Electrophysiologic features of abetalipoproteinemia: Functional consequences of vitamin E deficiency

Mitchell F. Brin, Timothy A. Pedley, Robert E. Lovelace, Ronald G. Emerson, Peter Gouras, Cynthia Mackay, Herbert J. Kayden, Joseph Levy, Herman Baker

Research output: Contribution to journalArticlepeer-review

61 Scopus citations

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.

Original languageEnglish
Pages (from-to)669-673
Number of pages5
JournalNeurology
Volume36
Issue number5
DOIs
StatePublished - May 1986
Externally publishedYes

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