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Electrophysiologic features of abetalipoproteinemia

Functional consequences of vitamin E deficiency

Departments of Neurology (Drs. Brin, Pedley, Lovelace, and Emerson), Ophthalmology (Drs. Gouras and MacKay). and Pediatrics (Dr. Levy), Columbia University College of Physicians and Surgeons; the Department of Medicine (Dr. Kayden), New York University School of Medicine, New York, NY; and the Departments of Preventive Medicine and Community Health, and Medicine (Dr. Baker), University of Medicine and Dentistry of New Jersey, Newark, NJ.

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. Neuro physiologic tests document retinal, central somatosensory and peripheral nerve lesions in vitamin E deficiency and provide an objective indication of response to treatment.

Address correspondence and reprint requests to Dr. Brin, The Neurological Institute, 710 West 168th Street, New York, NY 10032.

Supported in part by a grant from the Muscular Dystrophy Association and NIH RR00645–12. Dr. Brin was supported by a Peggy Engl Fellowship and a postdoctoral fellowship from the NINCDS NS-07654–01.

Accepted for publication September 20, 1985.

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