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Washington University Experience | NEURODEGENERATION | Polyglucosan Body Disease (PGBD) | 4A0 Case 4 History
Case 4 History The patient is a 77 year old man with a two year history of progressive distal lower extremity weakness, with development of a left 'foot drop' in late 2006, as well as upper extremity and back weakness. Electromyographic examination yielded evidence for distal axonal sensory motor polyneuropathy, supported by evidence of acute and chronic denervation in distal leg muscles. The changes in distal latency and conduction velocity were not sufficiently severe to suggest a primary demyelinating neuropathy and were thought to be secondary to axon loss. Laboratory studies are significant for a low serum IgA level, a high serum IgG level, and a monoclonal lambda peak on serum protein electrophoresis. Bone survey shows no osteolytic foci. Magnetic resonance imaging of the spine shows no spinal canal stenosis. Operative procedure: Left gastrocnemius muscle biopsy and left sural nerve biopsy.
