Cases reported "Motor Neuron Disease"

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1/114. Chronic motor axonal neuropathy: pathological evidence of inflammatory polyradiculoneuropathy.

    Chronic immune and inflammatory motor neuropathies may resemble motor neuron disease, and the distinction may be particularly difficult if conduction block or GM1 antibodies are absent. The pathology of this axonal type of chronic motor neuropathy has not been characterized except in a few cases associated with paraproteinemia. We describe the clinical, electrophysiological, and pathological findings in a patient with a chronic motor axonal neuropathy, normal immunoelectrophoresis, and no GM1 antibodies. At autopsy the spinal cord was normal with the exception of chromatolytic motor neurons. All the ventral roots were greatly thinned. Of 10 mixed nerves and numerous spinal roots sampled, five showed areas of perineurial, perivascular lymphocytic infiltration. There was severe axonal loss in the motor roots that was not as evident in mixed nerves, and the sensory nerves and roots were virtually unaffected. Our findings suggest that a chronic motor axonal neuropathy without paraproteinemia or GM1 antibodies may, in some cases, result from an inflammatory process.
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keywords = neuropathy, nerve
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2/114. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.

    The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.
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ranking = 0.13892440704985
keywords = neuropathy, peripheral, peripheral nerve, nerve, nervous system
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3/114. Troyer syndrome: a combination of central brain abnormality and motor neuron disease?

    Hereditary spastic paraplegia is a group of clinically and genetically heterogeneous disorders consisting of pure and complicated forms. A variant with the additional features of severe atrophy of the small hand muscles, dysarthria, mental retardation, and short stature has been termed Troyer syndrome (MIM#275900) after the name of Old Order amish families suffering from these symptoms. We report here an Austrian family with two individuals who exhibit all the features of Troyer syndrome, and provide additional data on this disorder. Electrophysiological studies showed chronic denervation and reduced motor nerve conduction velocities but normal sensory potentials. Muscle biopsy revealed a neurogenic pattern while the sural nerve was normal on histological examination. brain abnormalities on magnetic resonance imaging consisted of a thin corpus callosum with a poorly developed cingulate gyrus and mild periventricular signal hyperintensities. These findings characterize the Troyer syndrome as a disorder of the first and second motor neuron with additional damage in the brain. The morphological features observed in this family may contribute to the grouping and subsequent understanding of complicated forms of hereditary spastic paraplegia, together with similar observations in other, more recently reported families.
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ranking = 0.0021279958264469
keywords = nerve
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4/114. Immunoadsorption in multifocal motor neuropathy.

    To our knowledge, this is the first reported application of immunoadsorption in a patient with multifocal motor neuropathy (MMN). The diagnosis relied on the typical clinical features, markedly increased IgM-GM1 antibodies, multiple motor conduction blocks, and motor nerve biopsy. Immunoadsorption was carried out in seven cycles of two sessions each within 97 days. From the first therapy, muscle force as well as nerve conduction velocity and conduction blocks deteriorated continuously. As a consequence, immunoadsorption cannot be recommended as a treatment for MMN.
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ranking = 0.4552225441266
keywords = neuropathy, nerve
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5/114. Bilateral phrenic neuropathy as a presenting feature of multifocal motor neuropathy with conduction block.

    Diaphragmatic paralysis has previously been reported as a result of diverse pathologic processes involving the peripheral nervous system. We report the clinical history, physical findings, and antibody profile of an atypical case of multifocal motor neuropathy with conduction block initially presenting with respiratory failure secondary to bilateral phrenic neuropathy.
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ranking = 1.0305117065522
keywords = peripheral nervous system, neuropathy, peripheral, nervous system
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6/114. Motor neuron presentation of an ulnar neuropathy and Riche-Cannieu anastomosis.

    A Riche (7)-Cannieu (2) anastomosis (ulnar-to-median anastomosis in the hand) in the setting of an ulnar or median nerve lesion can produce confusing clinical and electrodiagnostic findings. We report a patient with a deep branch ulnar neuropathy complicated by a Riche-Cannieu anastomosis. His clinical presentation led to an initial diagnosis of motor neuron disease. Extensive electrophysiologic studies clarified the extent of the Riche-Cannieu anastomosis and the ulnar neuropathy.
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ranking = 0.5447774558734
keywords = neuropathy, nerve
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7/114. pathology of Madras type of motor neuron disease (MMND)--a histological and immunohistochemical study.

    A neuropathological report of Madras type of motor neuron disease (MMND) is presented and the differences from other forms of MND are discussed. An 18-year-old girl presented with nerve deafness and slowly progressive bulbo-spinal muscular atrophy, characteristic of MMND. Post-mortem examination of the spinal cord showed a severe loss of anterior horn cells, prominent dilatation of vessels, diffuse, but sparse sprinkling of microglial cells and lymphocytes, and demyelination and sclerosis of the ventrolateral columns. Neuronal depletion and marked gliosis was noted in the cochlear nucleus on both sides, while other bulbar motor nuclei were also involved. The cochlear nerve showed demyelination and axonal loss. Trigeminal and vestibular ganglia revealed features of ganglionitis. The possibility of an inflammatory aetiology for MMND needs to be considered.
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ranking = 0.0021279958264469
keywords = nerve
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8/114. Chronic motor axonal neuropathy associated with antibodies monospecific for N-acetylgalactosaminyl GD1a.

    We report on three patients with chronic motor neuropathy who had elevated titers of immunoglobulin (Ig)G antibodies against N-acetylgalactosaminyl GD1a (GalNAc-GD1a) and normal titers of antibodies against other gangliosides. Presenting with progressive muscular atrophy, fasciculations, and no sensory deficits, the patients had been diagnosed to have motor neuron disease. Electrodiagnostic features were predominantly axonal. Two patients clinically improved after intravenous Ig infusion and cyclophosphamide therapy. Increased titers of IgM antibodies to GalNAc-GD1a were also found in two of 15 patients with multifocal motor neuropathy with conduction block but were associated with concomitant rise of anti-GM1 antibodies. These three cases represent a chronic motor axonal neuropathy in which antibody testing for a minor ganglioside was helpful for instituting therapy.
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ranking = 0.63433236762021
keywords = neuropathy
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9/114. Delayed radiation-induced bulbar palsy mimicking ALS.

    We describe a patient presenting with progressive bulbar dysfunction and spasticity that clinically mimicked amyotrophic lateral sclerosis (ALS). electromyography, however, showed no evidence of denervation and revealed a rare combination of peripheral and central myokymia. We feel that this pattern of myokymia represented a marker of neural injury from remote radiation therapy. Nervous system disorders resulting from therapeutic radiation are described, and potential pathophysiologic mechanisms underlying myokymia are discussed.
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ranking = 0.0053642333288517
keywords = peripheral
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10/114. Activity-dependent conduction block in multifocal motor neuropathy.

    patients with multifocal motor neuropathy may complain of muscle fatigue, even though the degree of conduction block assessed at rest has improved with treatment. To explore the mechanism involved, we examined changes in muscle force during maximum voluntary contraction (MVC) and monitored conduction block before and after MVC in five patients with multifocal motor neuropathy. The results were compared with those for the contralateral unaffected homonymous muscles. For one patient, who had bilateral involvement, a normal subject of a similar age and stature served as the control. Results of conduction studies were also compared with those from six patients with amyotrophic lateral sclerosis (ALS) with similar compound muscle action potential (CMAP) amplitudes after proximal stimulation. During MVC for 60 s, the affected muscles developed prominent fatigue; the force at the end of contraction compared with the initial force was significantly lower for the affected muscles [42 /- 19% (mean /- standard deviation) of the initial force] than for the control muscles (94 /- 9%; P = 0.01). After MVC, the amplitude ratio of CMAPs after proximal versus distal nerve stimulation transiently decreased to 19 /- 14% of that before MVC in the affected muscles, but not in the control muscles (94 /- 3.8% of that before MVC) and in patients with ALS (95 /- 6.7%). In one patient with a focal lesion in the forearm, nerve excitability was monitored at the lesion site before and after MVC for 120 s. There were significant increases in axonal threshold (approximately 48%) and supernormality (approximately 135%) immediately after MVC, suggesting that the axonal membrane had undergone hyperpolarization and, by extrapolation, that this had precipitated the conduction block. This study is the first to show that activity-dependent conduction block plays a role in human disease by causing muscle fatigue.
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ranking = 0.54584145378663
keywords = neuropathy, nerve
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