Rhythmic Muscle Twitching in Primary Lateral Sclerosis


A 43-year-old man with slowly progressive motor weakness, difficulty swallowing (dysphagia), and slurred speech (dysarthria) over 30 years was diagnosed with an atypical form of primary lateral sclerosis (PLS) on the basis of upper motor neuron signs on physical examination, including increased deep tendon reflexes in all extremities, the Babinski reflex on the right side, and tap-elicited sustained clonus of the biceps and triceps brachii, as shown in this video.

Clonus is involuntary, rhythmic muscle contractions caused by damage to the upper motor neurons. Sustained clonus is usually encountered in the knee or ankle and is much less common in the wrist and jaw. Tap-elicited clonus is rare in the biceps and triceps brachii, and this patient’s clonus did not need continuous passive stretch of the muscle to be sustained, as is usually the case.

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