Persistent and transient "conduction block" in motor neuron diseases.
Although conduction block indicates dysfunction of peripheral nerve, it may occur in patients with clinically typical motor neuron disease. There are no universally accepted criteria to identify conduction block, so diagnosis may be difficult. In some peripheral neuropathies, conduction block persists over long periods of time. If conduction block persists in motor neuron disease, then a more reproducible means for identification would be available. We repeatedly studied 9 patients with different forms of motor neuron diseases; conduction block was suspected because of excessive loss of the amplitude of motor evoked responses between distal and proximal stimulation sites. Five showed persistent amplitude loss at intervals between 12 and 36 months. All had focal loss of amplitude and area across a specific segment; all were men; none had definite upper motor neuron signs, 2 had probable and 3 had no upper motor neuron signs; 1 had IgM paraproteinemia, one elevated anti-GM1 titers; the duration of symptoms spanned 4-13 years. Four patients had transient loss of amplitude that was not reproduced in intervals between 3 and 13 months. None had focal loss of both amplitude and area; 2 were men; all had definite upper motor neuron signs and none had symptoms for more than 3-13 months; and none had immunological abnormalities. Thus, patients with persistent amplitude loss fulfill other criteria for conduction block, have prolonged survival but otherwise have clinical syndromes indistinguishable from ALS, except that definite upper motor neuron signs seem to be exceptional.
Lange
D J
DJ
Department of Neurology, Columbia-Presbyterian Medical Center, New York, NY 10032-2603.
Trojaborg
W
W
McDonald
T D
TD
Blake
D M
DM
D000200:Action Potentials; D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D016472:Motor Neuron Disease / Q000503:physiopathology*; D009133:Muscular Atrophy / Q000503:physiopathology; D009431:Neural Conduction; D013997:Time Factors