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A 57-year-old man presented with 6 months’ history of gradual-onset painless fatigable proximal weakness, described as difficulty going up and down the stairs, as well getting out of low chairs. This was unaccompanied by neck/back pain, and sensory, bulbar or respiratory complaints. His constitutional symptoms were significant for fatigue unaccompanied by sleep or mood disturbances, postural lightheadedness, dry mouth and 50 lb unintentional weight loss. Clinical examination showed orthostatic hypotension, mild bilateral hip flexor weakness and generalised hyporeflexia–areflexia, which improved with brief exercise (video 1). Laboratory tests done at an outside hospital showed normal creatine kinase (CK) and thyroid hormone levels.
The differential diagnosis for a pure motor disorder without cranial nerve or sphincter involvement, neck or back …
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