The diagnostic dilemma of progressive muscular atrophy.

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The diagnostic dilemma of progressive muscular atrophy.

J Coll Physicians Surg Pak. 2015 Feb;25(2):149-50

Authors: Ayaz SB, Matee S, Gill ZA, Khan AA

Abstract
Progressive muscle atrophy is a rare subtype of motor neuron disease that affects only the lower motor neurons and presents as asymmetrical rapidly progressive muscle weakness, atrophy and normal sensations. The diagnostic electrophysiological findings are denervation potentials in three out of four body segments (bulbar, cervical, thoracic and lumbosacral). The disease is fatal and the management is supportive. We present the report of a 45-year-old female patient who presented with unilateral foot drop and rapidly progressed to profound weakness in muscles of all limbs, neck and back along with dysarthria and dysphagia. She had been operated twice for suspected cervical and lumbosacral intervertebral disc herniations and ultimately guided in right direction after muscle biopsy, nerve conduction studies and electromyography.

PMID: 25703763 [PubMed - indexed for MEDLINE]

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