Somatosensory evoked potentials in adrenomyeloneuropathy

Peter W. Kaplan, Ronald J. Tusa, Jo Ellen Rignani, Hugo W. Moser

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)1662-1667
Number of pages6
JournalNeurology
Volume48
Issue number6
DOIs
StatePublished - Jun 1997

ASJC Scopus subject areas

  • Clinical Neurology

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