Evoked Response Studies in Patients With Adrenoleukodystrophy and Heterozygous Relatives

Bhuwan P. Garg, Omkar N. Markand, William E. De Myer, Carroll Warren

Research output: Contribution to journalArticle

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Abstract

Brain-stem auditory evoked responses (BAERs) and somatosensory evoked responses (SERs) were studied in 3 patients and 7 female relatives in 3 families with adrenoleukodystrophy (ALD). All patients with ALD had abnormal BAERs and SERs, reflecting the central demyelination occurring in ALD. Four of the 7 female family members had abnormalities of SERs with or without abnormalities of BAERs, similar to those seen in the patients. We suggest that these abnormalities demonstrate the morphological effects of the ALD gene in the CNS and reflect the physiological alteration in the heterozygous females in families with ALD.

Original languageEnglish (US)
Pages (from-to)356-359
Number of pages4
JournalArchives of Neurology
Volume40
Issue number6
DOIs
StatePublished - Jun 1983

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ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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