Motor‐evoked responses in primary lateral sclerosis

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Abstract
Primary lateral sclerosis (PLS) may be distinguished on the basis of clinical and pathological features from amyotrophic lateral sclerosis (ALS). The former is featured by a much longer clinical course, exclusively upper motor neuron findings, losses of precentral pyramidal neurons, and preservation of anterior horn cells. Electrophsiological studies of 7 PLS cases have shown normal peripheral motor conduction, absent or very delayed motor‐evoked poteintials, the occasional late development of denervation activity in distal muscles, and normal somatosensory‐evoked potentials.