the brainstem, including those that control eye movements, facial movements, and hearing.

Neurodegenerative diseases are a group of disorders characterized by progressive and persistent loss of neuronal structure and function, often leading to cognitive decline, functional impairment, and ultimately death. These conditions are associated with the accumulation of abnormal protein aggregates, mitochondrial dysfunction, oxidative stress, chronic inflammation, and genetic mutations in the brain. Examples of neurodegenerative diseases include Alzheimer's disease, Parkinson's disease, Huntington's disease, Amyotrophic Lateral Sclerosis (ALS), and Spinal Muscular Atrophy (SMA). The underlying causes and mechanisms of these diseases are not fully understood, and there is currently no cure for most neurodegenerative disorders. Treatment typically focuses on managing symptoms and slowing disease progression.

Levodopa, also known as L-dopa, is a medication used primarily in the treatment of Parkinson's disease. It is a direct precursor to the neurotransmitter dopamine and works by being converted into dopamine in the brain, helping to restore the balance between dopamine and other neurotransmitters. This helps alleviate symptoms such as stiffness, tremors, spasms, and poor muscle control. Levodopa is often combined with carbidopa (a peripheral decarboxylase inhibitor) to prevent the conversion of levodopa to dopamine outside of the brain, reducing side effects like nausea and vomiting.

Nerve degeneration, also known as neurodegeneration, is the progressive loss of structure and function of neurons, which can lead to cognitive decline, motor impairment, and various other symptoms. This process occurs due to a variety of factors, including genetics, environmental influences, and aging. It is a key feature in several neurological disorders such as Alzheimer's disease, Parkinson's disease, Huntington's disease, and multiple sclerosis. The degeneration can affect any part of the nervous system, leading to different symptoms depending on the location and extent of the damage.

The Autonomic Nervous System (ANS) is a part of the peripheral nervous system that operates largely below the level of consciousness and controls visceral functions. It is divided into two main subdivisions: the sympathetic and parasympathetic nervous systems, which generally have opposing effects and maintain homeostasis in the body.

The Sympathetic Nervous System (SNS) prepares the body for stressful or emergency situations, often referred to as the "fight or flight" response. It increases heart rate, blood pressure, respiratory rate, and metabolic rate, while also decreasing digestive activity. This response helps the body respond quickly to perceived threats.

The Parasympathetic Nervous System (PNS), on the other hand, promotes the "rest and digest" state, allowing the body to conserve energy and restore itself after the stress response has subsided. It decreases heart rate, blood pressure, and respiratory rate, while increasing digestive activity and promoting relaxation.

These two systems work together to maintain balance in the body by adjusting various functions based on internal and external demands. Disorders of the Autonomic Nervous System can lead to a variety of symptoms, such as orthostatic hypotension, gastroparesis, and cardiac arrhythmias, among others.

Laryngoscopy is a medical procedure that involves the examination of the larynx, which is the upper part of the windpipe (trachea), and the vocal cords using a specialized instrument called a laryngoscope. The laryngoscope is inserted through the mouth or nose to provide a clear view of the larynx and surrounding structures. This procedure can be performed for diagnostic purposes, such as identifying abnormalities like growths, inflammation, or injuries, or for therapeutic reasons, such as removing foreign objects or taking tissue samples for biopsy. There are different types of laryngoscopes and techniques used depending on the reason for the examination and the patient's specific needs.

The basal ganglia are a group of interconnected nuclei, or clusters of neurons, located in the base of the brain. They play a crucial role in regulating motor function, cognition, and emotion. The main components of the basal ganglia include the striatum (made up of the caudate nucleus, putamen, and ventral striatum), globus pallidus (divided into external and internal segments), subthalamic nucleus, and substantia nigra (with its pars compacta and pars reticulata).

The basal ganglia receive input from various regions of the cerebral cortex and other brain areas. They process this information and send output back to the thalamus and cortex, helping to modulate and coordinate movement. The basal ganglia also contribute to higher cognitive functions such as learning, decision-making, and habit formation. Dysfunction in the basal ganglia can lead to neurological disorders like Parkinson's disease, Huntington's disease, and dystonia.

Ephedrine is a medication that stimulates the nervous system and is used to treat low blood pressure, asthma, and nasal congestion. It works by narrowing the blood vessels and increasing heart rate, which can help to increase blood pressure and open up the airways in the lungs. Ephedrine may also be used as a bronchodilator to treat COPD (chronic obstructive pulmonary disease).

Ephedrine is available in various forms, including tablets, capsules, and solutions for injection. It is important to follow the instructions of a healthcare provider when taking ephedrine, as it can have side effects such as rapid heart rate, anxiety, headache, and dizziness. Ephedrine should not be used by people with certain medical conditions, such as heart disease, high blood pressure, or narrow-angle glaucoma, and it should not be taken during pregnancy or breastfeeding without consulting a healthcare provider.

In addition to its medical uses, ephedrine has been used as a performance-enhancing drug and is banned by many sports organizations. It can also be found in some over-the-counter cold and allergy medications, although these products are required to carry warnings about the potential for misuse and addiction.

Medical Definition:

Magnetic Resonance Imaging (MRI) is a non-invasive diagnostic imaging technique that uses a strong magnetic field and radio waves to create detailed cross-sectional or three-dimensional images of the internal structures of the body. The patient lies within a large, cylindrical magnet, and the scanner detects changes in the direction of the magnetic field caused by protons in the body. These changes are then converted into detailed images that help medical professionals to diagnose and monitor various medical conditions, such as tumors, injuries, or diseases affecting the brain, spinal cord, heart, blood vessels, joints, and other internal organs. MRI does not use radiation like computed tomography (CT) scans.

The cerebellum is a part of the brain that lies behind the brainstem and is involved in the regulation of motor movements, balance, and coordination. It contains two hemispheres and a central portion called the vermis. The cerebellum receives input from sensory systems and other areas of the brain and spinal cord and sends output to motor areas of the brain. Damage to the cerebellum can result in problems with movement, balance, and coordination.

Dihydroxyphenylalanine is not a medical term per se, but it is a chemical compound that is often referred to in the context of biochemistry and neuroscience. It is also known as levodopa or L-DOPA for short.

L-DOPA is a precursor to dopamine, a neurotransmitter that plays a critical role in regulating movement, emotion, and cognition. In the brain, L-DOPA is converted into dopamine through the action of an enzyme called tyrosine hydroxylase.

L-DOPA is used medically to treat Parkinson's disease, a neurological disorder characterized by motor symptoms such as tremors, rigidity, and bradykinesia (slowness of movement). In Parkinson's disease, the dopamine-producing neurons in the brain gradually degenerate, leading to a deficiency of dopamine. By providing L-DOPA as a replacement therapy, doctors can help alleviate some of the symptoms of the disease.

It is important to note that L-DOPA has potential side effects and risks, including nausea, dizziness, and behavioral changes. Long-term use of L-DOPA can also lead to motor complications such as dyskinesias (involuntary movements) and fluctuations in response to the medication. Therefore, it is typically used in combination with other medications and under the close supervision of a healthcare provider.

Antiparkinson agents are a class of medications used to treat the symptoms of Parkinson's disease and related disorders. These agents work by increasing the levels or activity of dopamine, a neurotransmitter in the brain that is responsible for regulating movement and coordination.

There are several types of antiparkinson agents, including:

1. Levodopa: This is the most effective treatment for Parkinson's disease. It is converted to dopamine in the brain and helps to replace the missing dopamine in people with Parkinson's.
2. Dopamine agonists: These medications mimic the effects of dopamine in the brain and can be used alone or in combination with levodopa. Examples include pramipexole, ropinirole, and rotigotine.
3. Monoamine oxidase B (MAO-B) inhibitors: These medications block the breakdown of dopamine in the brain and can help to increase its levels. Examples include selegiline and rasagiline.
4. Catechol-O-methyltransferase (COMT) inhibitors: These medications block the breakdown of levodopa in the body, allowing it to reach the brain in higher concentrations. Examples include entacapone and tolcapone.
5. Anticholinergic agents: These medications block the action of acetylcholine, another neurotransmitter that can contribute to tremors and muscle stiffness in Parkinson's disease. Examples include trihexyphenidyl and benztropine.

It is important to note that antiparkinson agents can have side effects, and their use should be carefully monitored by a healthcare professional. The choice of medication will depend on the individual patient's symptoms, age, overall health, and other factors.

Ataxia is a medical term that refers to a group of disorders affecting coordination, balance, and speech. It is characterized by a lack of muscle control during voluntary movements, causing unsteady or awkward movements, and often accompanied by tremors. Ataxia can affect various parts of the body, such as the limbs, trunk, eyes, and speech muscles. The condition can be congenital or acquired, and it can result from damage to the cerebellum, spinal cord, or sensory nerves. There are several types of ataxia, including hereditary ataxias, degenerative ataxias, cerebellar ataxias, and acquired ataxias, each with its own specific causes, symptoms, and prognosis. Treatment for ataxia typically focuses on managing symptoms and improving quality of life, as there is no cure for most forms of the disorder.

The perineum is the region between the anus and the genitals. In anatomical terms, it refers to the diamond-shaped area located in the lower part of the pelvis and extends from the coccyx (tailbone) to the pubic symphysis, which is the joint in the front where the two pubic bones meet. This region contains various muscles that support the pelvic floor and contributes to maintaining urinary and fecal continence. The perineum can be further divided into two triangular regions: the urogenital triangle (anterior) and the anal triangle (posterior).

Nerve tissue proteins are specialized proteins found in the nervous system that provide structural and functional support to nerve cells, also known as neurons. These proteins include:

1. Neurofilaments: These are type IV intermediate filaments that provide structural support to neurons and help maintain their shape and size. They are composed of three subunits - NFL (light), NFM (medium), and NFH (heavy).

2. Neuronal Cytoskeletal Proteins: These include tubulins, actins, and spectrins that provide structural support to the neuronal cytoskeleton and help maintain its integrity.

3. Neurotransmitter Receptors: These are specialized proteins located on the postsynaptic membrane of neurons that bind neurotransmitters released by presynaptic neurons, triggering a response in the target cell.

4. Ion Channels: These are transmembrane proteins that regulate the flow of ions across the neuronal membrane and play a crucial role in generating and transmitting electrical signals in neurons.

5. Signaling Proteins: These include enzymes, receptors, and adaptor proteins that mediate intracellular signaling pathways involved in neuronal development, differentiation, survival, and death.

6. Adhesion Proteins: These are cell surface proteins that mediate cell-cell and cell-matrix interactions, playing a crucial role in the formation and maintenance of neural circuits.

7. Extracellular Matrix Proteins: These include proteoglycans, laminins, and collagens that provide structural support to nerve tissue and regulate neuronal migration, differentiation, and survival.

The pons is a part of the brainstem that lies between the medulla oblongata and the midbrain. Its name comes from the Latin word "ponte" which means "bridge," as it serves to connect these two regions of the brainstem. The pons contains several important structures, including nerve fibers that carry signals between the cerebellum (the part of the brain responsible for coordinating muscle movements) and the rest of the nervous system. It also contains nuclei (clusters of neurons) that help regulate various functions such as respiration, sleep, and facial movements.

Urodynamics is a medical test that measures the function and performance of the lower urinary tract, which includes the bladder, urethra, and sphincters. It involves the use of specialized equipment to record measurements such as bladder pressure, urine flow rate, and residual urine volume. The test can help diagnose various urinary problems, including incontinence, urinary retention, and overactive bladder.

During the test, a small catheter is inserted into the bladder through the urethra to measure bladder pressure while filling it with sterile water or saline solution. Another catheter may be placed in the rectum to record abdominal pressure. The patient is then asked to urinate, and the flow rate and any leaks are recorded.

Urodynamics can help identify the underlying cause of urinary symptoms and guide treatment decisions. It is often recommended for patients with complex or persistent urinary problems that have not responded to initial treatments.

Electromyography (EMG) is a medical diagnostic procedure that measures the electrical activity of skeletal muscles during contraction and at rest. It involves inserting a thin needle electrode into the muscle to record the electrical signals generated by the muscle fibers. These signals are then displayed on an oscilloscope and may be heard through a speaker.

EMG can help diagnose various neuromuscular disorders, such as muscle weakness, numbness, or pain, and can distinguish between muscle and nerve disorders. It is often used in conjunction with other diagnostic tests, such as nerve conduction studies, to provide a comprehensive evaluation of the nervous system.

EMG is typically performed by a neurologist or a physiatrist, and the procedure may cause some discomfort or pain, although this is usually minimal. The results of an EMG can help guide treatment decisions and monitor the progression of neuromuscular conditions over time.

Salicylamides are organic compounds that consist of a salicylic acid molecule (a type of phenolic acid) linked to an amide group. They are derivatives of salicylic acid and are known for their analgesic, anti-inflammatory, and antipyretic properties. Salicylamides have been used in various pharmaceutical and therapeutic applications, including the treatment of pain, fever, and inflammation. However, they have largely been replaced by other compounds such as acetylsalicylic acid (aspirin) due to their lower potency and potential side effects.

A neurological examination is a series of tests used to evaluate the functioning of the nervous system, including both the central nervous system (the brain and spinal cord) and peripheral nervous system (the nerves that extend from the brain and spinal cord to the rest of the body). It is typically performed by a healthcare professional such as a neurologist or a primary care physician with specialized training in neurology.

During a neurological examination, the healthcare provider will assess various aspects of neurological function, including:

1. Mental status: This involves evaluating a person's level of consciousness, orientation, memory, and cognitive abilities.
2. Cranial nerves: There are 12 cranial nerves that control functions such as vision, hearing, smell, taste, and movement of the face and neck. The healthcare provider will test each of these nerves to ensure they are functioning properly.
3. Motor function: This involves assessing muscle strength, tone, coordination, and reflexes. The healthcare provider may ask the person to perform certain movements or tasks to evaluate these functions.
4. Sensory function: The healthcare provider will test a person's ability to feel different types of sensations, such as touch, pain, temperature, vibration, and proprioception (the sense of where your body is in space).
5. Coordination and balance: The healthcare provider may assess a person's ability to perform coordinated movements, such as touching their finger to their nose or walking heel-to-toe.
6. Reflexes: The healthcare provider will test various reflexes throughout the body using a reflex hammer.

The results of a neurological examination can help healthcare providers diagnose and monitor conditions that affect the nervous system, such as stroke, multiple sclerosis, Parkinson's disease, or peripheral neuropathy.

The caudate nucleus is a part of the brain located within the basal ganglia, a group of structures that are important for movement control and cognition. It has a distinctive C-shaped appearance and plays a role in various functions such as learning, memory, emotion, and motivation. The caudate nucleus receives inputs from several areas of the cerebral cortex and sends outputs to other basal ganglia structures, contributing to the regulation of motor behavior and higher cognitive processes.

The Substantia Nigra is a region in the midbrain that plays a crucial role in movement control and reward processing. It is composed of two parts: the pars compacta and the pars reticulata. The pars compacta contains dopamine-producing neurons, whose loss or degeneration is associated with Parkinson's disease, leading to motor symptoms such as tremors, rigidity, and bradykinesia.

In summary, Substantia Nigra is a brain structure that contains dopamine-producing cells and is involved in movement control and reward processing. Its dysfunction or degeneration can lead to neurological disorders like Parkinson's disease.

Sudden death is a term used to describe a situation where a person dies abruptly and unexpectedly, often within minutes to hours of the onset of symptoms. It is typically caused by cardiac or respiratory arrest, which can be brought on by various medical conditions such as heart disease, stroke, severe infections, drug overdose, or trauma. In some cases, the exact cause of sudden death may remain unknown even after a thorough post-mortem examination.

It is important to note that sudden death should not be confused with "sudden cardiac death," which specifically refers to deaths caused by the abrupt loss of heart function (cardiac arrest). Sudden cardiac death is often related to underlying heart conditions such as coronary artery disease, cardiomyopathy, or electrical abnormalities in the heart.

"Multiple System Atrophy Clinical Presentation". Retrieved January 7, 2018. Swan L, Dupont J (May 1999). "Multiple system ... Parkinson's disease and multiple system atrophy, being the first process to give an objective diagnosis of Multiple System ... "The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: ... Multiple system atrophy can be explained as cell loss and gliosis or a proliferation of astrocytes in damaged areas of the ...
... -deafness syndrome "Multiple system atrophy - cerebellar subtype: MedlinePlus Medical Encyclopedia ... diseases formerly categorized as olivopontocerebellar atrophy have been reclassified as forms of multiple system atrophy as ... and multiple system atrophy (MSA), with which it is primarily associated. OPCA may also be found in the brains of individuals ... Overground harness systems may be used to allow OPCA patients to challenge their balance without chance of falling. Furthermore ...
In January 2015, Hare broadcast the BBC Radio 4 Appeal to raise money for the Multiple System Atrophy Trust, which was founded ... "Sir David Hare to broadcast the BBC Radio 4 Appeal for the MSA Trust". Multiple System Atrophy Trust. 18 December 2014. Hare, ... In 1993 Hare's best friend Sarah Matheson was diagnosed with Multiple System Atrophy and died from the disease in 1999. ... comes about to raze the current system altogether, to replace it with perfection. In 2016, Hare wrote the screenplay for Denial ...
"Multiple System Atrophy with Orthostatic Hypotension Information Page". Archived from the original on 2012-05-14. Retrieved ... They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). Dementia ... Mark, M. H. (2001). "Lumping and splitting the Parkinson Plus syndromes: dementia with Lewy bodies, multiple system atrophy, ... and may occur either as an inherited disorder or as a variant of multiple system atrophy. MSA is also characterized by ...
Kerry Simon, 60, American chef, multiple system atrophy. Ray Smolover, 94, American opera director and hazzan. Bruno Stutz, 77 ... C. K. Williams, 78, American poet, winner of the Pulitzer Prize for Poetry (2000), multiple myeloma. Ben Cauley, 67, American ...
Multiple system atrophy (MSA) causes degeneration of multiple parts of the nervous system and results in muscle rigidity, ... It took several years before Schnarndorf received the correct diagnosis of Multiple System Atrophy. Her condition worsened in ... 19 (1). "Multiple System Atrophy: Background, Etiology and Pathophysiology, Epidemiology". July 19, 2017. {{cite journal}}: ... "Multiple System Atrophy Fact Sheet , National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved ...
Almost a third of people with isolated, late onset cerebellar ataxia go on to develop multiple system atrophy. The cerebellum's ... Multiple System Atrophy~differential at eMedicine Manto, Mario; Marien, Peter (2015). "Schmahmann's syndrome - identification ... such as progressive supranuclear palsy and multiple system atrophy). Gluten ataxia accounts for 40% of all sporadic idiopathic ... Systemic atrophies primarily affecting the central nervous system). ...
Ubhi, K; Low, P; Masliah, E (2011). "Multiple System Atrophy: A Clinical and Neuropathological Perspective". Trends in ... Multiple system atrophy Muscular dystrophies (MD) Neuronal ceroid lipofuscinosis Niemann-Pick diseases Osteoarthritis ... The other two common groups of degenerative diseases are those that affect circulatory system (e.g. coronary artery disease) ... In neurodegenerative diseases, cells of the central nervous system stop working or die via neurodegeneration. An example of ...
"Pareidolia in Parkinson's Disease and Multiple System Atrophy". Parkinson's Disease. 2021: 2704755. doi:10.1155/2021/2704755. ... These objects include a number of plant species, a coin with Roman numerals, and multiple insect species. In an experimental ...
Martha Kostuch, 58, Canadian environmentalist, multiple system atrophy. Cook Lougheed, 86, American entrepreneur and ... Carmen Silva, 92, Brazilian actress, multiple organ failure. Al Wilson, 68, American soul singer ("Show and Tell"), kidney ... Renata Fronzi, 82, Argentine-born Brazilian actress, multiple organ dysfunction syndrome. Hendrik S. Houthakker, 83, American ...
Wataru Mimura, 67, Japanese screenwriter (Godzilla), multiple system atrophy. Barbara Moore, 89, English composer. Hans Müller ... Anupam Shyam, 63, Indian actor (Hazaaron Khwaishein Aisi, Slumdog Millionaire, The Curse of King Tut's Tomb), multiple organ ... Robin Miller, 71, American motorsports journalist (The Indianapolis Star, Speed Channel, NBCSN), multiple myeloma and leukemia ... dies at 76 Actor Anupam Shyam passes away at 63 due to multiple organ failure PBA Charter Member, Hall of Famer Harry "Tiger" ...
Parkinson's disease and multiple system atrophy, being the first process to give an objective diagnosis of Multiple System ... "Method Can Distinguish Parkinson's Disease From multiple system atrophy". Diagnostics from Technology Networks. Retrieved 23 ... "Discriminating α-synuclein strains in Parkinson's disease and multiple system atrophy". Nature. 578 (7794): 273-277. Bibcode: ... PMCA is conceptually analogous to the polymerase chain reaction - in both systems a template grows at the expense of a ...
Alpha-synuclein from Multiple system atrophy and TMEM106B. Scheres has been a member of the Board of Reviewing Editors for ... "Structures of alpha-synuclein filaments from multiple system atrophy". Nature. 585 (7825): 464-469. Bibcode:2020Natur.585..464S ... Since then, Scheres and Goedert have also solved the cryo-EM structures of tau filaments from multiple other Tauopathies, as ...
Gary I. Wadler, 78, American physician, multiple system atrophy. Edith Windsor, 88, American mathematician and activist, lead ... M. Cherif Bassiouni, 79, Egyptian lawyer and human rights activist, multiple myeloma. Richard Beckler, 77, American attorney. ...
Poewe W, Mahlknecht P, Krismer F (September 2015). "Therapeutic advances in multiple system atrophy and progressive ... 255 Rasagiline was tested for efficacy in people with multiple system atrophy in a large randomized, placebo-controlled, double ... "Peripheral and Central Nervous System Advisory Committee Background Package on Azilect" (PDF). FDA. Retrieved December 7, 2011 ... Multiple chemicals in Infobox drug, Chemicals using indexlabels, Drug has EMA link, Drugboxes which contain changes to verified ...
... multiple systems atrophy. Alois Eisenträger, 90, German footballer. Don Gross, 86, American baseball player (Cincinnati Redlegs ... 2000), Out of the shadows, [The Program], OCLC 46605442{{citation}}: CS1 maint: multiple names: authors list (link) Reinhard ... Emerson H. Fly, 82, American academic administrator, president of the University of Tennessee system (2001-2002), Parkinson's ... Boris Spremo, 81, Yugoslav-born Canadian photojournalist (Toronto Star, The Globe and Mail), complications from multiple ...
Karen was diagnosed with Multiple System Atrophy (MSA) in 2016. Since that time, she worked to promote end of life options for ...
Paul Humphrey, 61, Canadian musician (Blue Peter), multiple system atrophy. Digvijaysinh Jhala, 88, Indian politician, MP (1980 ... multiple system atrophy. Normand Cherry, 82, Canadian politician, Quebec MNA (1989-1998). Massimo Cuttitta, 54, Italian rugby ...
On August 26, 2021, Mimura died from multiple system atrophy. Born in Mie Prefecture and graduating from the College of Art of ...
In 2020 Olsen was diagnosed with multiple system atrophy (MSA). In January 2023 music journalist Jane Gazzo, singer Adalita and ...
He died on June 22, 2020, from multiple system atrophy. Four-minute mile World record progression for the mile run ^1 The "i" ... Deaths from multiple system atrophy). ...
... and multiple system atrophy). It has been found that EBNA1 may induce chromosomal breakage in the 11th chromosome, specifically ... Other late lytic gene products, such as BCRF1, help EBV evade the immune system. EGCG, a polyphenol in green tea, has shown in ... It's only after Epstein-Barr virus infection that the risk of multiple sclerosis jumps up by over 30 fold", and that only EBV ... Hassani A, Corboy JR, Al-Salam S, Khan G (2018). "Epstein-Barr virus is present in the brain of most cases of multiple ...
May 2008). "Clinical outcomes of progressive supranuclear palsy and multiple system atrophy". Brain. 131 (Pt 5): 1362-72. doi: ... and multiple system atrophy (MSA). Canada: PSP Society of Canada, a federally registered non-profit organization which serves ... MRI may show atrophy in the midbrain with preservation of the pons giving a "hummingbird" sign. Based on the pathological ... CS1 maint: multiple names: authors list, CS1 Japanese-language sources (ja), CS1 Chinese-language sources (zh), CS1: long ...
Victor Braun, 65, Canadian baritone, Shy-Drager syndrome, multiple system atrophy. Nadezhda Grigoryevna Grekova, 90, Soviet/ ... Maxine Mesinger, 75, American newspaper columnist (Houston Chronicle), complications of multiple sclerosis. Paul Olum, 82, ...
Sydney Selwyn, 62, British physician and medical scientist, multiple system atrophy. Gudmundur Arnlaugsson, 83, Icelandic chess ... Mark Lenard, 72, American actor (Star Trek), multiple myeloma. Adeniran Ogunsanya, 78, Nigerian lawyer and politician. Edmund ...
... multiple system atrophy, and pure autonomic failure. Sudomotor dysfunction can manifest as increased or decreased sweating ... "Anhidrosis in multiple system atrophy involves pre‐ and postganglionic sudomotor dysfunction". Movement Disorders. 32 (3): 397- ... Sudomotor function refers to the autonomic nervous system control of sweat gland activity in response to various environmental ... Impaired sudomotor function can occur in any disorder that directly and/or indirectly affects the autonomic nervous system, ...
... multiple system atrophy. Robert V. Levine, 73, American psychologist. Ambachew Mekonnen, Ethiopian politician, President of ... Ning Bin, 60, Chinese control systems engineer, President of Beijing Jiaotong University (2008-2019), traffic collision. Vaira ... multiple organ failure. Yves Bot, 71, French magistrate, Advocate General of the European Court of Justice (since 2006). Bill ... developer of GEORGE operating system. Babayo Garba Gamawa, 53, Nigerian politician. Rod Hall, 81, American off-road racing ...
April 2020). "RFC1 Intronic Repeat Expansions Absent in Pathologically Confirmed Multiple Systems Atrophy". Movement Disorders ... researchers have also investigated the presence of RFC1 expansions in pathologically confirmed multiple system atrophy (MSA) ...
... multiple system atrophy. At first she was diagnosed as having Parkinson's disease but that was later defined as Multiple System ... She died at home of the rare disease, multiple system atrophy. Perry was born in Burbank, California, in 1943. Her passion for ... Deaths from multiple system atrophy, Pan American Games medalists in volleyball, Medalists at the 1963 Pan American Games, ... Atrophy, aka Parkinson plus syndrome. "Obituaries Audrey Young, Mary Perry". LA Times. Retrieved June 14, 2012. Evans, Hilary; ...
... multiple system atrophy. Jan D. Achenbach, 85, Dutch-American acoustical engineer and academic. Edward Alexander, 83, American ... Winner of multiple world bowls titles dies aged 88 Morto Pierluigi Camiscioni, azzurro del rugby e controfigura di Bud Spencer ... multiple organ failure caused by COVID-19. Nikolai Gubenko, 78, Russian actor, director, and screenwriter (A Soldier Came Back ... multiple myeloma. Franca Valeri, 100, Italian actress (Il vedovo, The Shortest Day, Gli onorevoli). Laurent Vicomte, 64, French ...
"Multiple System Atrophy Clinical Presentation". Retrieved January 7, 2018. Swan L, Dupont J (May 1999). "Multiple system ... Parkinsons disease and multiple system atrophy, being the first process to give an objective diagnosis of Multiple System ... "The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: ... Multiple system atrophy can be explained as cell loss and gliosis or a proliferation of astrocytes in damaged areas of the ...
Multiple system atrophy is a progressive brain disorder that affects movement and balance and disrupts the function of the ... autonomic nervous system. Explore symptoms, inheritance, genetics of this condition. ... Multiple-System Atrophy Research Collaboration. Mutations in COQ2 in familial and sporadic multiple-system atrophy. N Engl J ... medlineplus.gov/genetics/condition/multiple-system-atrophy/ Multiple system atrophy. ...
The clinical diagnosis of multiple system atrophy (MSA) is fraught with difficulty and there are no pathognomonic features to ... Red flags for multiple system atrophy Mov Disord. 2008 Jun 15;23(8):1093-9. doi: 10.1002/mds.21992. ... The clinical diagnosis of multiple system atrophy (MSA) is fraught with difficulty and there are no pathognomonic features to ... Multiple System Atrophy / classification * Multiple System Atrophy / diagnosis* * Neurologic Examination / statistics & ...
Multiple system atrophy (MSA) is defined as an adult-onset, sporadic, rapidly progressive, multisystem, neurodegenerative fatal ... encoded search term (Multiple System Atrophy) and Multiple System Atrophy What to Read Next on Medscape ... Brain monoamine systems in multiple system atrophy: A positron emission tomography study. Neurobiol Dis. 2012 Jan 12. [QxMD ... Autopsy confirmed multiple system atrophy cases: Mayo experience and role of autonomic function tests. J Neurol Neurosurg ...
... did not show a difference between the levels of MCP-1 in the cerebrospinal fluid of people with multiple system atrophy and ...
... and multiple system atrophy (MSA). To further explore the haplotype structure at the SNCA locus in these two related disorders ... whether the haplotype structure at the SNCA locus allows us to differentiate individuals with multiple system atrophy from ... and multiple system atrophy (MSA). To further explore the haplotype structure at the SNCA locus in these two related disorders ... whether the haplotype structure at the SNCA locus allows us to differentiate individuals with multiple system atrophy from ...
Clinical features and natural history of multiple system atrophy (MSA) have been established in four recent series.1-4 Multiple ... Survival in multiple system atrophy: a study of prognostic factors in 59 cases. J Neurol1996;243:401-4. ... Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT. J Neurol Neurosurg ... Multiple system atrophy-the nature of the beast. J Neurol Neurosurg Psychiatry1989;52(suppl):78-89. ...
... multiple system atrophy. April 2017. Ritjes husband JiePie passed away from the rare disease multiple system atrophy (MSA) in ... Multiple system atrophy (MSA) is a progressive neurological disorder that affects adult men and women. It is caused by ... Ritje & JiePies story: multiple system atrophy. Ritje & JiePies story: ...
Multiple system atrophy Articles Case Reports Symptoms Treatment, Spain. ...
Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy. ... Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy. ... Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy. ...
Gene therapy is under development for the treatment of Parkinsons disease and multiple system atrophy. The drug candidate ... Likelihood of Approval and Phase Transition Success Rate Model - Gene Therapy Programs for Multiple System Atrophy and ... Gene Therapy Programs for Multiple System Atrophy and Parkinsons Disease Drug Details ... Gene Therapy Programs for Multiple System Atrophy and Parkinsons Disease dashboard for 12 months, with up-to-the-minute ...
Multiple system atrophy (MSA). Overview. Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting ... Multiple system atrophy (MSA) affects many parts of your body. Symptoms usually start in adulthood, usually in the 50s or 60s. ... Multiple system atrophy (MSA) is not known to be an inherited condition, so a family history of a condition with similar ... Diagnosing multiple system atrophy (MSA) can be challenging. Certain signs and symptoms of MSA - such as muscle rigidity and ...
Multiple System Atrophy Symptoms, Treatment, epidemiology, companies, drugs, infographic is a short guide to understand the ... Multiple System Atrophy Market Insight. The market size of Multiple System Atrophy in the 7MM was found to be approximately USD ... Multiple System Atrophy. Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by a ... Multiple System Atrophy Market Infographics. Multiple System Atrophy (MSA) - Market Insight, Epidemiology And Market Forecast ...
Learn more about the Multiple System Atrophy Trust staff that make us who we are: ... Learn more about the Multiple System Atrophy Trust staff that make us who we are:. Chief Executive Officer. Karen Walker Karen ... Multiple System Atrophy Trust. 51 St Olavs Court. City Business Centre. Lower Road, London. SE16 2XB, UK ... Multiple System Atrophy Trust. Registered Charity Number 1137652 (England & Wales) and SC044535 (Scotland). Site by Base Media. ...
Multiple System Atrophy (MSA) - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - ... Etiology of Multiple System Atrophy Etiology of multiple system atrophy is unknown, but neuronal degeneration occurs in several ... Symptoms and Signs of Multiple System Atrophy Initial symptoms of multiple system atrophy vary but include a combination of ... Both types involve autonomic nervous system dysfunction. Although multiple system atrophy begins as one type, symptoms of the ...
Multiple system atrophy (MSA) is a rare atypical parkinsonian disorder characterized by a rapidly progressing clinical course ... Multiple system atrophy (MSA) is a rare atypical parkinsonian disorder characterized by a rapidly progressing clinical course ... α-Synuclein-induced myelination deficit defines a novel interventional target for multiple system atrophy ... Multiple system atrophy; Oligodendrocytes; Oligodendrocyte progenitor cells; Myelin; alpha-Synuclein. Dewey Decimal ...
Multiple Systems Atrophy (MSA), Magnetoencephalography. Gait Analysis in Neurological Disease Diagnosing Frontotemporal Lobar ... PROgressive Supranuclear Palsy CorTico-Basal Syndrome Multiple System Atrophy Longitudinal Study UK Study of BIIB092 in ... Multiple Systems Atrophy (MSA), Magnetoencephalography.. May 27, 2020. checkorphan ... Analysis of the Enteric Nervous System Using Colonic Biopsies Safety Study of TPI-287 to Treat CBS and PSP Phase 0 Evaluation ...
Olivopontocerebellar atrophy (OPCA) is a neurodegenerative syndrome characterized by prominent cerebellar and extrapyramidal ... Evolution of sporadic olivopontocerebellar atrophy into multiple system atrophy. Neurology. 2000 Aug 22. 55(4):527-32. [QxMD ... Multiple-system atrophy. N Engl J Med. 2015 Jan 15. 372 (3):249-63. [QxMD MEDLINE Link]. [Full Text]. ... Neuropathology of multiple system atrophy: New thoughts about pathogenesis. Mov Disord. 2014 Oct 9. [QxMD MEDLINE Link]. ...
... November 20, 2023. ... 1. AskBio announces first patient randomized in phase 1 trial of AB-1005 (AAV2-GDNF) gene therapy for multiple system atrophy- ... Additionally, investigators will observe changes in quality of life using the Multiple System Atrophy Quality of Life ... 2. GDNF Gene therapy for multiple system atrophy. Clinicaltrials.gov. Updated November 16, 2023. Accessed November 19, 2023. ...
Olivopontocerebellar atrophy-deafness syndrome "Multiple system atrophy - cerebellar subtype: MedlinePlus Medical Encyclopedia ... diseases formerly categorized as olivopontocerebellar atrophy have been reclassified as forms of multiple system atrophy as ... and multiple system atrophy (MSA), with which it is primarily associated. OPCA may also be found in the brains of individuals ... Overground harness systems may be used to allow OPCA patients to challenge their balance without chance of falling. Furthermore ...
I have heard of too many MSA patients having leg & back pain. MSA is a loss of function and death of different types of nerve cells in the brain and spinal cord. It only makes sense that if it affects the spinal cord, the patients would have back and leg pain. The neurologists need to listen to patients and realize there is definitely a connection between MSA & back/leg pain.. ...
He walks down a hallway straight line, turning head from side to side looking at walls Sits in a chair and turns his head from side to side but keeping his eyes on the same spot on the wall. Walks down the hallway one foot in front of the other ,have the wall near buy as this is a hard one to do for him, needs to have wall near by to slide hand along when gets overbalanced. Standing still near support has one foot right in front of other, heel to toe of other foot. Daily walking very important as well. My advise would be for your husband be assessed by a physio first. This has helped my hubby as he is walking far more confidently during the day. At night is another matter.. ...
Laryngeal dystonia in multiple system atrophy [6]. / Simpson, D. M.; Kaufmann, H.; Sanders, I. et al. In: Muscle and Nerve, Vol ... Laryngeal dystonia in multiple system atrophy [6]. In: Muscle and Nerve. 1992 ; Vol. 15, No. 10. pp. 1213-1215. ... Simpson, DM, Kaufmann, H, Sanders, I & Wolfe, DE 1992, Laryngeal dystonia in multiple system atrophy [6], Muscle and Nerve, ... Simpson, D. M., Kaufmann, H., Sanders, I., & Wolfe, D. E. (1992). Laryngeal dystonia in multiple system atrophy [6]. Muscle and ...
Multiple System Atrophy MSA. Please Visit and Join the WeHeal Multiple System Atrophy Community. ... Please Visit and Join the WeHeal Multiple System Atrophy Community.. WeHeal is very grateful to our valued sources of ... Multiple system atrophy (MSA) is a rare neurological disorder that impairs your bodys involuntary (autonomic) functions, ...
Barry Gillespie focuses on multiple system atrophy (MSA), showing how the Gillespie Approach can target causes and help improve ... Birth Trauma, Emotional Trauma, Fascial Web, Golden Hour, Multiple System Atrophy (MSA), Physical Trauma, Tightness, Trauma ... "Multiple System Atrophy is a rare neurodegenerative disorder that can cause many diverse symptoms in any combination with ... In the early 50s the ever tightening fascial web starts to cumulatively create malfunction in some of her or his body systems ...
... multiple system atrophy Treatment Options for Multiple System Atrophy November 21, 2013. multiple system atrophy, Parkinsons ... multiple-system-atrophy. There are many causes of neurological symptoms. I believe it is always wise to pause and consider what ... My doctor recently switched my diagnosis from Parkinsons Disease to MSA (Multiple System Atrophy). Do you know of any ...
We investigated the survival of patients with multiple system atrophy (MSA) in a follow-up study of 59 patients admitted to ... Although in OPCA, SND and SDS the pathological alterations of the central nervous system are known to be very similar, ... Keywords: prognosis, follow-up study, olivopontocerebellar atrophy, Shy-Drager syndrome, striatonigral degeneration ... They consisted of 24 patients with olivopontocerebellar atrophy (OPCA), 25 with Shy-Drager syndrome (SDS) and 10 with ...
Neurological disorders like multiple system atrophy can make everyday tasks like walking difficult. Treatment for symptoms is ... Multiple system atrophy causes deterioration and shrinkage (atrophy) of the parts of your brain that regulate body functions ... Multiple System Atrophy (MSA) Treatment in Estero, FL. Do you have rigid muscles, balance problems, or slurred speech? Do you ... Multiple system atrophy is rare, occurring in about five in every 100,000 people. While there is currently no permanent cure, ...
This is the system of nerves that controls functions that help you survive. ... Autonomic dysfunction occurs when the autonomic nervous system is damaged. ... 2014). Multiple system atrophy (MSA): Definition.. http://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/basics ... Multiple system atrophy (MSA). MSA is a fatal form of autonomic dysfunction. Early on, it has symptoms similar to Parkinsons ...
... and Parkinsons Disease are degenerative diseases of the nervous system that affect movement. How are they different? ... Both multiple system atrophy (MSA) and Parkinsons disease are degenerative diseases of the nervous system that affect movement ... whereas MSA affects whats called the autonomic nervous system. This is the system that controls the automatic functions of the ...
  • MSA was formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration. (middlesexhealth.org)
  • It includes 3 disorders previously thought to be distinct: olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager syndrome. (msdmanuals.com)
  • This condition is also known by the names like olivopontocerebellar atrophy or striatonigral degeneration. (naturalayurvedictreatment.com)
  • They consisted of 24 patients with olivopontocerebellar atrophy (OPCA), 25 with Shy-Drager syndrome (SDS) and 10 with striatonigral degeneration (SND). (go.jp)
  • Although in OPCA, SND and SDS the pathological alterations of the central nervous system are known to be very similar, characterized as MSA, the present study suggests that the earlier and the more severe the involvement of the autonomic nervous system, and to a lesser extent the striatonigral system, the poorer the prognosis may be. (go.jp)
  • In 1960, van de Eecken, Adams, and van Bogaert reported 3 patients with striatonigral degeneration (SND) with atrophy of the caudate nucleus and putamen. (medscape.com)
  • MSA includes conditions that were previously known individually as Shy-Drager syndrome, striatonigral degeneration and sporadic olivopontocerebellar atrophy. (parkinson.ca)
  • The first cases of MSA were presented as olivopontocerebellar atrophy (OPCA) about a century ago. (medscape.com)
  • Care of olivopontocerebellar atrophy (OPCA) is directed to the treatment of symptoms. (medscape.com)
  • At times, olivopontocerebellar atrophy (OPCA) patients may require enteral feeding to decrease the risk of aspiration. (medscape.com)
  • As dysphagia progresses with olivopontocerebellar atrophy (OPCA), a pureed diet or enteral feeding may be required. (medscape.com)
  • Olivopontocerebellar atrophy (OPCA) is the degeneration of neurons in specific areas of the brain - the cerebellum, pons, and inferior olivary nucleus. (wikipedia.org)
  • OPCA is present in several neurodegenerative syndromes, including inherited and non-inherited forms of ataxia (such as the hereditary spinocerebellar ataxia known as Machado-Joseph disease) and multiple system atrophy (MSA), with which it is primarily associated. (wikipedia.org)
  • Overground harness systems may be used to allow OPCA patients to challenge their balance without chance of falling. (wikipedia.org)
  • Dejerine and Thomas first used the term olivopontocerebellar atrophy (OPCA) in 1900 when they described 2 patients with a degenerative disorder leading to progressive cerebellar dysfunction and parkinsonism. (medscape.com)
  • The clinical diagnosis of multiple system atrophy (MSA) is fraught with difficulty and there are no pathognomonic features to discriminate the parkinsonian variant (MSA-P) from Parkinson's disease (PD). (nih.gov)
  • We have recently shown that genetic variants at the SNCA gene locus, coding for the alpha-synuclein protein, confer increased risk of Parkinson's disease (PD) and multiple system atrophy (MSA). (michaeljfox.org)
  • We plan to investigate whether the haplotype structure at the SNCA locus allows us to differentiate individuals with multiple system atrophy from individuals with Parkinson's disease and from normal controls. (michaeljfox.org)
  • Gene therapy is under development for the treatment of Parkinson's disease and multiple system atrophy. (globaldata.com)
  • My doctor recently switched my diagnosis from Parkinson's Disease to MSA (Multiple System Atrophy). (parkinsonsrecovery.com)
  • Both multiple system atrophy (MSA) and Parkinson's disease are degenerative diseases of the nervous system that affect movement and worsen over time. (parkinsonsdisease.net)
  • In this study, we induced α-synuclein aggregation in human iPSC-derived dopaminergic neurons using fibrils generated de novo or amplified in the presence of brain homogenates from Parkinson's disease or multiple system atrophy. (ox.ac.uk)
  • We investigated the effect of a balanced liquid meal on blood pressure (BP) and heart rate (with patients supine and during head-up tilt), and on levels of plasma catecholamines, glucose, and insulin, in patients with idiopathic Parkinson's disease (IPD), multiple system atrophy (MSA), pure autonomic failure (PAF), and in healthy subjects (controls). (neurology.org)
  • We examined the positive rates of anti-NAE antibodies in 47 patients with multiple system atrophy (MSA), 29 patients with Parkinson's disease (PD), eight patients with corticobasal syndrome (CBS), and 18 patients with progressive supranuclear palsy (PSP) using conventional immunoblot analysis . (bvsalud.org)
  • As an iron chaperone, it has excellent potential to treat Parkinson's disease as well as various Parkinsonian disorders such as Multiple System Atrophy (MSA). (tmcnet.com)
  • 1997). Apraxia in Parkinson's disease, progressive supranuclear palsy, multiple system atrophy and neuroleptic-induced parkinsonism. (bvsalud.org)
  • SCA6, N=5) or cerebellar multiple system atrophy (MSA-C, N=5) and 15 age-matched healthy controls. (umn.edu)
  • Researchers have described two major types of multiple system atrophy, which are distinguished by their major signs and symptoms at the time of diagnosis. (medlineplus.gov)
  • MSA is also different from multiple organ dysfunction syndrome, sometimes referred to as multiple organ failure, and from multiple organ system failures, an often-fatal complication of septic shock and other severe illnesses or injuries. (wikipedia.org)
  • Multiple system atrophy is also known as shy-drager syndrome. (naturalayurvedictreatment.com)
  • Shy drager's syndrome causes shrinkage or atrophy of portions of your brain that are cerebellum, basal ganglia and brainstem. (naturalayurvedictreatment.com)
  • Patients typically live for around 7-10 years after he or she has been diagnosed with multiple system atrophy or Shy-drager syndrome. (naturalayurvedictreatment.com)
  • An inadequate response to treatment in a patient with parkinsonian symptoms suggests the possibility of a Parkinson-plus syndrome and warrants a search for the signs and symptoms of degeneration in other neuronal systems. (medscape.com)
  • enolase antibodies in patients with multiple system atrophy and corticobasal syndrome. (bvsalud.org)
  • High prevalence of serum anti-NH 2 -terminal of α-enolase antibodies in patients with multiple system atrophy and corticobasal syndrome. (bvsalud.org)
  • Besides nerve localization in the peripheral nervous system, it occurs in diseases of the presynaptic neuromuscular junction such as botulism and myasthenic syndrome. (medscape.com)
  • Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) and ataxia. (wikipedia.org)
  • Multiple System Atrophy is a rare neurodegenerative disorder that can cause many diverse symptoms in any combination with typical onset in the early 50s. (gillespieapproach.com)
  • If you develop any of the signs and symptoms associated with multiple system atrophy, see your doctor for an evaluation and diagnosis. (middlesexhealth.org)
  • MSA causes deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that affect internal body functions and motor control. (middlesexhealth.org)
  • Multiple system atrophy causes deterioration and shrinkage (atrophy) of the parts of your brain that regulate body functions like digestion and motor control. (neurology-clinics.com)
  • The total number of incident cases of Multiple System Atrophy observed in the 7MM was observed to be 36K in the year 2021. (delveinsight.com)
  • Fast Five Quiz: Spinal Muscular Atrophy - Medscape - Jan 14, 2021. (medscape.com)
  • Therefore, this study demonstrated multiple neurochemical alterations in SCAs and MSA-C relative to controls and the potential for these neurochemical levels to differentiate ataxia types. (umn.edu)
  • Multiple system atrophy (MSA) is a horrible and unrelenting neurodegenerative disorder with an uncertain etiology and pathophysiology. (biomedcentral.com)
  • Dejerine-Thomas atrophy" J. J. Dejerine, A. Thomas. (wikipedia.org)
  • 1- 4 Multiple system atrophy is usually defined by the predominance of parkinsonian (MSA-P type) or cerebellar (MSA-C type) features. (bmj.com)
  • Multiple system atrophy (MSA) is a rare atypical parkinsonian disorder characterized by a rapidly progressing clinical course and at present without any efficient therapy. (uni-regensburg.de)
  • Multiple system atrophy (MSA) is a fatal neurodegenerative disorder that presents clinically with varying combinations of autonomic, parkinsonian, cerebellar, and pyramidal dysfunction [ 15 ]. (biomedcentral.com)
  • Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the autonomic nervous system and movement.The etiology of MSA is unknown and majority of the cases are sporadic. (delveinsight.com)
  • Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. (msdmanuals.com)
  • MELBOURNE, Australia and SAN FRANCISCO, May 30, 2023 (GLOBE NEWSWIRE) -- Alterity Therapeutics (ASX: ATH, NASDAQ: ATHE) ("Alterity" or "the Company"), a biotechnology company dedicated to developing disease modifying treatments for neurodegenerative diseases, today announced it has commenced a new Phase 2 clinical trial of ATH434 in patients with Multiple System Atrophy (MSA) and the first patient has been enrolled. (tmcnet.com)
  • Multiple System Atrophy (MSA) is a rare, neurodegenerative disease characterized by failure of the autonomic nervous system and impaired movement. (tmcnet.com)
  • In all cases, multiple system atrophy is characterized by clumps of abnormal alpha-synuclein protein that, for unknown reasons, build up in cells in many parts of the brain and spinal cord. (medlineplus.gov)
  • In multiple system atrophy there is overexpression of alpha- synuclein named protein in brain neurons. (naturalayurvedictreatment.com)
  • Multiple system atrophy is a complex condition that is likely caused by the interaction of multiple genetic, environmental, and lifestyle factors. (medlineplus.gov)
  • Olivopontocerebellar atrophy is hereditary, but has an unknown genetic basis. (wikipedia.org)
  • Is Multiple System Atrophy Hereditary? (multiplesystematrophy.org)
  • Spinal muscular atrophy (SMA) refers to a group of hereditary diseases that damage and kill motor neurons in the brain and spinal cord. (medscape.com)
  • Frontiers in Systems Neuroscience. (lu.se)
  • A smaller CND study published in Neurology suggests the test may also be able to differentiate PD from multiple system atrophy, which can look similar in its early stages. (medscape.com)
  • Multiple system atrophy has a prevalence of 2 to 5 per 100,000 people. (medlineplus.gov)
  • Multiple system atrophy is rare, occurring in about five in every 100,000 people. (neurology-clinics.com)
  • Many people affected by MSA experience dysfunction of the autonomic nervous system, which commonly manifests as orthostatic hypotension, impotence, loss of sweating, dry mouth and urinary retention and incontinence. (wikipedia.org)
  • The most frequent autonomic symptoms associated with multiple system atrophy are a sudden drop in blood pressure upon standing ( orthostatic hypotension ), urinary difficulties, and erectile dysfunction in men. (medlineplus.gov)
  • General signs include signs that are related to different systems like orthostatic hypotension that makes you feel dizzy and light headed, constipation, bowel incontinence, reduced production of sweat, tears and saliva. (naturalayurvedictreatment.com)
  • Multiple system atrophy is a progressive brain disorder that affects movement and balance and disrupts the function of the autonomic nervous system. (medlineplus.gov)
  • The progressive loss of cells in these regions underlies the major features of multiple system atrophy. (medlineplus.gov)
  • Multiple system atrophy (MSA) is a progressive neurological disorder that affects adult men and women. (eurordis.org)
  • The clinical definition of multiple system atrophy (MSA) is a progressive, idiopathic, degenerative process beginning in adulthood. (medscape.com)
  • They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP ), corticobasal degeneration (CBD) and Dementia with Lewy bodies (DLB). (parkinson.ca)
  • Multiple System Atrophy (MSA) is a progressive brain disorder caused by loss of nerve cells in specific areas of the brain. (parkinson.ca)
  • Multiple system atrophy (MSA) affects many parts of your body. (middlesexhealth.org)
  • Multiple system atrophy affects men and women equally. (msdmanuals.com)
  • One key difference is that the nerve destruction in Parkinson's tends to occur in the areas of the brain that control movement, whereas MSA affects what's called the autonomic nervous system. (parkinsonsdisease.net)
  • Nemo defect affects multiple organs and presents with vesiculo-bullous lesions with erythema with linear arrangement on the extremities and lateral aspects of the trunk. (lu.se)
  • The 2 major divisions are the Sympathetic system Parasympathetic. (msdmanuals.com)
  • The ANS includes the sympathetic autonomic nervous system (SANS) and the parasympathetic autonomic nervous system (PANS). (healthline.com)
  • Most organs have nerves from both the sympathetic and parasympathetic systems. (healthline.com)
  • The neurotransmitter for preganglionic sympathetic and parasympathetic nervous system (PNS) as well as postganglionic parasympathetic nervous system is acetylcholine (ACh). (medscape.com)
  • The Multiple System Atrophy Coalition® is very proud to have a world-class Scientific Advisory Board (SAB) comprised of renowned globally-diverse researchers, scientists and physicians who are considered thought-leaders in the field of multiple system atrophy research. (multiplesystematrophy.org)
  • Cerebellar abnormalities predominate in olivopontocerebellar atrophy. (msdmanuals.com)
  • This disorder is usually prenatally lethal in males and contributes to abnormalities of skin, hair, nails, teeth and central nervous system in carrier females. (lu.se)
  • Researchers have also examined environmental factors that could contribute to the risk of multiple system atrophy. (medlineplus.gov)
  • In 2017, he founded the Austrian Otto Loewi Society to promote the study of all diseases of the autonomic nervous system. (multiplesystematrophy.org)
  • Defining Phenotypes of Movement Disorders :Parkinson's Plus Disorders (PD), Essential Tremor (ET), Cortical Basal Degeneration (CBD), Multiple Systems Atrophy (MSA), Magnetoencephalography. (checkorphan.org)
  • Available at https://www.ninds.nih.gov/health-information/disorders/olivopontocerebellar-atrophy . (medscape.com)
  • These disorders have complex clinical presentations that reflect degeneration in various neuronal systems. (medscape.com)
  • Given the complex nature of this system, a stepwise approach to autonomic disorders is required for proper understanding. (medscape.com)
  • Because the autonomic nervous system maintains internal physiologic homeostasis, disorders of this system can be present with both central as well as peripheral nervous system localization. (medscape.com)
  • Variations in the COQ2 gene have been associated with multiple system atrophy in people of Japanese descent, but this association has not been found in other populations. (medlineplus.gov)
  • It is unclear how changes in the SNCA or COQ2 gene increase the risk of developing multiple system atrophy. (medlineplus.gov)
  • Recently, recessive mutations of the COQ2 gene have been identified in two unrelated Japanese families with multiple system atrophy (MSA). (elsevierpure.com)
  • Based on the dominant clinical features, multiple system atrophy (MSA) is classified into two subgroups: MSA-C and MSA-P. However, the major pathology of MSA appears in three regions: nigrostriatal dopamine neurons, striatal projection neurons and pontocerebellar neurons. (mdsabstracts.org)
  • The market size of Multiple System Atrophy in the 7MM was found to be approximately USD 125 billion in 2022. (delveinsight.com)
  • Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple system atrophy. (bmj.com)
  • This is the system that controls the automatic functions of the body, like blood pressure, breathing, bladder function, and muscle control. (parkinsonsdisease.net)
  • Developmental disuse (i.e. hind limb immobilization) associated with PA induced muscle fiber atrophy, extracellular matrix changes in the muscle, and mild to moderate ankle and knee joint degeneration at levels greater than disuse alone. (cdc.gov)
  • The trial will assess a number of secondary outcomes as well, including MSA symptoms and signs, through the Unified Multiple System Atrophy Rating Scale (UMSARS), and change in striatal dopamine transporter (DaT) binding, using Single Photon Emission Computed Tomography DaT imaging. (neurologylive.com)
  • Which parts of the brain are affected in Multiple System Atrophy? (parkinson.ca)
  • In MSA, brain cells in the affected areas shrink (atrophy). (parkinson.ca)
  • The region in question includes the SHC2 gene which, in mice and rats, appears to have some function in the nervous system. (wikipedia.org)
  • Multiple system atrophy can be explained as cell loss and gliosis or a proliferation of astrocytes in damaged areas of the central nervous system. (wikipedia.org)
  • The autonomic nervous system controls body functions that are mostly involuntary, such as regulation of blood pressure. (medlineplus.gov)
  • Over time, these clumps (which are known as inclusions) damage cells in parts of the nervous system that control movement, balance and coordination, and autonomic functioning. (medlineplus.gov)
  • Overview of the Autonomic Nervous System The autonomic nervous system regulates physiologic processes. (msdmanuals.com)
  • Both types involve autonomic nervous system dysfunction. (msdmanuals.com)
  • The autonomic nervous system (ANS) controls several basic functions. (healthline.com)
  • What is the autonomic nervous system? (healthline.com)
  • Any problem with the functioning of the autonomic nervous system, which controls unconscious body functions that affect the bladder, bowels, sweating, sexual function and blood pressure. (michaeljfox.org)
  • A thin layer of tightly packed cells separating the central nervous system from the body's blood stream. (michaeljfox.org)
  • The autonomic nervous system (ANS) is a very complex, multifaceted neural network that maintains internal physiologic homeostasis. (medscape.com)
  • The goal for this article remains focused at step III on the anatomy of the autonomic nervous system, as follows. (medscape.com)
  • The neurotransmitter for the postganglionic sympathetic nervous system (innervating sweat glands) is also acetylcholine, whereas that for the remaining postganglionic sympathetic nervous system is norepinephrine (NE). (medscape.com)
  • Isolated systems anomalies included central nervous system (12 cases), cardiovascular system (9 cases), skeletal system (7 cases) and gastrointestinal system (6 cases). (who.int)
  • Some research suggests that there may be too much buildup of this protein in multiple system atrophy. (middlesexhealth.org)