Pathological findings in a patient with amyotrophic lateral sclerosis and multifocal motor neuropathy with conduction block.
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.
Veugelers
B
B
Department of Neurology, University Hospital Gasthuisberg, Leuven, Belgium.
Theys
P
P
Lammens
M
M
Van Hees
J
J
Robberecht
W
W
D000200:Action Potentials / Q000502:physiology; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D001917:Brachial Plexus / Q000473:pathology / Q000503:physiopathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004576:Electromyography; D004594:Electrophysiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D018908:Muscle Weakness / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D009431:Neural Conduction / Q000187:drug effects / Q000502:physiology*; D010243:Paralysis / Q000473:pathology / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide