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J Neurol Neurosurg Psychiatry 2013 Nov; 84(11):e2
Authors: Amy Davidson, Cheryl Longman, Maria Farrugia
Southern General Hospital.
A 49 year old gentleman presented to Neurology with a 10 year history of deteriorating mobility. He described an evolving bilateral foot drop from his mid-thirties, with progressive symptoms affecting his left hand, manifesting as difficulties performing fine tasks such as snapping his fingers. He also had bilateral sensorineural deafness. He was known to cardiology with a tachy-brady syndrome which had evolved into persistent atrial fibrillation not requiring a pacemaker. His mother had Alzheimer's disease, with a "shuffling gait". His brother suffered from childhood polio. On examination, he was dysarthric, with wasting and weakness of the interossei and thenar eminence in both hands. There was wasting of the calf muscles and of tibialis anterior, with normal arched feet. Weakness was present at hips, knees and ankles with marked symmetrical weakness of ankle dorsiflexion bilaterally.
PMID: 24108974 [PubMed - as supplied by publisher]