Somatosensory evoked potentials in adrenomyeloneuropathy

Neurology
P W KaplanHugo W Moser

Abstract

Adrenomyeloneuropathy (AMN) is an X-linked metabolic disorder causing accumulation of very-long-chain fatty acids with multifocal nervous system demyelination of the peripheral nerves, spinal cord, and cerebrum. The extent to which the disorder affects upper versus lower limbs or peripheral versus CNS has not been electrophysiologically defined in a large population nor differentiated in men and women. To determine patterns of nervous system demyelination and define gender differences, we studied 83 AMN patients with short latency median and posterior tibial nerve somatosensory evoked potentials (SSEPs). Most women (10/16) had abnormal median SSEPs all involving central pathways, whereas most men (59/67) had abnormal median SSEPs involving both peripheral and central pathways. Tibial SSEPs were abnormal in both sexes (14/15 women, 67/67 men), with either peripheral or central pathway involvement. This study demonstrates the frequent widespread involvement of both peripheral nerve and central somatosensory pathways in men with AMN and the predominantly central involvement in women.

Citations

Jun 14, 2003·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Jean SchoenenAlain Maertens de Noordhout
Nov 20, 2002·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Stavros KorresEleftherios Ferekidis
Dec 8, 2009·Clinical Neurology and Neurosurgery·Hideyuki MatsumotoYoshikazu Ugawa
Jun 2, 2006·Journal of the Neurological Sciences·J P PillionH Shimizu
Sep 29, 1998·Arthritis and Rheumatism·D Y LeungH C Meissner

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