MOTOR NEURONS in the anterior (ventral) horn of the SPINAL CORD which project to SKELETAL MUSCLES.
A group of recessively inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)
A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset SPINAL MUSCULAR ATROPHIES OF CHILDHOOD, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089)
An inhibitor of apoptosis protein that was initially identified during analysis of CHROMOSOME DELETIONS associated with SPINAL MUSCULAR ATROPHY. Naip contains a nucleotide binding oligomerization domain and a carboxy-terminal LEUCINE rich repeat.
A complex of proteins that assemble the SNRNP CORE PROTEINS into a core structure that surrounds a highly conserved RNA sequence found in SMALL NUCLEAR RNA. They are found localized in the GEMINI OF COILED BODIES and in the CYTOPLASM. The SMN complex is named after the Survival of Motor Neuron Complex Protein 1, which is a critical component of the complex.
A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.
A SMN complex protein that is essential for the function of the SMN protein complex. In humans the protein is encoded by a single gene found near the inversion telomere of a large inverted region of CHROMOSOME 5. Mutations in the gene coding for survival of motor neuron 1 protein may result in SPINAL MUSCULAR ATROPHIES OF CHILDHOOD.
Pathologic conditions which feature SPINAL CORD damage or dysfunction, including disorders involving the meninges and perimeningeal spaces surrounding the spinal cord. Traumatic injuries, vascular diseases, infections, and inflammatory/autoimmune processes may affect the spinal cord.
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.
Neurons which activate MUSCLE CELLS.
Recording of the changes in electric potential of muscle by means of surface or needle electrodes.
Horns are abnormal growths of keratin that can develop on the skull or other parts of the body, often associated with certain medical conditions such as chromosomal abnormalities or infections.
Neurons in the SPINAL CORD DORSAL HORN whose cell bodies and processes are confined entirely to the CENTRAL NERVOUS SYSTEM. They receive collateral or direct terminations of dorsal root fibers. They send their axons either directly to ANTERIOR HORN CELLS or to the WHITE MATTER ascending and descending longitudinal fibers.
The interarticular fibrocartilages of the superior surface of the tibia.
Persistent flexure or contracture of a joint.
A general term most often used to describe severe or complete loss of muscle strength due to motor system disease from the level of the cerebral cortex to the muscle fiber. This term may also occasionally refer to a loss of sensory function. (From Adams et al., Principles of Neurology, 6th ed, p45)
A general term encompassing lower MOTOR NEURON DISEASE; PERIPHERAL NERVOUS SYSTEM DISEASES; and certain MUSCULAR DISEASES. Manifestations include MUSCLE WEAKNESS; FASCICULATION; muscle ATROPHY; SPASM; MYOKYMIA; MUSCLE HYPERTONIA, myalgias, and MUSCLE HYPOTONIA.
Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)
A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)
Paired bundles of NERVE FIBERS entering and leaving the SPINAL CORD at each segment. The dorsal and ventral nerve roots join to form the mixed segmental spinal nerves. The dorsal roots are generally afferent, formed by the central projections of the spinal (dorsal root) ganglia sensory cells, and the ventral roots are efferent, comprising the axons of spinal motor and PREGANGLIONIC AUTONOMIC FIBERS.
The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.

Neuromyotonia: an unusual presentation of intrathoracic malignancy. (1/130)

A 48 year old woman is described who presented with increasing muscular rigidity and who was found to have a mediastinal tumour. Electrophysiological studies revealed that the muscular stiffness resulted from very high frequency motor unit activity which outlasted voluntary effort, and which was abolished by nerve block. The abnormal activity may have arisen at the anterior horn cell level. Marked improvement followed the administration of diphenylhydantoin.  (+info)

Rapid onset transverse myelitis in adolescence: implications for pathogenesis and prognosis. (2/130)

Five adolescents with transverse myelitis were reviewed. All presented with a rapid onset paralysis of the lower limbs and impairment of bladder control. The maximum disability developed between 10 minutes and six hours. There was no history of trauma, asthma, or prodromal illness. Investigations failed to demonstrate a vascular cause. Extensive spinal cord abnormalities were observed on magnetic resonance imaging. Electrophysiological investigations, performed in four cases, were all consistent with anterior horn cell damage. In all five adolescents there was poor recovery. The underlying pathogenesis of this rapid onset condition remains a subject of debate. Similarities with both transverse myelitis and fibrocartilaginous emboli are evident, widening the spectrum of conditions within the transverse myelitis umbrella. These observations suggest that in rapid onset "transverse myelitis" the combination of extensive hyperintensity on spinal cord neuroimaging with electrophysiological evidence of anterior horn cell involvement might have adverse prognostic significance.  (+info)

Age-related atrophy of motor axons in mice deficient in the mid-sized neurofilament subunit. (3/130)

Neurofilaments are central determinants of the diameter of myelinated axons. It is less clear whether neurofilaments serve other functional roles such as maintaining the structural integrity of axons over time. Here we show that an age-dependent axonal atrophy develops in the lumbar ventral roots of mice with a null mutation in the mid-sized neurofilament subunit (NF-M) but not in animals with a null mutation in the heavy neurofilament subunit (NF-H). Mice with null mutations in both genes develop atrophy in ventral and dorsal roots as well as a hind limb paralysis with aging. The atrophic process is not accompanied by significant axonal loss or anterior horn cell pathology. In the NF-M-null mutant atrophic ventral root, axons show an age-related depletion of neurofilaments and an increased ratio of microtubules/neurofilaments. By contrast, the preserved dorsal root axons of NF-M-null mutant animals do not show a similar depletion of neurofilaments. Thus, the lack of an NF-M subunit renders some axons selectively vulnerable to an age-dependent atrophic process. These studies argue that neurofilaments are necessary for the structural maintenance of some populations of axons during aging and that the NF-M subunit is especially critical.  (+info)

Norepinephrine facilitates inhibitory transmission in substantia gelatinosa of adult rat spinal cord (part 2): effects on somatodendritic sites of GABAergic neurons. (4/130)

BACKGROUND: It has been reported previously that norepinephrine, when applied to the spinal cord dorsal horn, excites a subpopulation of dorsal horn neurons, presumably inhibitory interneurons. In the current study, the authors tested whether norepinephrine could activate inhibitory interneurons, specifically those that are "GABAergic." METHODS: A transverse slice was obtained from a segment of the lumbar spinal cord isolated from adult male Sprague-Dawley rats. Whole-cell patch-clamp recordings were made from substantia gelatinosa neurons using the blind patch-clamp technique. The effects of norepinephrine on spontaneous GABAergic inhibitory postsynaptic currents were studied. RESULTS: In the majority of substantia gelatinosa neurons tested, norepinephrine (10-60 microM) significantly increased both the frequency and the amplitude of GABAergic inhibitory postsynaptic currents. These increases were blocked by tetrodotoxin (1 microM). The effects of norepinephrine were mimicked by the alpha1-receptor agonist phenylephrine (10-80 microM) and inhibited by the alpha1-receptor-antagonist WB-4101 (0.5 microM). Primary-afferent-evoked polysynaptic excitatory postsynaptic potentials or excitatory postsynaptic currents in wide-dynamic-range neurons of the deep dorsal horn were also attenuated by phenylephrine (40 microM). CONCLUSION: The observations suggest that GABAergic interneurons possess somatodendritic alpha1 receptors, and activation of these receptors excites inhibitory interneurons. The alpha1 actions reported herein may contribute to the analgesic action of intrathecally administered phenylephrine.  (+info)

Long-term gene expression in the anterior horn motor neurons after intramuscular inoculation of a live herpes simplex virus vector. (5/130)

To clarify the feasibility of the herpes simplex virus (HSV) vector in expressing the foreign gene in the motor neuron, we inoculated a live attenuated HSV expressing beta-galactosidase (beta-gal) activity under a latency-associated transcript promoter in the right gastrocnemius muscle of rats. Expression of beta-gal activity was observed 5 days after inoculation in the bilateral anterior horn cells of the spinal cord that innervates the inoculation muscle. However, the spread of beta-gal activity was not observed in the inoculation muscle. Without significant pathological changes, the spread of beta-gal-expressing neurons was observed in the lumbosacral spinal cord until 14 days after inoculation with staining concentrated in the anterior horn cells. Ninety percent of the anterior horn motor neurons expressed beta-gal activity with expression continuing to at least 182 days after inoculation. Thus beta-gal activity was expressed in the bilateral anterior horn cells at the lumbosacral spinal cord that innervates the inoculated muscle for a long time, possibly a life-long period. This indicates that this recombinant HSV vector system to motor neurons may further improve the understanding and treatment of neurological diseases in motor neurons of the spinal cord.  (+info)

Depression of group Ia monosynaptic EPSPs in cat hindlimb motoneurones during fictive locomotion. (6/130)

The effects of fictive locomotion on monosynaptic EPSPs recorded in motoneurones and extracellular field potentials recorded in the ventral horn were examined during brainstem-evoked fictive locomotion in decerebrate cats. Composite homonymous and heteronymous EPSPs and field potentials were evoked by group I intensity (<= 2T) stimulation of ipsilateral hindlimb muscle nerves. Ninety-one of the 98 monosynaptic EPSPs were reduced in amplitude during locomotion (mean depression of the 91 was to 66 % of control values); seven increased in amplitude (to a mean of 121 % of control). Twenty-one of the 22 field potentials were depressed during locomotion (mean depression to 72 % of control). All but 14 Ia EPSPs were smaller during both the flexion and extension phases of locomotion than during control. In 35 % of the cases there was < 5 % difference between the amplitudes of the EPSPs evoked during the flexion and extension phases. In 27 % of the cases EPSPs evoked during flexion were larger than those evoked during extension. The remaining 38 % of EPSPs were larger during extension. There was no relation between either the magnitude of EPSP depression or the locomotor phase in which maximum EPSP depression occurred and whether an EPSP was recorded in a flexor or extensor motoneurone. The mean recovery time of both EPSP and field potential amplitudes following the end of a bout of locomotion was approximately 2 min (range, < 10 to > 300 s). Motoneurone membrane resistance decreased during fictive locomotion (to a mean of 61 % of control, n = 22). Because these decreases were only weakly correlated to EPSP depression (r 2 = 0.31) they are unlikely to fully account for this depression. The depression of monosynaptic EPSPs and group I field potentials during locomotion is consistent with the hypothesis that during fictive locomotion there is a tonic presynaptic regulation of synaptic transmission from group Ia afferents to motoneurones and interneurones. Such a reduction in neurotransmitter release would decrease group Ia monosynaptic reflex excitation during locomotion. This reduction may contribute to the tonic depression of stretch reflexes occurring in the decerebrate cat during locomotion.  (+info)

White matter injury in spinal cord ischemia: protection by AMPA/kainate glutamate receptor antagonism. (7/130)

BACKGROUND AND PURPOSE: Spinal cord ischemia is a serious complication of surgery of the aorta. NMDA receptor activation secondary to ischemia-induced release of glutamate is a major mechanism of neuronal death in gray matter. White matter injury after ischemia results in long-tract dysfunction and disability. The AMPA/kainate receptor mechanism has recently been implicated in white matter injury. METHODS: We studied the effects of AMPA/kainate receptor blockade on ischemic white matter injury in a rat model of spinal cord ischemia. RESULTS: Intrathecal administration of an AMPA/kainate antagonist, 6-nitro-7-sulfamoyl-(f)-quinoxaline-2, 3-dione (NBQX), 1 hour before ischemia reduced locomotor deficit, based on the Basso-Beattie-Bresnahan scale (0=total paralysis; 21=normal) (sham: 21+/-0, n=3; saline: 3.7+/-4.5, n=7; NBQX: 12. 7+/-7.0, n=7, P<0.05) 6 weeks after ischemia. Gray matter damage and neuronal loss in the ventral horn were evident after ischemia, but no difference was noted between the saline and NBQX groups. The extent of white matter injury was quantitatively assessed, based on axonal counts, and was significantly less in the NBQX as compared with the saline group in the ventral (sham: 1063+/-44/200x200 microm, n=3; saline: 556+/-104, n=7; NBQX: 883+/-103, n=7), ventrolateral (sham: 1060+/-135, n=3; saline: 411+/-66, n=7; NBQX: 676+/-122, n=7), and corticospinal tract (sham: 3391+/-219, n=3; saline: 318+/-23, n=7; NBQX: 588+/-103, n=7) in the white matter on day 42. CONCLUSIONS: Results indicate severe white matter injury in the spinal cord after transient ischemia. NBQX, an AMPA/kainate receptor antagonist, reduced ischemia-induced white matter injury and improved locomotor function.  (+info)

Delaying caspase activation by Bcl-2: A clue to disease retardation in a transgenic mouse model of amyotrophic lateral sclerosis. (8/130)

Molecular mechanisms of apoptosis may participate in motor neuron degeneration produced by mutant copper/zinc superoxide dismutase (mSOD1), the only proven cause of amyotrophic lateral sclerosis (ALS). Consistent with this, herein we show that the spinal cord of transgenic mSOD1 mice is the site of the sequential activation of caspase-1 and caspase-3. Activated caspase-3 and its produced beta-actin cleavage fragments are found in apoptotic neurons in the anterior horn of the spinal cord of affected transgenic mSOD1 mice; although such neurons are few, their scarcity should not undermine the potential importance of apoptosis in the overall mSOD1-related neurodegeneration. Overexpression of the anti-apoptotic protein Bcl-2 attenuates neurodegeneration and delays activation of the caspases and fragmentation of beta-actin. These data demonstrate that caspase activation occurs in this mouse model of ALS during neurodegeneration. Our study also suggests that modulation of caspase activity may provide protective benefit in the treatment of ALS, a view that is consistent with our recent demonstration of caspase inhibition extending the survival of transgenic mSOD1 mice.  (+info)

Anterior horn cells, also known as motor neurons, are a type of nerve cell located in the anterior horn of the spinal cord and in the brainstem. These cells are responsible for transmitting signals from the brain to the muscles, allowing for voluntary movement. They receive input from sensory neurons in the periphery, process that information, and then send signals to the muscles to produce movement. Damage to anterior horn cells can result in various motor neuron diseases, such as amyotrophic lateral sclerosis (ALS) or spinal muscular atrophy (SMA).

Spinal Muscular Atrophies of Childhood (SMA) is a group of genetic disorders that affect the muscles responsible for movement. SMA is caused by a deficiency or absence of the survival motor neuron (SMN) protein, which is necessary for the development and maintenance of motor neurons in the spinal cord and brainstem. There are four main types of SMA, which are classified based on the severity of the symptoms and the age of onset. Type I SMA, also known as Werdnig-Hoffmann disease, is the most severe form of SMA and typically presents in infancy, with symptoms including weakness and loss of muscle tone in the arms and legs, difficulty breathing, and poor head control. Type II SMA, also known as intermediate SMA, typically presents in early childhood and is characterized by progressive weakness and loss of muscle tone in the arms and legs, but with better head control and breathing ability than in type I SMA. Type III SMA, also known as Kugelberg-Welander disease, typically presents in late childhood or adolescence and is characterized by progressive weakness and loss of muscle tone in the arms and legs, but with better head control and breathing ability than in type I and II SMA. Type IV SMA, also known as adult-onset SMA, typically presents in adulthood and is characterized by progressive weakness and loss of muscle tone in the arms and legs, but with better head control and breathing ability than in the other types of SMA. SMA is a progressive disorder, meaning that the symptoms typically worsen over time. There is currently no cure for SMA, but there are treatments available that can help manage the symptoms and improve quality of life. These treatments include physical therapy, respiratory support, and, in some cases, medication and surgery.

Muscular atrophy, spinal, is a medical condition characterized by the wasting away or shrinkage of muscles in the spinal cord. This type of atrophy is caused by damage or injury to the spinal cord, which can result from a variety of factors such as trauma, disease, or surgery. Symptoms of spinal muscular atrophy may include weakness or paralysis in the affected muscles, difficulty with movement or coordination, and muscle cramps or spasms. The severity of the condition can vary widely depending on the extent of the spinal cord damage and the location of the affected muscles. Treatment for spinal muscular atrophy typically involves a combination of physical therapy, medication, and assistive devices such as braces or wheelchairs. In some cases, surgery may be necessary to address underlying spinal cord damage or to improve mobility and function.

Neuronal Apoptosis-Inhibitory Protein (NAIP) is a protein that plays a role in regulating programmed cell death, or apoptosis, in neurons. It is expressed in the brain and spinal cord, and is thought to play a protective role by inhibiting apoptosis, which is the process by which cells undergo programmed death. NAIP has been implicated in a number of neurological disorders, including Alzheimer's disease, Parkinson's disease, and multiple sclerosis. It is also involved in the development and maintenance of the nervous system, and may play a role in the regulation of synaptic plasticity, which is the ability of synapses to change in strength in response to experience.

SMN Complex Proteins are a group of proteins that play a crucial role in the process of splicing pre-mRNA molecules in the nucleus of cells. The SMN complex is responsible for the assembly of small nuclear ribonucleoproteins (snRNPs), which are essential for the proper splicing of pre-mRNA molecules. Mutations in the genes encoding SMN complex proteins can lead to a group of genetic disorders known as spinal muscular atrophies (SMAs), which are characterized by progressive muscle weakness and atrophy. These disorders are caused by a deficiency in the production of functional SMN protein, leading to impaired splicing of pre-mRNA molecules and ultimately, the death of motor neurons in the spinal cord and brainstem.

Survival of Motor Neuron 1 (SMN1) protein is a protein that plays a crucial role in the development and maintenance of motor neurons, which are specialized nerve cells that control muscle movement. Mutations in the SMN1 gene can lead to a group of inherited disorders known as spinal muscular atrophies (SMAs), which are characterized by progressive muscle weakness and atrophy. SMN1 protein is synthesized in the nucleus of cells and then transported to the cytoplasm, where it helps to assemble and stabilize snRNPs (small nuclear ribonucleoproteins), which are essential for the processing of pre-mRNA (messenger RNA) into mature mRNA. This process is critical for the production of proteins, including those involved in muscle function. In individuals with SMA, mutations in the SMN1 gene lead to a deficiency in SMN1 protein, which in turn disrupts the assembly and function of snRNPs. This results in a reduction in the production of proteins necessary for muscle function, leading to progressive muscle weakness and atrophy. SMN1 protein is the primary gene responsible for SMA, but a second gene called SMN2 can also produce a functional protein. However, the amount of functional protein produced by SMN2 is much lower than that produced by SMN1, which contributes to the severity of SMA in affected individuals.

Spinal cord diseases refer to a group of medical conditions that affect the spinal cord, which is a long, thin, tubular bundle of nerves that runs from the base of the brain down through the back. The spinal cord is responsible for transmitting signals between the brain and the rest of the body, and any damage to the spinal cord can result in a range of symptoms and complications. Spinal cord diseases can be classified into several categories, including: 1. Inflammatory diseases: These are conditions that cause inflammation of the spinal cord, such as multiple sclerosis, spinal cord inflammation, and transverse myelitis. 2. Traumatic injuries: These are injuries to the spinal cord caused by accidents, falls, or other external forces, such as spinal cord compression, spinal cord contusion, and spinal cord avulsion. 3. Tumors: These are abnormal growths of cells that can develop on or within the spinal cord, such as spinal cord tumors, schwannomas, and meningiomas. 4. Degenerative diseases: These are conditions that cause the spinal cord to deteriorate over time, such as spinal stenosis, spinal cord compression, and spinal cord atrophy. 5. Genetic disorders: These are conditions that are caused by genetic mutations and can affect the spinal cord, such as spinal muscular atrophy, Friedreich's ataxia, and spinal muscular dystrophy. Spinal cord diseases can cause a range of symptoms, including pain, numbness, weakness, loss of sensation, difficulty walking, and loss of bladder or bowel control. Treatment for spinal cord diseases depends on the underlying cause and severity of the condition, and may include medications, physical therapy, surgery, or other interventions.

Muscular atrophy refers to the loss of muscle mass and strength due to various factors such as disuse, injury, disease, or genetic disorders. It can result in a decrease in muscle size, decreased muscle strength, and a decrease in muscle tone. There are different types of muscular atrophy, including: 1. Neurogenic muscular atrophy: This type of atrophy occurs when there is damage to the nerves that control the muscles. It can be caused by conditions such as spinal cord injury, multiple sclerosis, or amyotrophic lateral sclerosis (ALS). 2. Myogenic muscular atrophy: This type of atrophy occurs when there is damage to the muscle fibers themselves. It can be caused by conditions such as muscular dystrophy, myotonic dystrophy, or polymyositis. 3. Metabolic muscular atrophy: This type of atrophy occurs when there is a problem with the body's metabolism that affects muscle function. It can be caused by conditions such as diabetes, thyroid disorders, or vitamin deficiencies. Muscular atrophy can have a significant impact on a person's quality of life, as it can lead to decreased mobility, difficulty with daily activities, and reduced independence. Treatment for muscular atrophy depends on the underlying cause and may include physical therapy, medication, or surgery.

Arthrogryposis is a medical condition characterized by the presence of multiple joint contractures (stiffness) in a newborn or infant. These contractures can affect any joint in the body, but are most commonly seen in the arms, legs, and hands. The severity of arthrogryposis can vary widely, ranging from mild to severe, and can be caused by a variety of factors, including genetic mutations, problems during fetal development, and certain medical conditions. Treatment for arthrogryposis typically involves physical therapy, occupational therapy, and in some cases, surgery to improve joint mobility and function. Early intervention is important to prevent muscle atrophy and to improve the child's ability to move and function.

In the medical field, paralysis refers to a loss of muscle function or weakness in one or more areas of the body. This can be caused by a variety of factors, including injury, disease, or neurological disorders. There are several types of paralysis, including: 1. Complete paralysis: This is when a person is unable to move any part of their body. 2. Partial paralysis: This is when a person has some muscle function, but not all of it. 3. Flaccid paralysis: This is when the muscles are weak and floppy, and the person may have difficulty moving or maintaining their posture. 4. Spastic paralysis: This is when the muscles are tight and tense, and the person may have difficulty controlling their movements. Paralysis can affect any part of the body, including the arms, legs, face, and voice. It can be temporary or permanent, and can range from mild to severe. Treatment for paralysis depends on the underlying cause and can include physical therapy, medication, surgery, or other interventions.

Neuromuscular diseases are a group of disorders that affect the muscles and nerves. These diseases can cause weakness, wasting, and muscle stiffness, as well as difficulty with movement and coordination. Some common examples of neuromuscular diseases include muscular dystrophy, amyotrophic lateral sclerosis (ALS), and spinal muscular atrophy (SMA). These diseases can be caused by genetic mutations, infections, or other factors, and they can be progressive, meaning that they worsen over time. Treatment for neuromuscular diseases may include medications, physical therapy, and assistive devices to help manage symptoms and improve quality of life.

Motor Neuron Disease (MND) is a group of neurological disorders that affect the motor neurons, which are the nerve cells responsible for controlling voluntary muscle movement. These diseases are characterized by the progressive degeneration and death of motor neurons, leading to muscle weakness, atrophy, and eventually paralysis. There are several types of MND, including amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), and spinal muscular atrophy (SMA). ALS is the most common form of MND and is also known as Lou Gehrig's disease, after the famous baseball player who died from the disease. MND is a fatal disease, and there is currently no cure. Treatment is focused on managing symptoms and improving quality of life. This may include physical therapy, occupational therapy, speech therapy, and the use of assistive devices. Medications may also be used to manage symptoms such as muscle stiffness, spasticity, and pain.

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurodegenerative disorder that affects nerve cells in the brain and spinal cord. These nerve cells, called motor neurons, control voluntary muscle movement. In ALS, the motor neurons gradually degenerate and die, leading to muscle weakness, wasting, and paralysis. The symptoms of ALS typically begin with muscle weakness or stiffness in the arms or legs, which may progress to difficulty speaking, swallowing, or breathing. The disease usually progresses slowly, and affected individuals may eventually become completely paralyzed and require assistance with basic activities of daily living. There is currently no cure for ALS, and treatment is focused on managing symptoms and improving quality of life. Some medications and therapies may help to slow the progression of the disease and alleviate symptoms, but they cannot stop the underlying neurodegeneration.

... (LAAHD) is an autosomal recessive genetic disorder characterized by ... "Lethal arthrogryposis with anterior horn cell disease". Hum Pathol. 26 (1): 12-19. doi:10.1016/0046-8177(95)90109-4. PMID ... The size and shape of spinal cord at different levels are normal but anterior horn motoneurons are diminished in number and ...
... with anterior horn cell disease; 611890; GLE1 Arthrogryposis, renal dysfunction, and cholestasis 1; 208085; VPS33B ... T cell-negative, B-cell/natural killer-cell positive; 608971; CD3D Severe combined immunodeficiency, T cell-negative, B-cell/ ... T cell-negative, B-cell/natural killer-cell positive; 608971; PTPRC Severe combined immunodeficiency, T-cell negative, B-cell/ ... FLCN Renal cell carcinoma; 144700; DIRC2 Renal cell carcinoma; 144700; HNF1A Renal cell carcinoma; 144700; RNF139 Renal cell ...
Familial agenesis of corpus callosum with anterior horn cell disease. Trans Am Neurol Assoc 1972; 97: 242-244 Andermann E, ...
... the anterior horn cells subserving the affected muscles are often damaged. The evidence for anterior horn cell involvement ... In one case, a biopsy of an affected muscle "revealed scattered atrophic fibers, indicating lesions in the anterior horn cells ... Arita, J; Nakae, Y; Matsushima, H; Maekawa, K (1995). "Hopkins syndrome: T2-weighted high intensity of anterior horn on spinal ...
This is seen in anterior (ventral) horn cells or certain cranial nerve nuclei. Whereas the extrapyramidal system centers around ... the modulation and regulation through indirect control of anterior (ventral) horn cells. The extrapyramidal subcortical nuclei ... and descend in the anterior funiculus of the spinal cord. Fibers run down to the anterior funiculus to the cervical spinal cord ... The vestibular system is able to respond correctly by recording sensory information from hairs cells in the labyrinth of the ...
Loss of anterior horn cells of the spinal cord are observed in some cases. Dorsal root ganglia, posterior roots and peripheral ... another nerve cell or a muscle). Significant for this mechanism is the L1CAM gene, a cell surface glycoprotein of the ... and other substances through the cell. Long nerve fibers (axons) are affected because long distances make nerve cells ... The affected cells are the primary motor neurons; therefore, the disease is an upper motor neuron disease. HSP is not a form of ...
The fatal childhood disorder is associated with loss of anterior horn cells and infantile death. Clinical features include ... Cell. 134 (2): 268-78. doi:10.1016/j.cell.2008.05.046. PMID 18662542. This figure is adapted from Scheme 1 of "The Mechanism of ... Cell. 134 (2): 268-78. doi:10.1016/j.cell.2008.05.046. PMID 18662542. Schulman BA, Harper JW (May 2009). "Ubiquitin-like ... In normally functioning cells, the covalent linkage of ubiquitin or ubiquitin-like protein to a target protein changes the ...
In amyotrophic lateral sclerosis, Onuf's nucleus is preserved but the other anterior horn cell groups atrophy. This discovery ... Onuf's nucleus is a distinct group of neurons located in the ventral part (lamina IX) of the anterior horn of the sacral region ... Onuf's nucleus is a distinct group of neurons located in the ventral part (lamina IX) of the anterior horn of the sacral region ... Both cell types are spared by amyotrophic lateral sclerosis. Onuf's nucleus cells are anatomically linked with the sacral ...
The hyperexcitable corticomotoneurons drive anterior horn cells into metabolic deficit, resulting in cell degeneration and ... If this exocitotoxic process occurs rapidly, it results in a more rapid death of anterior horn cells resulting in lower motor ... In addition cortico spinal tract lesions do not cause anterograde transneuronal degeneration of spinal anterior horn cells. ... a long survival period for those affected cells. The degeneration led to cell shrinkage and cell death, and these symptoms were ...
... arthrogryposis and anterior horn cell loss (Lethal arthrogryposis with anterior horn cell disease, LAAHD) was also shown to ... Lethal arthrogryposis with anterior horn cell disease Online Mendelian Inheritance in Man (OMIM): 255310 Norio R (2003). "The ... Neuropathological analysis shows lack of anterior horn motoneurons and severe atrophy of the ventral spinal cord. The skeletal ...
A severe loss of anterior horn cells within the spinal cord and meningoencephalitis of the brain were discovered. A ... considerable loss of Purkinje cells was evident in the patient. Alkhurma virus Louping ill Tavakoli NP, Wang H, Dupuis M, Hull ...
... examples are pyramidal cells, Purkinje cells, and anterior horn cells Golgi II: neurons whose axonal process projects locally; ... interneurons with unique dendrite ending in a brush-like tuft Granule cells, a type of Golgi II neuron Anterior horn cells, ... researchers have converted connective tissue cells found in skin into heart cells, blood cells, and liver cells. Wang Z, Tang B ... Neurons are special cells which are made up of some structures that are common to all other eukaryotic cells such as the cell ...
One notable sign of SMAX2 is the loss of anterior horn cells in the spinal cord and brain stem. SMAX2 is typically confirmed ... XL-SMA is characterized by severe hypotonia and areflexia with loss of anterior horn cells in the spinal cord (i.e., lower ... Evidence of degeneration and loss of anterior horn cells in the spinal cord and brain stem Normal SMN1 molecular genetic ... Digital contractures at birth that remain throughout the child's life Evidence of degeneration and loss of anterior horn cells ...
She was diagnosed with anterior horn cell myelitis and died on 22 September 1974 at the age of 45. 1965: Invasion - Elaine 1972 ...
"Familial agenesis of the corpus callosum with anterior horn cell disease: a syndrome of mental retardation, areflexia, and ... The anterior commissure is almost always absent, but occasionally hypoplastic. A bundle of Probst can be found running ...
The condition is a result of a loss of anterior horn cells localized to lumbar and cervical regions of the spinal cord early in ... a true form of spinal muscular atrophy caused by early loss of anterior horn cells". Brain. 135 (Pt 6): 1714-23. doi:10.1093/ ...
Disconnect of upper motor neuron influences on the anterior horn cells possibly by myelitis or glutamate excitotoxicity have ... which is not expected for a pure anterior horn involvement. ... Anderson RC, Horn KB, Hoang MP, Gottlieb E, Bennin B (November ... West Nile meningitis (WNM) usually involves fever, headache, stiff neck and pleocytosis, an increase of white blood cells in ... Morpholino antisense oligos conjugated to cell penetrating peptides have been shown to partially protect mice from WNV disease ...
Jean-Martin Charcot encouraged him to study neurology, and together they demonstrated atrophy of anterior horn cells in the ...
... anterior horn cell disorders, Isaacs syndrome, and Hoffmann syndrome. Generally, repeated contraction of the muscle can ... Molecular Cell. 10 (1): 45-53. doi:10.1016/s1097-2765(02)00572-5. PMID 12150906. Kwieciński, H; Lehmann-Horn, F; Rüdel, R ( ... The ClC-1 ion channel is responsible for the major part of chloride conductance in the skeletal muscle cell, and lack of ... Molecular Cell. 10 (1): 35-44. doi:10.1016/s1097-2765(02)00563-4. PMID 12150905. Charlet-B, N; Savkur, RS; Singh, G; Philips, ...
... and found that the spinal cord had extreme loss of anterior horn cells and demyelination and sclerosis of the ventrolateral ... They also found changes in the color of the myelin of the optic nerves, decreases in Purkinje cells, increase in Bergman glia, ...
... the reduced availability of the SMN protein results in gradual death of motor neuron cells in the anterior horn of spinal cord ... Whilst stem cells never form a part of any recognised therapy for SMA, a number of private companies, usually located in ... In 2013-2014, a small number of SMA1 children in Italy received court-mandated stem cell injections following the Stamina scam ... Carrozzi M, Amaddeo A, Biondi A, Zanus C, Monti F, Alessandro V (November 2012). "Stem cells in severe infantile spinal ...
There are 36 identified Finnish heritage diseases: Amyloidosis, Finnish type Lethal arthrogryposis with anterior horn cell ...
... cell bodies of anterior horn cells" Histology at anhb.uwa.edu.au Tissues containing Nissl bodies at harvard.edu (Articles with ... Similar to the ergastoplasm of glandular cells, Nissl bodies are the main site of protein synthesis in the neuronal cytoplasm. ... Fawcett, Don W. (1966). The Cell: Its Organelles and Inclusions. W.B. Saunders Company. ISBN 0-7216-3585-7. Peters A, Palay SL ... Masses of rough endoplasmic reticulum also occur in some non-neuronal cells, where they are referred to as ergastoplasm, ...
Because a different population of anterior horn cells is stimulated with each stimulation, F waves are characterized as ... As the antidromic impulse reaches the cell bodies within the anterior horn of the motor neuron pool by retrograde transmission ... towards the dorsal horn), as well as antidromic activity (along alpha motor neurons towards the ventral horn). Antidromic ... This impulse travels both in orthodromic fashion (towards the muscle fibers) and antidromic fashion (towards the cell body in ...
Fibers from the lateral vestibular nucleus also pass via the vestibulospinal tract, to anterior horn cells at many levels in ... The latter end by arborizing around the cells of the medial nucleus, which is situated in the area acustica of the rhomboid ... Some of the axons of the cells of the lateral nucleus, and possibly also of the medial nucleus, are continued upward through ... while the remainder ascend in the medial longitudinal fasciculus to arborize around the cells of the nuclei of the oculomotor ...
Poliovirus attacks specific neurons in the brainstem and the anterior horn cells of the spinal cord, generally resulting in the ... The most widely accepted theory of the mechanism behind the disorder is "neural fatigue". A motor unit is a nerve cell (or ... in which the body's immune system attacks normal cells as if they were foreign substances. Again, compared to neural fatigue, ... neural fatigue theory proposes that the enlargement of the motor neuron fibers places added metabolic stress on the nerve cell ...
... genetic mutations in various genes and are characterized by degeneration and loss of motor neuron cells in the anterior horn of ...
Cerebellar ataxia in diseases classified elsewhere 334.8 Other 334.9 Unspecified 335 Anterior horn cell disease 335.0 Werdnig- ... ciliary body and anterior chamber 364.7 Adhesions and disruptions of iris and ciliary body 364.8 Other disorders of iris and ...
... where in humans they synapse directly with anterior horn cells, which in turn synapse directly with their target muscles. Betz ... Betz cells (also known as pyramidal cells of Betz) are giant pyramidal cells (neurons) located within the fifth layer of the ... According to one study, Betz cells represent about 10% of the total pyramidal cell population in layer Vb of the human primary ... Stained brain slice images which include the "Betz cell" at the BrainMaps project NIF Search - Betz Cell via the Neuroscience ...
... limb muscle atrophy nonreactive degeneration of anterior horn cells degeneration and partial loss of pyramidal neurons of the ... behavioral dysfunction conduction deficits in the central motor pathway neuropathological changes of motor cortex Betz cells ...
Lethal arthrogryposis with anterior horn cell disease (LAAHD) is an autosomal recessive genetic disorder characterized by ... "Lethal arthrogryposis with anterior horn cell disease". Hum Pathol. 26 (1): 12-19. doi:10.1016/0046-8177(95)90109-4. PMID ... The size and shape of spinal cord at different levels are normal but anterior horn motoneurons are diminished in number and ...
Dive into the research topics of Expression of hepatocyte growth factor and c-Met in the anterior horn cells of the spinal ... title = "Expression of hepatocyte growth factor and c-Met in the anterior horn cells of the spinal cord in the patients with ... T1 - Expression of hepatocyte growth factor and c-Met in the anterior horn cells of the spinal cord in the patients with ... Expression of hepatocyte growth factor and c-Met in the anterior horn cells of the spinal cord in the patients with amyotrophic ...
... disease characterized by an excessive accumulation of glycogen within lysosome-derived vacuoles in nearly all types of cells. ... "Metabolic" anterior horn cell pathology (uncommon) Juvenile AMD is characterized by the following [5, 6, 7, 8, 9] :. * Motor ... The presence of abnormal quantities of glycogen disrupts the normal architecture and function of the affected cells. The excess ... disease characterized by an excessive accumulation of glycogen within lysosome-derived vacuoles in nearly all types of cells. ...
... partial anterior horn cell dysfunction is known to occur. [70] ... 200 cells/µL) peripheral blood CD4+ T-cell counts. ... The diagnosis of neurosyphilis is based on a CSF WBC count of 20 cells/µL or greater, and/or a reactive CSF VDRL, and/or a ... Adhesions of the iris to the anterior lens (synechiae) may be present, which may produce a fixed pupil; this should not be ... PCN acts by interfering with the synthesis of cell walls and is active only against organisms that, like T pallidum, synthesize ...
Anterior Horn Cell Disease,Lethal Congenital Contracture Syndrome 1,Lethal Congenital Contracture Syndrome ... Basal Cell Nevus Syndrome,Precursor T-Cell Acute Lymphoblastic Leukemia,Breast Cancer,Microcephaly,Hepatitis C Virus,Alacrima, ... Interdigitating Dendritic Cell Sarcoma,Xeroderma Pigmentosum, Variant Type,Fanconi Anemia, Complementation Group A ... DNA repair protein complementing XP-B cells) (TFIIH basal transcription factor complex 89 kDa subunit) (TFIIH 89 kDa subunit) ( ...
Misra UK, Kalita J. Anterior horn cells are also involved in Japanese encephalitis. Acta Neurol Scand 1997;96:114-7. CrossRef ... One-year immunogenicity kinetics and safety of a purified chick embryo cell rabies vaccine and an inactivated Vero cell-derived ... One-year immunogenicity kinetics and safety of a purified chick embryo cell rabies vaccine and an inactivated Vero cell-derived ... JE-VC is an inactivated vaccine derived from the attenuated SA14-14-2 JE virus strain propagated in Vero cells (Box 1) (14,201, ...
Pontocerebellar hypoplasia with infantile spinal muscular atrophy, pontocerebellar hypoplasia with anterior horn cell disease ... Onset in first or second decade and often bedbound by 20s; loss of cerebellar Purkinje cells, inferior olivary cells, myelin ... Current and Emerging Approaches to B-Cell-Targeted Therapies for Multiple Sclerosis 0.25 CME Credits ... Current and Emerging Approaches to B-Cell-Targeted Therapies for Multiple Sclerosis ...
Categories: Anterior Horn Cells Image Types: Photo, Illustrations, Video, Color, Black&White, PublicDomain, CopyrightRestricted ...
... anterior horn cell disease (including amyotrophic lateral sclerosis); Huntingtons disease; cirrhosis; dialysis; congestive ...
Lead neuropathy is believed to be a motor neuron, anterior horn cell disease with peripheral dying-back of the axons. Frank ... Lead interferes with a hormonal form of vitamin D, which affects multiple processes in the body, including cell maturation and ... Lead affects primarily the peripheral and central nervous systems, the blood cells, and metabolism of vitamin D and calcium. ... EP is elevated in iron deficiency anemia and in sickle cell and other hemolytic anemias. In erythropoietic protoporphyria, an ...
... an abnormality of red blood cells. Neurologic problems usually consist of either movement disorders or ataxia, personality ... Pathology reports showed abnormalities in Betz cells, brainstem neurons, and anterior horn cells. Muscle pathology results are ... Neuronal loss and mild gliosis can be seen in the thalamus, substantia nigra, and anterior horn of the spinal cord. ... Alterations of red cell membrane properties in neuroacanthocytosis. PLoS One. 2013. 8(10):e76715. [QxMD MEDLINE Link]. [Full ...
The anterior horn cells are located in the gray matter of the spinal cord and thus are technically part of the CNS. In contrast ... The anterior horn cells, although technically part of the central nervous system (CNS), are sometimes discussed with the ... Because sensory and motor cell bodies are in different locations, a nerve cell body disorder typically affects either the ... Cause is thought... read more ) can often be repaired by surviving Schwann cells in about 6 to 12 weeks. ...
1944: NIH reports that DDT damages the same anterior horn cells that are damaged in infantile paralysis. 1946: Gebhaedt shows ...
... increasing duration of after hyperpolarization for small anterior horn cells discharging, and influence of the Golgi tendon ... The middle of the proximal interphalangeal joint of the right little finger was fixed perpendicular to the load cell. Analysis ...
... two dorsal or posterior horns that contain cell bodies of sensory neurons and two ventral or anterior horns that contain cell ... The lateral tract carries sensory information for pain and temperature, while the anterior tract carries information for crude ... found inside a dorsal root ganglion carries sensory input from the skin to the dorsal horn of the spinal cord, where it ... levels and decussates or crosses to the opposite side of the spinal cord via an area of white matter called the anterior white ...
Anterior Horn Cell Disease. *Functional MRI, EEG. News & Publications * Publications & articles Lack of evidence for Cortical ...
anterior horn cell disease DOID:4873 * anterior foramen magnum meningioma DOID:6381 ...
Anterior Horn Cells 100% * Motor Neuron Disease 87% * Spinal Cord 57% * Motor Neurons 45% ... Distinct expression patterns of carbonic anhydrase IX in clear cell, microcystic, and angiomatous meningiomas. Chkheidze, R., ...
With Anterior Horn Cell Disease, Lethal Congenital Contracture Syndrome, Amyotrophic Lateral Sclerosis ... With T-Cell Inactivation Due To Calcium Entry Defect, Myopathy,. Tubular Aggregate, Stormorken Syndrome. Stormorken-Sjaastad- ...
This is just anterior horn cells, and may be deceived by removing any bone marrow transplant, thromboembolism, external iliac ... Abnormal play: eg pituitary enlargement in symptoms may be of the anterior horn cells at serum creatinine levels: provides a ... Flomax is a solid lines for squamous cell carcinoma, and mucous drainage. They may cause vague and benefits of crystalloid and ... Instead, picking up to epithelial cells may occur at cheapest flomax 0.4 conduct and susceptibility to do not to doctors and ...
Golgi I; neurons with long-projecting axonal processes; examples are pyramidal cells, Purkinje cells, and anterior horn cells. ... 1. Cell Membrane. The cell membrane or plasma membrane is a biological membrane that separates the interior of all cells from ... Cell polarity refers to spatial differences in the shape, structure, and function of cells. Almost all cell types exhibit some ... Cell membranes are involved in a variety of cellular processes such as cell adhesion, ion conductivity and cell signaling and ...
Electromyogram examination indicated chronic neurogenic changes and anterior horn cells involvement. Although there was no ... In myelinating Schwann cells, connection between myelin layers is mediated by gap junction channels (GJCs) formed by docked ... The GJB1 gene encodes connexin 32 (CX32), a gap junction protein expressed in myelinating glial cells. The gene is X-linked, ... We investigated DHX9 variant-associated cellular phenotypes in human cell lines. Whereas wild-type DHX9 was restricted to the ...
The upper motor neuron syndrome is caused by a lesion proximal to the anterior horn cell; in the spinal cord, brainstem, or ...
  • Lethal arthrogryposis with anterior horn cell disease (LAAHD) is an autosomal recessive genetic disorder characterized by reduced mobility of the foetus and early death. (wikipedia.org)
  • Mutations in GLE1 underlie Lethal Congenital Contracture syndrome (LCCS) and Lethal Arthrogryposis with Anterior Horn Cell Disease (LAAHD). (mendelian.org)
  • The phenotype of LAAHD is now expanded to include multiple individuals surviving into childhood suggesting that LAAHD is a misnomer and should be re-named Arthrogryposis with Anterior Horn Cell Disease (AAHD). (mendelian.org)
  • Nerve fibers outside the spinal cord join to form anterior (ventral) motor nerve roots and posterior (dorsal) sensory nerve roots. (msdmanuals.com)
  • Grey matter is found within the medial portion of the spinal cord and has two dorsal or posterior horns that contain cell bodies of sensory neurons and two ventral or anterior horns that contain cell bodies of motor neurons. (osmosis.org)
  • Histologically, the number of spinal anterior horn cells in ALS patients decreased along with disease progression. (elsevierpure.com)
  • 3] Inflammation of the spinal anterior roots may lead to disruption of the blood-CNS barrier. (medscape.com)
  • We were notified of sus- spinal anterior horn cells is the most common mechanism pected cases by infection control practitioners and health of WNV-associated paralysis and is associated with signif- professionals at 8 hospitals in and around the catchment icant short- and long-term illness and death. (cdc.gov)
  • However, throughout the course of the disease, certain residual anterior horn cells co-expressed both HGF and c-Met with the same, or even stronger intensity in comparison with those of normal subjects, irrespective of the reduction in the number of immunopositive cells. (elsevierpure.com)
  • These results suggest that the autocrine and/or paracrine trophic support of the HGF-c-Met system contributes to the attenuation of the degeneration of residual anterior horn cells in ALS, while disruption of the neuronal HGF-c-Met system at an advanced disease stage accelerates cellular degeneration and/or the process of cell death. (elsevierpure.com)
  • Acid maltase deficiency (AMD) is an autosomal recessive disease characterized by an excessive accumulation of glycogen within lysosome-derived vacuoles in nearly all types of cells. (medscape.com)
  • Neurosyphilis, quite directly, is defined as a CSF WBC count of 20 cells/µL or greater or a reactive CSF Venereal Disease Research Laboratory (VDRL) test result. (medscape.com)
  • Many authors believe that the mechanism of disease involves an abnormal T-cell response precipitated by an infection. (medscape.com)
  • These pathogens are believed to activate CD4+ helper-inducer T cells, which are particularly important mediators of disease. (medscape.com)
  • AMC has similar basis of anterior horn cell disease as polio does and with aging, it has similar problems with over use issues that polio does. (nothinspecialtb.com)
  • Spinal muscular atrophy is a genetic disease of the anterior horn cell with high morbidity rate in childhood. (omeka.net)
  • Spinal muscular atrophy (SMA) is a recessive, autosomal neuromuscular disease characterized by degeneration of anterior horn spinal cord motor cells and brain stem neurons 1-5 . (bvsalud.org)
  • The CSF findings, in combination with certain clinical features, allowed AIDP to be distinguished from anterior horn cell diseases such as poliomyelitis, spinal muscular atrophy and from other neuropathies. (medscape.com)
  • Replication of poliovirus in motor neurons of the anterior horn and brain stem results in cell destruction and causes the typical manifestations of poliomyelitis. (diseasesdic.com)
  • Terms in the spinal cord MRI report such as "affecting gray matter," "affecting the anterior horn or anterior horn cells," "affecting the central cord," "anterior myelitis," or "poliomyelitis" would all be consistent with this terminology. (cdc.gov)
  • In contrast to the motor system, the cell bodies of the afferent sensory fibers lie outside the spinal cord, in dorsal root ganglia. (msdmanuals.com)
  • For this to happen, a first order neuron, found inside a dorsal root ganglion carries sensory input from the skin to the dorsal horn of the spinal cord , where it synapses with the second order neuron. (osmosis.org)
  • The cell bodies of the sensory nerves are located in the dorsal root ganglia. (medscape.com)
  • The size and shape of spinal cord at different levels are normal but anterior horn motoneurons are diminished in number and degenerated. (wikipedia.org)
  • The anterior horn cells are located in the gray matter of the spinal cord and thus are technically part of the CNS. (msdmanuals.com)
  • And that neuron ascends 1-2 vertebral levels and decussates or crosses to the opposite side of the spinal cord via an area of white matter called the anterior white commissure. (osmosis.org)
  • Muscle weakness is associated with a defect in the anterior horn cell of the spinal cord. (nothinspecialtb.com)
  • The cell body (ie, soma) is in the anterior horn within the cord parenchyma. (medscape.com)
  • Schwann cells form a thin cytoplasmic tube around each fiber and further wrap larger fibers in a multilayered insulating membrane (myelin sheath). (msdmanuals.com)
  • RESULTS: Both alleles produce protein detectable by immunofluorescence in Schwann cells, with some protein properly localizing to nodes of Ranvier. (bvsalud.org)
  • To clarify the trophic mechanism of residual anterior horn cells affected by sporadic amyotrophic lateral sclerosis (SALS) and familial ALS (FALS) with superoxide dismutase 1 (SOD1) mutations, we investigated the immunohistochemical expression of hepatocyte growth factor (HGF), a novel neurotrophic factor, and its receptor, c-Met. (elsevierpure.com)
  • In 1916, Guillain, Barré, and Strohl broadened the clinical description and first reported the characteristic cerebrospinal fluid (CSF) finding, albuminocytologic dissociation (ie, elevation of CSF protein with normal CSF cell count). (medscape.com)
  • The GJB1 gene encodes connexin 32 (CX32), a gap junction protein expressed in myelinating glial cells. (bvsalud.org)
  • He has shown that several accessory proteins regulate the assembly and stability of the bacterial cell division protein FtsZ. (sunpharmasciencefoundation.net)
  • This strain of the poliovirus attacks the spinal column where it destroys the anterior horn cells which control movement of the trunk and limb muscles. (the-medical-dictionary.com)
  • Cell membranes are involved in a variety of cellular processes such as cell adhesion, ion conductivity and cell signaling and serve as the attachment surface for several extracellular structures, including the cell wall, glycocalyx, and intracellular cytoskeleton. (neuroenlight.com)
  • We also show the presence of abundant migratory neuroblasts in the anterior horn SVZ forming structures here denominated cell throngs. (elsevierpure.com)
  • In normal subjects, immunoreactivity to both anti-HGF and anti-c-Met antibodies was observed in almost all anterior horn cells, whereas no significant immunoreactivity was observed in astrocytes and oligodendrocytes. (elsevierpure.com)
  • The anterior horn cells, although technically part of the central nervous system (CNS), are sometimes discussed with the peripheral nervous system because they are part of the motor unit. (msdmanuals.com)
  • The lateral tract carries sensory information for pain and temperature, while the anterior tract carries information for crude touch, or the sense one has been touched, without being able to localize where they were touched. (osmosis.org)
  • Notably, he has elucidated the role of microtubule dynamics in the regulation of cell cycle, cell migration, and apoptosis. (sunpharmasciencefoundation.net)
  • The fetal development of the anterior subventricular zone (SVZ) involves the transformation of radial glia into neural stem cells, in addition to the migration of neuroblasts from the SVZ towards different regions in the brain. (elsevierpure.com)
  • In adult rodents this migration from the anterior SVZ is restricted to the olfactory bulb following a rostral migratory stream (RMS) formed by chains of migratory neuroblasts. (elsevierpure.com)
  • The membranes of all nerve cells have a potential difference across them, with the cell interior negative with respect to the exterior (a). (neuroenlight.com)
  • This means that there is an unequal distribution of ions (atoms with a positive or negative charge) on the two sides of the nerve cell membrane. (neuroenlight.com)
  • Neuroacanthocytosis encompasses a group of genetically heterogenous disorders characterized by neurologic signs and symptoms associated with acanthocytosis, an abnormality of red blood cells. (medscape.com)
  • Robotic biopsy with a daunting task, if symptoms settle in eyeball and 73% specific cell production. (govtjobslatest.org)
  • The author suggest that the decrease in SMUAP discharge rate as fatigue begins may be explained by central and peripheral sources, including feedback from muscle spindles, inhibition offered by the Renshaw feedback system, increasing duration of after hyperpolarization for small anterior horn cells discharging, and influence of the Golgi tendon organ discharge. (cdc.gov)
  • Almost all cell types exhibit some sort of polarity, which enables them to carry out specialized functions. (neuroenlight.com)
  • The virus invades local lymphoid tissue, enters the bloodstream, and then may infect cells of the central nervous system. (diseasesdic.com)
  • In the demyelinating form, segmental demyelination of peripheral nerves is thought to be immune mediated and both humoral and cell-mediated immune mechanisms have been implicated. (medscape.com)
  • The cell membrane is selective permeable to ions and organic molecules and controls the movement of substances in and out of cells.The basic function of the cell membrane is to protect the cell from its surroundings. (neuroenlight.com)
  • In SOD1-mutated FALS patients, Lewy body-like hyaline inclusions (LBHIs) in some residual anterior horn cells exhibited co-aggregation of both HGF and c-Met, although the cytoplasmic staining intensity for HGF and c-Met in the LBHI-bearing neurons was either weak or negative. (elsevierpure.com)
  • Cell organization in this region is heterogeneous along the ventricular wall, with GFAP-positive cells aligned to the ventricle. (elsevierpure.com)