Electrophysiologic features of abetalipoproteinemia: functional consequences of vitamin E deficiency. Academic Article uri icon

Overview

abstract

  • We performed electrophysiologic testing in 10 patients with abetalipoproteinemia (ABL). Peripheral nerve studies implied an axonal disorder. Visual evoked potentials demonstrated prolonged P100 latency in three patients and abnormal electroretinograms in six. Somatosensory evoked potentials indicated dorsal column dysfunction in eight patients. Brainstem auditory evoked potentials were normal. Findings were consistent with the known neuropathology of ABL and of experimental vitamin E deficiency. Stabilization or improvement in electrophysiologic findings occurred with vitamin E supplementation. Neurophysiologic tests document retinal, central somatosensory and peripheral nerve lesions in vitamin E deficiency and provide an objective indication of response to treatment.

publication date

  • May 1, 1986

Research

keywords

  • Abetalipoproteinemia
  • Peripheral Nerves
  • Peripheral Nervous System Diseases
  • Vitamin E Deficiency

Identity

Scopus Document Identifier

  • 0022532882

Digital Object Identifier (DOI)

  • 10.1212/wnl.36.5.669

PubMed ID

  • 3010179

Additional Document Info

volume

  • 36

issue

  • 5