1/24. Alternate four-point sweep-through gait--a technique for patients with combined neuromuscular and visual impairments: case reports.This article reports on two patients with combined neuromuscular and visual impairments who used a modification of the classic alternate four-point crutch gait, which allowed them to simultaneously explore the upcoming environment for obstacles or change in terrain while maintaining sufficient support for their lower limbs. The technique should be useful for patients with diabetic neuropathy/retinopathy combinations, multiple sclerosis with optic neuritis, and neurosarcoidosis and in elderly patients with multiple disabilities.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/24. Extrapyramidal system involvement in motor neuron disease.Three cases of motor neuron disease (MND), in which neuropathological findings were atypical, are reported. The first case manifested widespread and severe degeneration of the spinal cord, as in spinal fibrosis. Case 2 revealed severe degeneration of the pyramidal tract with many spheroids, which made it difficult to differentiate from primary amyotrophic lateral sclerosis. The last case revealed degeneration of the nigro-pallido-luysian system, even though no clinical manifestation of extrapyramidal and/or cerebellar symptoms had been noted throughout the clinical course. In MND, degeneration might occur in various locations other than the motor system.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/24. Subacute, reversible motor neuron disease.Four patients with a clinical syndrome closely resembling amyotrophic lateral sclerosis recovered completely, without treatment, 5 to 12 months after onset. Electrodiagnostic tests revealed acute and chronic denervation, with normal motor and sensory nerve conduction studies. The CSF was normal, and tests for paraproteinemia, heavy metal intoxication, and systemic illness were negative. Although such cases are rare, the possibility of spontaneous recovery should always be considered when counseling patients with suspected ALS.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/24. Chronic multifocal demyelinating neuropathy simulating motor neuron disease.We describe a patient with a chronic acquired predominantly motor polyneuropathy. His clinical picture initially led to a diagnosis of lower motor neuron form of amyotrophic lateral sclerosis. However electrophysiological examination revealed multifocal, prevalently proximal, conduction blocks at sites not prone to compression. Distinguishing this unusual polyneuropathy from motor neuron diseases is critical, since the former is a potentially, treatable disorder.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
5/24. IgM deposits at nodes of Ranvier in a patient with amyotrophic lateral sclerosis, anti-GM1 antibodies, and multifocal motor conduction block.We studied a patient with amyotrophic lateral sclerosis, multifocal motor conduction block, and IgM anti-GM1 antibodies. A sural nerve biopsy demonstrated deposits of IgM at nodes of Ranvier by direct immunofluorescence. The deposits were granular and located in the nodal gap between adjacent myelin internodes, and in some instances, they extended along the surface of the paranodal myelin sheath. When injected into rat sciatic nerve, the serum IgM bound to the nodes of Ranvier, and the binding activity was removed by preincubation with GM1. These observations suggest that anti-GM1 antibodies may have caused motor dysfunction by binding to the nodal and paranodal regions of peripheral nerve.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
6/24. Paraneoplastic motor neuron disease and renal cell carcinoma: improvement after nephrectomy.A 74-year-old man had a paraneoplastic motor neuron disease mimicking amyotrophic lateral sclerosis. He had an elevated erythrocyte sedimentation rate, other laboratory abnormalities, and a previously undiagnosed renal cell carcinoma. Four months after nephrectomy, his strength had improved and he had no fasciculations. Seven other patients with cancer and motor neuron disease improved or stabilized after tumor treatment. Even though it is rare, paraneoplastic motor neuron disease is important to diagnose because it may be treatable.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/24. Immunologic reactivity against borrelia burgdorferi in patients with motor neuron disease.Of 19 unselected patients with the diagnosis of amyotrophic lateral sclerosis (ALS) living in Suffolk County, new york (an area of high lyme disease prevalence), 9 had serologic evidence of exposure to borrelia burgdorferi; 4 of 38 matched controls were seropositive. Eight of 9 seropositive patients were male (8 of 12 male patients vs 2 of 24 controls). Rates of seropositivity were lower among patients with ALS from nonendemic areas. All patients had typical ALS; none had typical lyme disease. cerebrospinal fluid was examined in 24 ALS patients--3 (all with severe bulbar involvement) appeared to have intrathecal synthesis of anti-B burgdorferi antibody. Following therapy with antibiotics, 3 patients with predominantly lower motor neuron abnormalities appeared to improve, 3 with severe bulbar dysfunction deteriorated rapidly, and all others appeared unaffected. There appears to be a statistically significant association between ALS and immunoreactivity to B burgdorferi, at least among men living in hyperendemic areas.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/24. Respiratory complications and their management in motor neuron disease.Although respiratory insufficiency is common in the advanced stages of motor neuron disease, some patients may develop distressing respiratory symptoms early in the course of the disease or even present with respiratory failure or arrest. We describe 14 patients with motor neuron disease who were considered for respiratory support; 11 received such support and all derived significant symptomatic improvement without distressing prolongation of life. Of the 8 patients with typical features of amyotrophic lateral sclerosis, 7 had predominant diaphragm weakness and 1 generalized respiratory muscle weakness; 7 received negative pressure ventilation by cuirass which improved both the quality of sleep and exercise tolerance. Three patients with predominantly bulbar disease had nocturnal apnoea or hypoventilation. Two received no support. One, who also developed diaphragm weakness, was treated by a cuirass, continuous positive airway pressure (CPAP), and later nocturnal intermittent positive pressure ventilation (IPPV). Three patients with progressive muscular atrophy had predominant diaphragm weakness or nocturnal apnoea. These patients received nocturnal CPAP, cuirass or IPPV with symptomatic benefit. This series shows that some patients with motor neuron disease, mainly those with symptoms due to respiratory muscle weakness in the absence of severe bulbar impairment, derive symptomatic benefit from supported ventilation.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
9/24. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside.We report 2 patients with a treatable, immune-mediated motor polyneuropathy associated with antibodies to defined neural antigens. In these patients asymmetrical weakness developed in one arm and progressed over 2 to 3 years to involve the other arm, legs, and trunk. Both patients were initially diagnosed as having lower motor neuron forms of amyotrophic lateral sclerosis. However, repeated electrophysiological testing eventually showed multifocal conduction blocks in motor but not sensory fibers compatible with patchy selective demyelination. serum testing by thin-layer chromatography and enzyme-linked immunosorbent assay revealed that both patients had high titers of antibody directed against GM1 and other gangliosides. Initial therapeutic trials of prednisone (100 mg daily for 4 to 6 months) and plasmapheresis were unsuccessful. Treatment with cyclophosphamide, however, was followed by marked improvement in strength in both patients.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/24. Anterior horn changes of motor neuron disease associated with demyelinating radiculopathy.Morphologic study of the spinal cord of a patient with generalized motor deficits revealed changes in the anterior horns characterized by the selective loss of large motor neurons, gliosis and the abnormal accumulation of 10 nm filaments which appeared as argyrophilic spheroids in the perikarya and axons of motor neurons. The ventral roots were predominantly affected and showed a variable loss of axons. The remaining axons displayed prominent onion-bulb formations, frequent axonal sprouting and occasionally evidence of active demyelination. The coexistence of a demyelinating motor radiculopathy and anterior horn changes simulating those of amyotrophic lateral sclerosis (ALS) may contribute to our understanding of the unresolved question of whether the neuronal perikaryon or its axon is the primary target in the pathogenesis of ALS. These observations also indicate that a rigid separation of pathogenetic mechanisms into neuronopathy, axonopathy and myelinopathy may not be always possible.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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