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 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 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)
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.
Striped GRAY MATTER and WHITE MATTER consisting of the NEOSTRIATUM and paleostriatum (GLOBUS PALLIDUS). It is located in front of and lateral to the THALAMUS in each cerebral hemisphere. The gray substance is made up of the CAUDATE NUCLEUS and the lentiform nucleus (the latter consisting of the GLOBUS PALLIDUS and PUTAMEN). The WHITE MATTER is the INTERNAL CAPSULE.
The representation of the phylogenetically oldest part of the corpus striatum called the paleostriatum. It forms the smaller, more medial part of the lentiform nucleus.
A retrogressive pathological change in the retina, focal or generalized, caused by genetic defects, inflammation, trauma, vascular disease, or aging. Degeneration affecting predominantly the macula lutea of the retina is MACULAR DEGENERATION. (Newell, Ophthalmology: Principles and Concepts, 7th ed, p304)
Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.
A subfamily of G-PROTEIN-COUPLED RECEPTORS that bind the neurotransmitter DOPAMINE and modulate its effects. D1-class receptor genes lack INTRONS, and the receptors stimulate ADENYLYL CYCLASES.
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.
A phosphoprotein that was initially identified as a major target of DOPAMINE activated ADENYLYL CYCLASE in the CORPUS STRIATUM. It regulates the activities of PROTEIN PHOSPHATASE-1 and PROTEIN KINASE A, and it is a key mediator of the biochemical, electrophysiological, transcriptional, and behavioral effects of DOPAMINE.
The phylogenetically newer part of the CORPUS STRIATUM consisting of the CAUDATE NUCLEUS and PUTAMEN. It is often called simply the striatum.
A subfamily of G-PROTEIN-COUPLED RECEPTORS that bind the neurotransmitter DOPAMINE and modulate its effects. D2-class receptor genes contain INTRONS, and the receptors inhibit ADENYLYL CYCLASES.
A neurotransmitter analogue that depletes noradrenergic stores in nerve endings and induces a reduction of dopamine levels in the brain. Its mechanism of action is related to the production of cytolytic free-radicals.
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.
Degeneration of distal aspects of a nerve axon following injury to the cell body or proximal portion of the axon. The process is characterized by fragmentation of the axon and its MYELIN SHEATH.
Neural tracts connecting one part of the nervous system with another.
Compounds with BENZENE fused to AZEPINES.
Any drugs that are used for their effects on dopamine receptors, on the life cycle of dopamine, or on the survival of dopaminergic neurons.
Large subcortical nuclear masses derived from the telencephalon and located in the basal regions of the cerebral hemispheres.
The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.
An eleven-amino acid neurotransmitter that appears in both the central and peripheral nervous systems. It is involved in transmission of PAIN, causes rapid contractions of the gastrointestinal smooth muscle, and modulates inflammatory and immune responses.
Drugs that bind to and activate dopamine receptors.
One of the catecholamine NEUROTRANSMITTERS in the brain. It is derived from TYROSINE and is the precursor to NOREPINEPHRINE and EPINEPHRINE. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of receptors (RECEPTORS, DOPAMINE) mediate its action.
A class of opioid peptides including dynorphin A, dynorphin B, and smaller fragments of these peptides. Dynorphins prefer kappa-opioid receptors (RECEPTORS, OPIOID, KAPPA) and have been shown to play a role as central nervous system transmitters.
A subclass of LIM domain proteins that include an additional centrally-located homeodomain region that binds AT-rich sites on DNA. Many LIM-homeodomain proteins play a role as transcriptional regulators that direct cell fate.
Degenerative changes in the INTERVERTEBRAL DISC due to aging or structural damage, especially to the vertebral end-plates.
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.
Elongated gray mass of the neostriatum located adjacent to the lateral ventricle of the brain.
One of the three major families of endogenous opioid peptides. The enkephalins are pentapeptides that are widespread in the central and peripheral nervous systems and in the adrenal medulla.
Surface ligands that mediate cell-to-cell adhesion and function in the assembly and interconnection of the vertebrate nervous system. These molecules promote cell adhesion via a homophilic mechanism. These are not to be confused with NEURAL CELL ADHESION MOLECULES, now known to be expressed in a variety of tissues and cell types in addition to nervous tissue.
The making of a radiograph of an object or tissue by recording on a photographic plate the radiation emitted by radioactive material within the object. (Dorland, 27th ed)
A medical specialty concerned with the use of physical agents, mechanical apparatus, and manipulation in rehabilitating physically diseased or injured patients.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
Treatment for individuals with speech defects and disorders that involves counseling and use of various exercises and aids to help the development of new speech habits.
Skilled treatment that helps individuals achieve independence in all facets of their lives. It assists in the development of skills needed for independent living.
Acquired or developmental conditions marked by an impaired ability to comprehend or generate spoken forms of language.
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)

Inhibition of calpains prevents neuronal and behavioral deficits in an MPTP mouse model of Parkinson's disease. (1/6)

The molecular mechanisms mediating degeneration of midbrain dopamine neurons in Parkinson's disease (PD) are poorly understood. Here, we provide evidence to support a role for the involvement of the calcium-dependent proteases, calpains, in the loss of dopamine neurons in a mouse model of PD. We show that administration of N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) evokes an increase in calpain-mediated proteolysis in nigral dopamine neurons in vivo. Inhibition of calpain proteolysis using either a calpain inhibitor (MDL-28170) or adenovirus-mediated overexpression of the endogenous calpain inhibitor protein, calpastatin, significantly attenuated MPTP-induced loss of nigral dopamine neurons. Commensurate with this neuroprotection, MPTP-induced locomotor deficits were abolished, and markers of striatal postsynaptic activity were normalized in calpain inhibitor-treated mice. However, behavioral improvements in MPTP-treated, calpain inhibited mice did not correlate with restored levels of striatal dopamine. These results suggest that protection against nigral neuron degeneration in PD may be sufficient to facilitate normalized locomotor activity without necessitating striatal reinnervation. Immunohistochemical analyses of postmortem midbrain tissues from human PD cases also displayed evidence of increased calpain-related proteolytic activity that was not evident in age-matched control subjects. Taken together, our findings provide a potentially novel correlation between calpain proteolytic activity in an MPTP model of PD and the etiology of neuronal loss in PD in humans.  (+info)

The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations. (2/6)

Multiple system atrophy (MSA) has varying clinical (MSA-P versus MSA-C) and pathological [striatonigral degeneration (SND) versus olivopontocerebellar atrophy (OPCA)] phenotypes. To investigate the spectrum of clinicopathological correlations, we performed a semi-quantitative pathological analysis of 100 MSA cases with well-characterized clinical phenotypes. In 24 areas, chosen from both the striatonigral (StrN) and olivopontocerebellar (OPC) regions, the severity of neuronal cell loss and gliosis as well as the frequency of glial (oligodendroglial) cytoplasmic inclusions (GCIs) and neuronal cytoplasmic inclusions (NCIs) were determined. Clinical information was abstracted from the patients' medical records, and the severity of bradykinesia in the first year of disease onset and in the final stages of disease was graded retrospectively. The degree of levodopa responsiveness and the presence or absence of cerebellar ataxia and autonomic symptoms were also recorded. We report that 34% of the cases were SND- and 17% were OPCA-predominant, while the remainder (49%) had equivalent SND and OPCA pathology. We found a significant correlation between the frequency of GCIs and the severity of neuronal cell loss, and between these pathological changes and disease duration. Our data also suggest that GCIs may have more influence on the OPC than on the StrN pathology. Moreover, we raise the possibility that a rapid process of neuronal cell loss, which is independent of the accumulation of GCIs, occurs in the StrN region in MSA. There was no difference in the frequency of NCIs in the putamen, pontine nucleus and inferior olivary nucleus between the SND and OPCA subtypes of MSA, confirming that this pathological abnormality is not associated with a particular subtype of the disease. In the current large post-mortem series, 10% of the cases had associated Lewy body pathology, suggesting that this is not a primary process in MSA. As might be expected, there was a significant difference in the severity of bradykinesia and the presence of cerebellar signs between the pathological phenotypes: the SND phenotype demonstrates the most severe bradykinesia and the OPCA phenotype the more frequent occurrence of cerebellar signs, confirming that the clinical phenotype is dependent on the distribution of pathology within the basal ganglia and cerebellum. Putaminal involvement correlated with a poor levodopa response in MSA. Our finding that relatively mild involvement of the substantia nigra is associated clinically with manifest parkinsonism, while more advanced cerebellar pathology is required for ataxia, may explain why the parkinsonian presentation is predominant over ataxia in MSA.  (+info)

Clinical-radiological correlation. Report of two cases. (3/6)

Multiple system atrophy (MSA) is a sporadic, neurodegenerative disorder, clinically characterized by parkinsonian, autonomic, cerebellar and pyramidal signs. We describe two patients showing different presentations of the same disease. The patient on case 1 presents features of MSA-C or olivopontocerebellar atrophy with the pontine "cross sign" on brain MRI. The second case reports a patient presenting MSA-P or striatonigral degeneration and the brain MRI shows lenticular nucleus sign alteration. We think that brain MRI might increase the accuracy diagnostic of MSA.  (+info)

Grooved pegboard test as a biomarker of nigrostriatal denervation in Parkinson's disease. (4/6)

Recent pharmacotherapy trials in Parkinson's disease (PD) using dopaminergic neuroimaging as outcome parameter failed to show significant relationships between imaging and clinical results. One possible explanation is that there is a non-linear relationship between striatal denervation and motor performance reflecting a statistical "floor" effect in the imaging data with advanced disease. Both the motor manifestations and the striatal dopamine denervation of idiopathic PD, however, are typically asymmetric and more meaningful associations may be found by comparing data from the least denervated striatum with motor performance in the corresponding body side. PD patients (n=28) underwent [11C]beta-CFT dopamine transporter (DAT) positron emission tomography (PET) and grooved pegboard testing. Voxel-based analysis of DAT PET and bimanual pegboard scores demonstrated significant correlation clusters within the bilateral striata (P<0.001). However, findings were most prominent in the least denervated striatum. There was a significant inverse correlation between pegboard scores of the least affected arm and DAT binding of the least denervated striatum (Rs=-0.69, P<0.0001) but no significant correlation between pegboard scores of the clinically most affected arm and DAT binding of the most denervated striatum (Rs=-0.15, ns). These data indicate that the robustness of the grooved pegboard test as a biomarker for nigrostriatal denervation in PD mainly reflects the relationship between test performance of the clinically least affected limb and the least denervated striatum. These findings indicate that there is both a statistical "floor" and "ceiling" effect for the most affected striatal and body sides that must be considered when employing imaging as an outcome measure in clinical trials in PD.  (+info)

Recovery of hypothalamic tuberoinfundibular dopamine neurons from acute toxicant exposure is dependent upon protein synthesis and associated with an increase in parkin and ubiquitin carboxy-terminal hydrolase-L1 expression. (5/6)

 (+info)

Sirtuin 2 (SIRT2) enhances 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced nigrostriatal damage via deacetylating forkhead box O3a (Foxo3a) and activating Bim protein. (6/6)

Sirtuins are NAD-dependent protein deacetylases that were shown to have beneficial effects against age-related diseases. SIRT2 is a strong deacetylase that is highly expressed in brain. It has been associated with neurodegenerative diseases. 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) is a dopaminergic neurotoxin that replicates most of the clinical features of Parkinson disease (PD) and produces a reliable and reproducible lesion of the nigrostriatal dopaminergic pathway and neurodegeneration after its systemic administration. Chronic administration of MPTP induces lesion via apoptosis. We show here that SIRT2 deacetylates Foxo3a, increases RNA and protein levels of Bim, and as a result, enhances apoptosis in the MPTP model of PD. We also show that neurodegeneration induced by chronic MPTP regimen is prevented by genetic deletion of SIRT2 in mouse. Deletion of SIRT2 leads to the reduction of apoptosis due to an increase in acetylation of Foxo3a and a decrease in Bim levels. We demonstrate that SIRT2 deacetylates Foxo3a, activates Bim, and induces apoptosis only in 1-methyl-4-phenylpyridinium-treated cells. Therefore, designing SIRT2 inhibitors might be helpful to develop effective treatments for PD.  (+info)

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.

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.

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.

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.

The corpus striatum is a part of the brain that plays a crucial role in movement, learning, and cognition. It consists of two structures called the caudate nucleus and the putamen, which are surrounded by the external and internal segments of the globus pallidus. Together, these structures form the basal ganglia, a group of interconnected neurons that help regulate voluntary movement.

The corpus striatum receives input from various parts of the brain, including the cerebral cortex, thalamus, and other brainstem nuclei. It processes this information and sends output to the globus pallidus and substantia nigra, which then project to the thalamus and back to the cerebral cortex. This feedback loop helps coordinate and fine-tune movements, allowing for smooth and coordinated actions.

Damage to the corpus striatum can result in movement disorders such as Parkinson's disease, Huntington's disease, and dystonia. These conditions are characterized by abnormal involuntary movements, muscle stiffness, and difficulty initiating or controlling voluntary movements.

The Globus Pallidus is a structure in the brain that is part of the basal ganglia, a group of nuclei associated with movement control and other functions. It has two main subdivisions: the external (GPe) and internal (GPi) segments. The GPe receives input from the striatum and sends inhibitory projections to the subthalamic nucleus, while the GPi sends inhibitory projections to the thalamus, which in turn projects to the cerebral cortex. These connections allow for the regulation of motor activity, with abnormal functioning of the Globus Pallidus being implicated in various movement disorders such as Parkinson's disease and Huntington's disease.

Retinal degeneration is a broad term that refers to the progressive loss of photoreceptor cells (rods and cones) in the retina, which are responsible for converting light into electrical signals that are sent to the brain. This process can lead to vision loss or blindness. There are many different types of retinal degeneration, including age-related macular degeneration, retinitis pigmentosa, and Stargardt's disease, among others. These conditions can have varying causes, such as genetic mutations, environmental factors, or a combination of both. Treatment options vary depending on the specific type and progression of the condition.

Macular degeneration, also known as age-related macular degeneration (AMD), is a medical condition that affects the central part of the retina, called the macula. The macula is responsible for sharp, detailed vision, which is necessary for activities such as reading, driving, and recognizing faces.

In AMD, there is a breakdown or deterioration of the macula, leading to gradual loss of central vision. There are two main types of AMD: dry (atrophic) and wet (exudative). Dry AMD is more common and progresses more slowly, while wet AMD is less common but can cause rapid and severe vision loss if left untreated.

The exact causes of AMD are not fully understood, but risk factors include age, smoking, family history, high blood pressure, obesity, and exposure to sunlight. While there is no cure for AMD, treatments such as vitamin supplements, laser therapy, and medication injections can help slow its progression and reduce the risk of vision loss.

Dopamine D1 receptors are a type of G protein-coupled receptor that bind to the neurotransmitter dopamine. They are classified as D1-like receptors, along with D5 receptors, and are activated by dopamine through a stimulatory G protein (Gs).

D1 receptors are widely expressed in the central nervous system, including the striatum, prefrontal cortex, hippocampus, and amygdala. They play important roles in various physiological functions, such as movement control, motivation, reward processing, working memory, and cognition.

Activation of D1 receptors leads to increased levels of intracellular cyclic adenosine monophosphate (cAMP) and activation of protein kinase A (PKA), which in turn modulate the activity of various downstream signaling pathways. Dysregulation of dopamine D1 receptor function has been implicated in several neurological and psychiatric disorders, including Parkinson's disease, schizophrenia, attention deficit hyperactivity disorder (ADHD), and drug addiction.

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.

Dopamine and cAMP-regulated phosphoprotein 32 (DARPP-32) is a protein that plays a crucial role in the regulation of signal transduction pathways in the brain. It is primarily expressed in neurons of the striatum, a region involved in movement control, motivation, and reward processing.

DARPP-32 acts as a molecular switch in response to various neurotransmitters, including dopamine and glutamate. When phosphorylated by protein kinase A (PKA), DARPP-32 inhibits protein phosphatase-1 (PP-1), thereby enhancing the effects of PKA and promoting long-term changes in synaptic plasticity. Conversely, when phosphorylated by other kinases such as cyclin-dependent kinase 5 (Cdk5) or protein kinase C (PKC), DARPP-32 inhibits PKA, counteracting its effects.

Dysregulation of DARPP-32 has been implicated in several neurological and psychiatric disorders, including drug addiction, Parkinson's disease, and schizophrenia. Therefore, understanding the molecular mechanisms underlying DARPP-32 function is essential for developing novel therapeutic strategies to treat these conditions.

The neostriatum is a component of the basal ganglia, a group of subcortical nuclei in the brain that are involved in motor control, procedural learning, and other cognitive functions. It is composed primarily of two types of neurons: medium spiny neurons and aspiny interneurons. The neostriatum receives input from various regions of the cerebral cortex and projects to other parts of the basal ganglia, forming an important part of the cortico-basal ganglia-thalamo-cortical loop.

In medical terminology, the neostriatum is often used interchangeably with the term "striatum," although some sources reserve the term "neostriatum" for the caudate nucleus and putamen specifically, while using "striatum" to refer to the entire structure including the ventral striatum (also known as the nucleus accumbens).

Damage to the neostriatum has been implicated in various neurological conditions, such as Huntington's disease and Parkinson's disease.

Dopamine D2 receptor is a type of metabotropic G protein-coupled receptor that binds to the neurotransmitter dopamine. It is one of five subtypes of dopamine receptors (D1-D5) and is encoded by the gene DRD2. The activation of D2 receptors leads to a decrease in the activity of adenylyl cyclase, which results in reduced levels of cAMP and modulation of ion channels.

D2 receptors are widely distributed throughout the central nervous system (CNS) and play important roles in various physiological functions, including motor control, reward processing, emotion regulation, and cognition. They are also involved in several neurological and psychiatric disorders, such as Parkinson's disease, schizophrenia, drug addiction, and Tourette syndrome.

D2 receptors have two main subtypes: D2 short (D2S) and D2 long (D2L). The D2S subtype is primarily located in the presynaptic terminals and functions as an autoreceptor that regulates dopamine release, while the D2L subtype is mainly found in the postsynaptic neurons and modulates intracellular signaling pathways.

Antipsychotic drugs, which are used to treat schizophrenia and other psychiatric disorders, work by blocking D2 receptors. However, excessive blockade of these receptors can lead to side effects such as extrapyramidal symptoms (EPS), tardive dyskinesia, and hyperprolactinemia. Therefore, the development of drugs that selectively target specific subtypes of dopamine receptors is an active area of research in the field of neuropsychopharmacology.

Oxidopamine is not a recognized medical term or a medication commonly used in clinical practice. However, it is a chemical compound that is often used in scientific research, particularly in the field of neuroscience.

Oxidopamine is a synthetic catecholamine that can be selectively taken up by dopaminergic neurons and subsequently undergo oxidation, leading to the production of reactive oxygen species. This property makes it a useful tool for studying the effects of oxidative stress on dopaminergic neurons in models of Parkinson's disease and other neurological disorders.

In summary, while not a medical definition per se, oxidopamine is a chemical compound used in research to study the effects of oxidative stress on dopaminergic neurons.

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.

Wallerian degeneration is a process that occurs following damage to the axons of neurons (nerve cells). After an axon is severed or traumatically injured, it undergoes a series of changes including fragmentation and removal of the distal segment of the axon, which is the part that is separated from the cell body. This process is named after Augustus Waller, who first described it in 1850.

The degenerative changes in the distal axon are characterized by the breakdown of the axonal cytoskeleton, the loss of myelin sheath (the fatty insulating material that surrounds and protects the axon), and the infiltration of macrophages to clear away the debris. These events lead to the degeneration of the distal axon segment, which is necessary for successful regeneration of the injured nerve.

Wallerian degeneration is a crucial process in the nervous system's response to injury, as it enables the regrowth of axons and the reestablishment of connections between neurons. However, if the regenerative capacity of the neuron is insufficient or the environment is not conducive to growth, functional recovery may be impaired, leading to long-term neurological deficits.

Neural pathways, also known as nerve tracts or fasciculi, refer to the highly organized and specialized routes through which nerve impulses travel within the nervous system. These pathways are formed by groups of neurons (nerve cells) that are connected in a series, creating a continuous communication network for electrical signals to transmit information between different regions of the brain, spinal cord, and peripheral nerves.

Neural pathways can be classified into two main types: sensory (afferent) and motor (efferent). Sensory neural pathways carry sensory information from various receptors in the body (such as those for touch, temperature, pain, and vision) to the brain for processing. Motor neural pathways, on the other hand, transmit signals from the brain to the muscles and glands, controlling movements and other effector functions.

The formation of these neural pathways is crucial for normal nervous system function, as it enables efficient communication between different parts of the body and allows for complex behaviors, cognitive processes, and adaptive responses to internal and external stimuli.

Benzazepines are a class of heterocyclic compounds that contain a benzene fused to a diazepine ring. In the context of pharmaceuticals, benzazepines refer to a group of drugs with various therapeutic uses, such as antipsychotics and antidepressants. Some examples of benzazepine-derived drugs include clozapine, olanzapine, and loxoprofen. These drugs have complex mechanisms of action, often involving multiple receptor systems in the brain.

Dopamine agents are medications that act on dopamine receptors in the brain. Dopamine is a neurotransmitter, a chemical messenger that transmits signals in the brain and other areas of the body. It plays important roles in many functions, including movement, motivation, emotion, and cognition.

Dopamine agents can be classified into several categories based on their mechanism of action:

1. Dopamine agonists: These medications bind to dopamine receptors and mimic the effects of dopamine. They are used to treat conditions such as Parkinson's disease, restless legs syndrome, and certain types of dopamine-responsive dystonia. Examples include pramipexole, ropinirole, and rotigotine.
2. Dopamine precursors: These medications provide the building blocks for the body to produce dopamine. Levodopa is a commonly used dopamine precursor that is converted to dopamine in the brain. It is often used in combination with carbidopa, which helps to prevent levodopa from being broken down before it reaches the brain.
3. Dopamine antagonists: These medications block the action of dopamine at its receptors. They are used to treat conditions such as schizophrenia and certain types of nausea and vomiting. Examples include haloperidol, risperidone, and metoclopramide.
4. Dopamine reuptake inhibitors: These medications increase the amount of dopamine available in the synapse (the space between two neurons) by preventing its reuptake into the presynaptic neuron. They are used to treat conditions such as attention deficit hyperactivity disorder (ADHD) and depression. Examples include bupropion and nomifensine.
5. Dopamine release inhibitors: These medications prevent the release of dopamine from presynaptic neurons. They are used to treat conditions such as Tourette's syndrome and certain types of chronic pain. Examples include tetrabenazine and deutetrabenazine.

It is important to note that dopamine agents can have significant side effects, including addiction, movement disorders, and psychiatric symptoms. Therefore, they should be used under the close supervision of a healthcare provider.

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.

Neurons, also known as nerve cells or neurocytes, are specialized cells that constitute the basic unit of the nervous system. They are responsible for receiving, processing, and transmitting information and signals within the body. Neurons have three main parts: the dendrites, the cell body (soma), and the axon. The dendrites receive signals from other neurons or sensory receptors, while the axon transmits these signals to other neurons, muscles, or glands. The junction between two neurons is called a synapse, where neurotransmitters are released to transmit the signal across the gap (synaptic cleft) to the next neuron. Neurons vary in size, shape, and structure depending on their function and location within the nervous system.

Substance P is an undecapeptide neurotransmitter and neuromodulator, belonging to the tachykinin family of peptides. It is widely distributed in the central and peripheral nervous systems and is primarily found in sensory neurons. Substance P plays a crucial role in pain transmission, inflammation, and various autonomic functions. It exerts its effects by binding to neurokinin 1 (NK-1) receptors, which are expressed on the surface of target cells. Apart from nociception and inflammation, Substance P is also involved in regulating emotional behaviors, smooth muscle contraction, and fluid balance.

Dopamine agonists are a class of medications that mimic the action of dopamine, a neurotransmitter in the brain that regulates movement, emotion, motivation, and reinforcement of rewarding behaviors. These medications bind to dopamine receptors in the brain and activate them, leading to an increase in dopaminergic activity.

Dopamine agonists are used primarily to treat Parkinson's disease, a neurological disorder characterized by motor symptoms such as tremors, rigidity, bradykinesia (slowness of movement), and postural instability. By increasing dopaminergic activity in the brain, dopamine agonists can help alleviate some of these symptoms.

Examples of dopamine agonists include:

1. Pramipexole (Mirapex)
2. Ropinirole (Requip)
3. Rotigotine (Neupro)
4. Apomorphine (Apokyn)

Dopamine agonists may also be used off-label to treat other conditions, such as restless legs syndrome or certain types of dopamine-responsive dystonia. However, these medications can have significant side effects, including nausea, dizziness, orthostatic hypotension, compulsive behaviors (such as gambling, shopping, or sexual addiction), and hallucinations. Therefore, they should be used with caution and under the close supervision of a healthcare provider.

Dopamine is a type of neurotransmitter, which is a chemical messenger that transmits signals in the brain and nervous system. It plays several important roles in the body, including:

* Regulation of movement and coordination
* Modulation of mood and motivation
* Control of the reward and pleasure centers of the brain
* Regulation of muscle tone
* Involvement in memory and attention

Dopamine is produced in several areas of the brain, including the substantia nigra and the ventral tegmental area. It is released by neurons (nerve cells) and binds to specific receptors on other neurons, where it can either excite or inhibit their activity.

Abnormalities in dopamine signaling have been implicated in several neurological and psychiatric conditions, including Parkinson's disease, schizophrenia, and addiction.

Dynorphins are a type of opioid peptide that is naturally produced in the body. They bind to specific receptors in the brain, known as kappa-opioid receptors, and play a role in modulating pain perception, emotional response, and reward processing. Dynorphins are derived from a larger precursor protein called prodynorphin and are found throughout the nervous system, including in the spinal cord, brainstem, and limbic system. They have been implicated in various physiological processes, as well as in the development of certain neurological and psychiatric disorders, such as chronic pain, depression, and substance use disorders.

LIM-homeodomain proteins are a family of transcription factors that contain both LIM domains and homeodomains. LIM domains are cysteine-rich motifs that function in protein-protein interactions, often mediating the formation of multimeric complexes. Homeodomains are DNA-binding domains that recognize and bind to specific DNA sequences, thereby regulating gene transcription.

LIM-homeodomain proteins play important roles in various developmental processes, including cell fate determination, differentiation, and migration. They have been implicated in the regulation of muscle, nerve, and cardiovascular development, as well as in cancer and other diseases. Some examples of LIM-homeodomain proteins include LMX1A, LHX2, and ISL1.

These proteins are characterized by the presence of two LIM domains at the N-terminus and a homeodomain at the C-terminus. The LIM domains are involved in protein-protein interactions, while the homeodomain is responsible for DNA binding and transcriptional regulation. Some LIM-homeodomain proteins also contain other functional domains, such as zinc fingers or leucine zippers, which contribute to their diverse functions.

Overall, LIM-homeodomain proteins are important regulators of gene expression and play critical roles in various developmental and disease processes.

Intervertebral disc degeneration is a physiological and biochemical process that occurs in the spinal discs, which are located between each vertebra in the spine. These discs act as shock absorbers and allow for movement and flexibility of the spine.

The degenerative process involves changes in the structure and composition of the disc, including loss of water content, decreased production of proteoglycans (which help to maintain the disc's elasticity), and disorganization of the collagen fibers that make up the disc's outer layer (annulus fibrosus). These changes can lead to a decrease in the disc's height and mobility, as well as the development of tears or cracks in the annulus fibrosus.

In advanced stages of degeneration, the disc may herniate or bulge outward, causing pressure on nearby nerves and potentially leading to pain, numbness, tingling, or weakness in the affected area. It's worth noting that while intervertebral disc degeneration is a normal part of aging, certain factors such as injury, smoking, obesity, and repetitive stress can accelerate the process.

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.

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.

Enkephalins are naturally occurring opioid peptides that bind to opiate receptors in the brain and other organs, producing pain-relieving and other effects. They are derived from the precursor protein proenkephalin and consist of two main types: Leu-enkephalin and Met-enkephalin. Enkephalins play a role in pain modulation, stress response, mood regulation, and addictive behaviors. They are also involved in the body's reward system and have been implicated in various physiological processes such as respiration, gastrointestinal motility, and hormone release.

Cell adhesion molecules (CAMs) are a type of protein that mediates the attachment or binding of cells to their surrounding extracellular matrix or to other cells. Neuronal cell adhesion molecules (NCAMs) are a specific subtype of CAMs that are primarily expressed on neurons and play crucial roles in the development, maintenance, and function of the nervous system.

NCAMs are involved in various processes such as cell recognition, migration, differentiation, synaptic plasticity, and neural circuit formation. They can interact with other NCAMs or other types of CAMs to form homophilic or heterophilic bonds, respectively. The binding of NCAMs can activate intracellular signaling pathways that regulate various cellular responses.

NCAMs are classified into three major families based on their molecular structure: the immunoglobulin superfamily (Ig-CAMs), the cadherin family, and the integrin family. The Ig-CAMs include NCAM1 (also known as CD56), which is a glycoprotein with multiple extracellular Ig-like domains and intracellular signaling motifs. The cadherin family includes N-cadherin, which mediates calcium-dependent cell-cell adhesion. The integrin family includes integrins such as α5β1 and αVβ3, which mediate cell-matrix adhesion.

Abnormalities in NCAMs have been implicated in various neurological disorders, including schizophrenia, Alzheimer's disease, and autism spectrum disorder. Therefore, understanding the structure and function of NCAMs is essential for developing therapeutic strategies to treat these conditions.

Autoradiography is a medical imaging technique used to visualize and localize the distribution of radioactively labeled compounds within tissues or organisms. In this process, the subject is first exposed to a radioactive tracer that binds to specific molecules or structures of interest. The tissue is then placed in close contact with a radiation-sensitive film or detector, such as X-ray film or an imaging plate.

As the radioactive atoms decay, they emit particles (such as beta particles) that interact with the film or detector, causing chemical changes and leaving behind a visible image of the distribution of the labeled compound. The resulting autoradiogram provides information about the location, quantity, and sometimes even the identity of the molecules or structures that have taken up the radioactive tracer.

Autoradiography has been widely used in various fields of biology and medical research, including pharmacology, neuroscience, genetics, and cell biology, to study processes such as protein-DNA interactions, gene expression, drug metabolism, and neuronal connectivity. However, due to the use of radioactive materials and potential hazards associated with them, this technique has been gradually replaced by non-radioactive alternatives like fluorescence in situ hybridization (FISH) or immunofluorescence techniques.

Physical and Rehabilitation Medicine (PRM), also known as Physiatry, is a medical specialty that deals with the prevention, diagnosis, and treatment of patients with disabilities or functional limitations related to musculoskeletal, cardiovascular, pulmonary, neurologic, and other systems. The main goal of this discipline is to restore optimal function, reduce symptoms, and improve the overall quality of life for individuals who have experienced injuries, illnesses, or disabling conditions.

PRM physicians use a variety of techniques, including physical therapy, occupational therapy, speech-language pathology, assistive devices, medications, and various types of injections to manage pain and spasticity. They also perform electrodiagnostic studies to diagnose neuromuscular disorders and provide comprehensive rehabilitation plans tailored to each patient's unique needs and goals.

In addition to direct patient care, PRM specialists often work as part of multidisciplinary teams in hospitals, rehabilitation centers, and outpatient clinics, collaborating with other healthcare professionals such as nurses, therapists, psychologists, and social workers to provide coordinated, holistic care for patients.

An encyclopedia is a comprehensive reference work containing articles on various topics, usually arranged in alphabetical order. In the context of medicine, a medical encyclopedia is a collection of articles that provide information about a wide range of medical topics, including diseases and conditions, treatments, tests, procedures, and anatomy and physiology. Medical encyclopedias may be published in print or electronic formats and are often used as a starting point for researching medical topics. They can provide reliable and accurate information on medical subjects, making them useful resources for healthcare professionals, students, and patients alike. Some well-known examples of medical encyclopedias include the Merck Manual and the Stedman's Medical Dictionary.

Speech Therapy, also known as Speech-Language Pathology, is a medical field that focuses on the assessment, diagnosis, treatment, and prevention of communication and swallowing disorders in children and adults. These disorders may include speech sound production difficulties (articulation disorders or phonological processes disorders), language disorders (expressive and/or receptive language impairments), voice disorders, fluency disorders (stuttering), cognitive-communication disorders, and swallowing difficulties (dysphagia).

Speech therapists, who are also called speech-language pathologists (SLPs), work with clients to improve their communication abilities through various therapeutic techniques and exercises. They may also provide counseling and education to families and caregivers to help them support the client's communication development and management of the disorder.

Speech therapy services can be provided in a variety of settings, including hospitals, clinics, schools, private practices, and long-term care facilities. The specific goals and methods used in speech therapy will depend on the individual needs and abilities of each client.

Occupational therapy (OT) is a healthcare profession that aims to improve the daily living and functional abilities of individuals who have physical, sensory, or cognitive disabilities. OT focuses on helping people participate in the activities of everyday life, such as self-care tasks (e.g., dressing, grooming), productive tasks (e.g., work, school), and leisure activities (e.g., hobbies, sports).

Occupational therapists use a variety of interventions to achieve these goals, including:

1. Customized treatment plans that focus on the individual's specific needs and goals.
2. Adaptive equipment and assistive technology to help individuals perform activities more independently.
3. Education and training for individuals, families, and caregivers on how to use adaptive equipment and techniques.
4. Environmental modifications to make daily activities safer and more accessible.
5. Skill development and practice in areas such as fine motor coordination, cognitive skills, and sensory processing.

Occupational therapy can be provided in a variety of settings, including hospitals, rehabilitation centers, outpatient clinics, schools, and private homes. OT is often recommended for individuals who have experienced a stroke, brain injury, spinal cord injury, or other conditions that affect their ability to perform daily activities.

Speech disorders refer to a group of conditions in which a person has difficulty producing or articulating sounds, words, or sentences in a way that is understandable to others. These disorders can be caused by various factors such as developmental delays, neurological conditions, hearing loss, structural abnormalities, or emotional issues.

Speech disorders may include difficulties with:

* Articulation: the ability to produce sounds correctly and clearly.
* Phonology: the sound system of language, including the rules that govern how sounds are combined and used in words.
* Fluency: the smoothness and flow of speech, including issues such as stuttering or cluttering.
* Voice: the quality, pitch, and volume of the spoken voice.
* Resonance: the way sound is produced and carried through the vocal tract, which can affect the clarity and quality of speech.

Speech disorders can impact a person's ability to communicate effectively, leading to difficulties in social situations, academic performance, and even employment opportunities. Speech-language pathologists are trained to evaluate and treat speech disorders using various evidence-based techniques and interventions.

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.

EPB72 Striatal degeneration, autosomal dominant; 609161; PDE8B Striatonigral degeneration, infantile; 271930; NUP62 Stuve- ... CHST6 Macular degeneration, age-related, 11; 611953; CST3 Macular degeneration, age-related, 2; 153800; ABCA4 Macular ... ALX3 Frontotemporal lobar degeneration with ubiquitin-positive inclusions; 607485; GRN Frontotemporal lobar degeneration, ... degeneration, age-related, 3; 608895; FBLN5 Macular degeneration juvenile; 248200; CNGB3 Macular dystrophy, autosomal dominant ...
These include striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and Shy-Drager syndrome. A table ... It is sometimes termed striatonigral degeneration, a parkinsonian variant.[citation needed] MSA with cerebellar features (MSA-C ... striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome)". Journal of the Neurological Sciences. 94 (1 ... This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior ...
... characterized by neuronal degeneration in the striatum and substantia nigra was previously called striatonigral degeneration. ... Grosch J, Winkler J, Kohl Z (2016). "Early Degeneration of Both Dopaminergic and Serotonergic Axons - A Common Mechanism in ... the symptoms of nigral degeneration due to Parkinson's is a poignant example of the substantia nigra's influence on movement. ...
Here he founded the Australian Venom Research Unit, where he worked until 1999 when striatonigral degeneration, a condition ...
... disease Pantothenate kinase-associated neurodegeneration Ramsay Hunt syndrome Huntington's disease Striatonigral degeneration ...
... striatonigral degeneration MeSH C10.228.662.600 - parkinsonian disorders MeSH C10.228.662.600.200 - lewy body disease MeSH ... striatonigral degeneration MeSH C10.574.781.500 - lambert-eaton myasthenic syndrome MeSH C10.574.781.550 - limbic encephalitis ... striatonigral degeneration MeSH C10.228.140.079.737 - neuroleptic malignant syndrome MeSH C10.228.140.079.862 - parkinsonian ... paraneoplastic cerebellar degeneration MeSH C10.228.140.252.700 - spinocerebellar degenerations MeSH C10.228.140.252.700.150 - ...
Group B Strep throat Striatonigral degeneration infantile Strømme syndrome Strongyloidiasis Strudwick syndrome Strumpell- ... congenital Stuccokeratosis Sturge-Weber syndrome Stuve-Wiedemann dysplasia Stye Stupidity Subacute cerebellar degeneration ... X-linked Spondylometaphyseal dysplasia Spondyloperipheral dysplasia short ulna Spongiform encephalopathy Spongy degeneration of ... ataxia amyotrophy deafness Spinocerebellar ataxia dysmorphism Spinocerebellar atrophy type 3 Spinocerebellar degeneration ...
"Continuous intraventricular drug infusion for the in vivo study of transneuronal degeneration in the striatonigral system of ... Retrograde transneuronal degeneration is degeneration caused by loss of trophic support from the target. It occurs in ... It is often termed "dying forward," and is also referred to as trans-synaptic degeneration. Anterograde degeneration can occur ... There are varying degrees of degeneration. In mild degeneration the cytoplasmic areas shrinks and increases in density and ...
Degeneration of dopaminergic neurons in the SNc is one of the main pathological features of Parkinson's disease, leading to a ... Because the striatonigral / striatoentopeduncular and nigrothalamic pathways are inhibitory, activation of the direct pathway ... Degeneration of dopamine producing neurons in the substantia nigra pars compacta and the putamen-caudate complex leads to ...
... and sporadic olivopontocerebellar degeneration, which is characterized primarily ... ... Striatonigral degeneration is a sporadic, progressive neurodegenerative disorder that represents one manifestation of multiple ... encoded search term (Striatonigral Degeneration) and Striatonigral Degeneration What to Read Next on Medscape ... While symptoms of autonomic failure and cerebellar degeneration may be present in striatonigral degeneration, the predominant ...
EPB72 Striatal degeneration, autosomal dominant; 609161; PDE8B Striatonigral degeneration, infantile; 271930; NUP62 Stuve- ... CHST6 Macular degeneration, age-related, 11; 611953; CST3 Macular degeneration, age-related, 2; 153800; ABCA4 Macular ... ALX3 Frontotemporal lobar degeneration with ubiquitin-positive inclusions; 607485; GRN Frontotemporal lobar degeneration, ... degeneration, age-related, 3; 608895; FBLN5 Macular degeneration juvenile; 248200; CNGB3 Macular dystrophy, autosomal dominant ...
These include striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and Shy-Drager syndrome. A table ... It is sometimes termed striatonigral degeneration, a parkinsonian variant.[citation needed] MSA with cerebellar features (MSA-C ... striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome)". Journal of the Neurological Sciences. 94 (1 ... This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior ...
Striato-nigral degeneration: apropos of a clinical, therapeutic, and anatomic study of 2 cases. Rev Neurol (Paris)1976;132:137- ... "Pure" striatonigral degeneration and Parkinsons disease: a comparative clinical study. Mov Disord 1995;10:288-94. ... Striatonigral degeneration associated with olivopontocerebellar atrophy. Anatomo-clinical study of 3 cases. Nosologic ... 37 In MSA-P degeneration in the caudal and dorsal part of the putamen, which receives topographical projections from cortical ...
Multiple system atrophy (MSA; MSA-A, formerly Shy-Drager syndrome; MSA-P, striatonigral degeneration; MSA-C, ...
corticobasal degeneration. * frontotemporal lobar degeneration (FTLD) (not all are tau) *behavioral variant frontotemporal ... MSA-P (striatonigral degeneration)*putaminal rim sign. *olivopontocerebellar atrophy (MSA-C). * tauopathies * Alzheimer disease ... it is characterized by nigrostriatal dopaminergic degeneration leading to neuronal loss in the substantia nigra pars compacta ( ... rigidity and hypokinesia due to progressive degeneration of dopaminergic neurons in the substantia nigra. ...
corticobasal degeneration. * frontotemporal lobar degeneration (FTLD) (not all are tau) *behavioral variant frontotemporal ... MSA-P (striatonigral degeneration)*putaminal rim sign. *olivopontocerebellar atrophy (MSA-C). * tauopathies * Alzheimer disease ...
Corticobasal ganglionic degeneration or CBGD, is a kind of ... Striatonigral degeneration. Laboratory studies are also ... What is Corticobasal Degeneration Corticobasal degeneration, otherwise termed as, ... What is Corticobasal Degeneration. Corticobasal degeneration, otherwise termed as, Corticobasal ganglionic degeneration or CBGD ... Corticobasal degeneration is a rather uncommon disease and usually occurs in people in their advanced age, usually in the sixth ...
Progressive striatonigral degeneration in a transgenic mouse model of multiple system atrophy: translational implications for ... Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, ... The QTc prolongation in MSA patients may provide valuable information on the degeneration of cardioselective sympathetic and ... abnormalities observed in the QTc interval may reflect the degeneration of cardioselective sympathetic and parasympathetic ...
MSA includes conditions that were previously known individually as Shy-Drager syndrome, striatonigral degeneration and sporadic ... What causes the degeneration of brain tissue in CBD?. Unfortunately, the cause of CBD is entirely unknown. There is currently ... In AD there is wide spread cell loss in the cortex producing degeneration throughout the brain. If you look at a MRI or CT scan ... Cortico-basal-ganglionic Degeneration. This is a rare drug resistant parkinsonism. It is characterized by the marked asymmetry ...
1992) Striatal D2 receptor status in patients with Parkinsons disease, striatonigral degeneration, and progressive ... 1C). If the degeneration of neurons is random, the appearance of voids will be variable in time and space. Nonrandom mechanisms ... The hallmark of PD is progressive degeneration of dopamine (DA) neurons (Hornykiewicz, 2001; Dauer and Przedborski, 2003). ...
Calbindin D28K as a marker for the degeneration of the striatonigral pathway in Huntingtons disease. Brain Research. 525: 209- ... Biochemical and anatomical observations on the degeneration of peptide-containing primary afferent neurons after neonatal ... Localization of calpain immunoreactivity in senile plaques and in neurones undergoing neurofibrillary degeneration in ...
Atypical parkinsonism refers to a group of neurodegenerative... read more predominate in striatonigral degeneration. They ... It includes 3 disorders previously thought to be distinct: olivopontocerebellar atrophy, striatonigral degeneration, and Shy- ... Etiology of multiple system atrophy is unknown, but neuronal degeneration occurs in several areas of the brain; the area and ...
MSA was formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration. MSA shares many ...
18F-Dopa PET is able to discriminate PD from the striatonigral degeneration form of MSA in 70% of cases and from PSP in 90% of ... This is probably due to the degeneration of axons following the infarct with disconnection of remote structures.78,79 ... Francavilla TL, Miletich RS, Di Chiro G, et al. Positron emission tomography in the detection of malignant degeneration of low- ... Corticobasal degeneration (CBD) shows asymmetric and equivalent reduction in 18F-dopa Ki of the caudate and putamen. ...
... of Gait as a Complication of Pallidal Deep Brain Stimulation in DYT-KMT2B Patients with Evidence of Striatonigral Degeneration. ...
Striatonigral Degeneration, Autosomal Dominant. *Autosomal Dominant Striatonigral Degeneration. *Azorean Disease, Nervous ...
Disease table of STRIATONIGRAL DEGENERATION, INFANTILE OMIM ID: 271930. Gene-disease associations table Gene. Associated with ... STRIATONIGRAL DEGENERATION, INFANTILE in. Link to HGNC. Cytogenetic band. Number of associated diseases. Associated diseases. ...
"Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 14 (Suppl 1): 5-18. doi:10.3109/21678421.2013.778548. PMC 3779649 ... "Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 18 (sup1): 5-10. doi:10.1080/21678421.2017.1353101. PMID 28872907. ... Classical, or classic ALS, involves degeneration to both the upper motor neurons in the brain and the lower motor neurons in ... "Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 18 (3-4): 227-232. doi:10.1080/21678421.2016.1265565. PMC 5425622 ...
PARANEOPLASTIC CEREBELLAR DEGENERATION. DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR. DEGENERACION ESTRIATONIGRAL. STRIATONIGRAL ...
PARANEOPLASTIC CEREBELLAR DEGENERATION. DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR. DEGENERACION ESTRIATONIGRAL. STRIATONIGRAL ...
PARANEOPLASTIC CEREBELLAR DEGENERATION. DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR. DEGENERACION ESTRIATONIGRAL. STRIATONIGRAL ...
STRIATONIGRAL DEGENERATION DEGENERACION ESTRIATONIGRAL DEGENERAÇÃO ESTRIATONIGRAL SUBARACHNOID HEMORRHAGE, TRAUMATIC HEMORRAGIA ... PARANEOPLASTIC CEREBELLAR DEGENERATION DEGENERACION CEREBELAR PARANEOPLASICA DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR ...
STRIATONIGRAL DEGENERATION DEGENERACION ESTRIATONIGRAL DEGENERAÇÃO ESTRIATONIGRAL SUBARACHNOID HEMORRHAGE, TRAUMATIC HEMORRAGIA ... PARANEOPLASTIC CEREBELLAR DEGENERATION DEGENERACION CEREBELAR PARANEOPLASICA DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR ...
STRIATONIGRAL DEGENERATION DEGENERACION ESTRIATONIGRAL DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR PARANEOPLASTIC CEREBELLAR ...
PARANEOPLASTIC CEREBELLAR DEGENERATION. DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR. DEGENERACION ESTRIATONIGRAL. STRIATONIGRAL ...
STRIATONIGRAL DEGENERATION DEGENERACION ESTRIATONIGRAL DEGENERAÇÃO ESTRIATONIGRAL SUBARACHNOID HEMORRHAGE, TRAUMATIC HEMORRAGIA ... PARANEOPLASTIC CEREBELLAR DEGENERATION DEGENERACION CEREBELAR PARANEOPLASICA DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR ...
STRIATONIGRAL DEGENERATION DEGENERACION ESTRIATONIGRAL DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR PARANEOPLASTIC CEREBELLAR ...
STRIATONIGRAL DEGENERATION DEGENERACION ESTRIATONIGRAL DEGENERAÇÃO ESTRIATONIGRAL SUBARACHNOID HEMORRHAGE, TRAUMATIC HEMORRAGIA ... PARANEOPLASTIC CEREBELLAR DEGENERATION DEGENERACION CEREBELAR PARANEOPLASICA DEGENERAÇÃO PARANEOPLÁSICA CEREBELAR ...
  • Striatonigral degeneration is a sporadic, progressive neurodegenerative disorder that represents one manifestation of multiple system atrophy (MSA). (medscape.com)
  • Other manifestations of multiple system atrophy are Shy-Drager syndrome, in which autonomic failure predominates, and sporadic olivopontocerebellar degeneration, which is characterized primarily by cerebellar signs. (medscape.com)
  • Thirty years later, the first consensus statement on the diagnosis of multiple system atrophy, by Gilman et al, recommended that the term striatonigral degeneration be replaced with multiple system atrophy with predominantly parkinsonian features (MSA-P) and that the term sporadic olivopontocerebellar degeneration be replaced with multiple system atrophy with predominantly cerebellar features (MSA-C). (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 has been called many things, including Olivopontocerebellar Atrophy, Striatonigral Degeneration, Parkinson's Plus and Shy-Drager Syndrome. (fightparkinsons.org.au)
  • MSA was formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration. (middlesexhealth.org)
  • This condition is also known by the names like olivopontocerebellar atrophy or striatonigral degeneration. (naturalayurvedictreatment.com)
  • Corticobasal degeneration, otherwise termed as, Corticobasal ganglionic degeneration or CBGD, is a kind of neurodegenerative disorder concerning a certain protein, called tau. (allhealthsite.com)
  • As an added information, a group of physicians led by doctor Rebeiz in the late 1960s first observed the occurrence of corticobasal degeneration in three affected people exhibiting the hallmark characteristics of the disorder. (allhealthsite.com)
  • Corticobasal degeneration is a rather uncommon disease and usually occurs in people in their advanced age, usually in the sixth decade of life. (allhealthsite.com)
  • This multiplication in number is indicative of a more sensitive diagnosis or simply, corticobasal degeneration is more frequently occuring in the present days. (allhealthsite.com)
  • The particular gene of interest that has been the focus of research studies, associated with corticobasal degeneration is the gene in chromosome 17. (allhealthsite.com)
  • Corticobasal degeneration affects both the subcortical as well as the cortical parts of the human brain. (allhealthsite.com)
  • As mentioned above, corticobasal degeneration is characterized by an abnormality called tauopathy. (allhealthsite.com)
  • This distinction is the main factor that sets corticobasal degeneration apart from its similar diseases such as Alzheimer's disease and progressive supranuclear palsy. (allhealthsite.com)
  • Corticobasal degeneration may be categorized into two. (allhealthsite.com)
  • In addition there are four signs and symptoms (both motion and cognitive) that are major characteristics associated with corticobasal degeneration. (allhealthsite.com)
  • Alien hand syndrome is fairly common in people with corticobasal degeneration. (allhealthsite.com)
  • MSA includes conditions that were previously known individually as Shy-Drager syndrome, striatonigral degeneration and sporadic olivopontocerebellar atrophy. (parkinson.ca)
  • It includes 3 disorders previously thought to be distinct: olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager syndrome. (msdmanuals.com)
  • They consisted of 24 patients with olivopontocerebellar atrophy (OPCA), 25 with Shy-Drager syndrome (SDS) and 10 with striatonigral degeneration (SND). (go.jp)
  • While symptoms of autonomic failure and cerebellar degeneration may be present in striatonigral degeneration, the predominant finding is parkinsonism. (medscape.com)
  • In many cases, the disease process begins with 1 of the 3 presentations predominating (ie, parkinsonism, autonomic failure, cerebellar signs) and then later converges to include a combination of all 3 plus additional degeneration of the corticospinal system, including tract and motor neuron degeneration, as well as cognitive deterioration. (medscape.com)
  • Parkinson disease (PD) , also known as idiopathic parkinsonism , is a neurodegenerative disease and movement disorder characterized by resting tremor, rigidity and hypokinesia due to progressive degeneration of dopaminergic neurons in the substantia nigra . (radiopaedia.org)
  • Features of parkinsonism often without tremor are combined with varying degrees of autonomic failure, cerebellar involvement and pyramidal tract degeneration. (naturalayurvedictreatment.com)
  • This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum. (wikipedia.org)
  • The dopaminergic tract is predominantly affected in Parkinson disease, and histologically, it is characterized by nigrostriatal dopaminergic degeneration leading to neuronal loss in the substantia nigra pars compacta (SNpc), most conspicuous in the ventrolateral tier of neurons 11 . (radiopaedia.org)
  • Given that QT interval depends on sympathetic and parasympathetic activities, abnormalities observed in the QTc interval may reflect the degeneration of cardioselective sympathetic and parasympathetic neurons ( 12 ). (frontiersin.org)
  • Clinical diagnosis of exclusion based on progressive symptoms of upper and lower motor neuron degeneration in which no other explanation can be found. (wikipedia.org)
  • The primary cause of degeneration is still under debate- whether cell loss in MSA is secondary to disruptions in the oligo-myelin-axon complex [ 29 , 64 ], or if MSA is a primary neuron disease, with the secondary formation of GCIs following pathological accumulation of α-syn that is neuronal in origin [ 55 ]. (biomedcentral.com)
  • The damage is predominantly in the striatonigral and olivopontocerebellar regions of the brain. (targetwoman.com)
  • Muscle cell degeneration predominantly occurs in the striatonigral region leading to tremors, lack of balance and postural impairment. (targetwoman.com)
  • 2005). In addition, PET scans have clinical utility for discerning between AD and dementia caused by frontotemporal lobar degeneration (FTLD) (Foster et al. (cd31-signal.com)
  • There have also been occasional instances of frontotemporal lobar degeneration associated with MSA. (wikipedia.org)
  • 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)
  • This phenomenon is medically referred to as tauopathy that ultimately leads to the deterioration or the degeneration of the individual's brain. (allhealthsite.com)
  • Machado-Joseph State (MJD-III), often referred to as spinocerebellar ataxia kind of III, try a rare, inherited, ataxia (insufficient muscle control) affecting the fresh central nervous system and you will characterized by new slow degeneration from form of areas of the brain called the hindbrain. (sch.id)
  • Yoshida M, Sone M. [Mechanism of neuronal degeneration of multiple system atrophy]. (medscape.com)
  • Yoshida M. [Multiple system atrophy - synuclein and neuronal degeneration]. (medscape.com)
  • MSA was formerly known as Shy-Drager syndrome, olivopontocerebellar atrophy (OCPA), or striatonigral degeneration. (nih.gov)
  • 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)
  • It includes 3 disorders previously thought to be distinct: olivopontocerebellar atrophy, striatonigral degeneration, and Shy-Drager syndrome. (msdmanuals.com)
  • Spinal muscular atrophy (SMA) is characterized by muscle weakness and atrophy resulting from progressive degeneration and irreversible loss of the anterior horn cells in the spinal cord (i.e., lower motor neurons) and the brain stem nuclei. (beds.ac.uk)
  • This condition is also known by the names like olivopontocerebellar atrophy or striatonigral degeneration. (naturalayurvedictreatment.com)
  • Thus, this result has implications for the choice of model and interpretation of studies used to investigate potential cannabinoid-based therapies for Parkinson's disease as well as striatonigral diseases such as Huntington's disease and Multiple Systems Atrophy. (thctotalhealthcare.com)
  • There have also been occasional instances of frontotemporal lobar degeneration associated with MSA. (wikipedia.org)
  • Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. (elsevierpure.com)
  • Comprises of several syndromes of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus with characteristics of developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. (cdc.gov)
  • Cardiac sympathetic impairment parallels nigrostriatal degeneration in Probable Dementia with Lewy Bodies. (medscape.com)
  • The goal of this review was to examine whether chronic Mn exposure produces dopamine neuron degeneration and PD or whether it has a distinct neuropathology and clinical presentation. (nih.gov)
  • Glial cytoplasmic inclusions (GCIs) and neuronal multisystem degeneration are the pathologic hallmarks of this clinically variable disorder (see the image below). (medscape.com)
  • Special attention is given to the first accurate description of striatonigral degeneration by Hans Joachim Scherer, his personal and scientific trajectory being clarified. (nih.gov)
  • These disorders have complex clinical presentations that reflect degeneration in various neuronal systems. (medscape.com)