Concurrent chronic motor axonal polyneuropathy and synaptic impairment of neuromuscular junction

Journal of Clinical Neuromuscular Disease
Giuliana GalassiFranco Valzania

Abstract

Polyneuropathies may exhibits clinical, electrophysiologic signs of neuromuscular junction impairment. Distal motor nerve terminals and neuromuscular junction contain pre or postsynaptically specific targets for circulating autoantibodies, if present in neuropathies. Motor nerve terminal blockade either reversible or permanent is a putative factor of muscle weakness. A 59-year-old patient exhibited oropharyngeal, facial, extremity weakness, fluctuating fatigability, and areflexia. Elecectrophysiologic studies showed purely motor axonal polyneuropathy. Thenar, facial slow rate repetitive stimulation revealed up to 47% decrement of compound muscle action potential size. Single fiber electromyography on voluntary activation confirmed increased jitter and impulse blocking in all muscles examined in one third of the fibers. Repeated testings for antibodies to gangliosides, acetylcholine, muscle tyrosine kinase receptors, voltage-gated calcium channels were negative. Oral pyridostigmine bromide improved bulbar symptoms. Pulse intravenous immunoglobulin, oral steroids, and azathioprine had steady benefit. Impairment of neuromuscular transmission if occurring in chronic axonal neuropathies highlights mechanisms and significance of neur...Continue Reading

References

Jun 1, 1982·Acta Neurologica Scandinavica·I RegevY Vardi
Oct 1, 1993·Journal of Neurology, Neurosurgery, and Psychiatry·N C NotermansF G Jennekens
Sep 1, 1996·Muscle & Nerve·D B Sanders, E V Stålberg
Jun 27, 1997·Journal of Neuroscience Methods·E Stålberg, J V Trontelj
Aug 26, 1998·Neuropediatrics·A J BarkovichP Evrard
Sep 8, 2001·Surgical Neurology·Y Kanpolat, A Savas
Jul 5, 2005·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·F SartucciL Murri
Apr 28, 2006·Acta Neurologica Scandinavica. Supplementum·A Vincent
Jun 30, 2006·Neuromuscular Disorders : NMD·M Baris BasloMarina Cuttini
Feb 28, 2007·Muscle & Nerve·Miharu SamurakiMasahito Yamada
Jan 16, 2009·Muscle & Nerve·Jasper R Daube, Devon I Rubin

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