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Pathological findings in a patient with amyotrophic lateral sclerosis and multifocal motor neuropathy with conduction block.

Abstract：

:We studied a 53-year-old woman with progressive weakness of the left arm, gradually spreading to the other limbs. Neurological examination revealed a motor neuron syndrome with paresis, fasciculations and atrophy. Electrophysiological studies showed multiple motor conduction blocks. The anti-GM1 IgM titer was elevated. The patient was thought to have a multifocal motor neuropathy. Despite intravenous cyclophosphamide treatment, however, she died with respiratory insufficiency. On postmortem examination, the brachial plexus showed patches of demyelination underlying different areas of motor conduction block. The spinal cord, however, revealed severe neuronal loss in the ventral horn and axonal loss in the corticospinal tract, indicative of amyotrophic lateral sclerosis. Demyelination of peripheral nerves could have been responsible for the other conduction blocks in this patient. The prominent degeneration of motor neurons, however, must also have played a role in the clinical picture. Some patients with the syndrome of a multifocal motor neuropathy may have MND rather than, or in addition to, a demyelinating peripheral motor neuropathy.

journal_name

J Neurol Sci

journal_title

Journal of the neurological sciences

authors

Veugelers B,Theys P,Lammens M,Van Hees J,Robberecht W

doi

10.1016/0022-510x(95)00295-d

subject

Has Abstract

pub_date

1996-03-01 00:00:00

pages

64-70

issue

1-2

eissn

0022-510X

issn

1878-5883

pii

0022-510X(95)00295-D

journal_volume

136

pub_type

杂志文章

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