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Suzanne L. Davis, MB, ChB; Michael J. Aminoff, MD; Bruce O. Berg, MD

Arch Neurol. 1985;42(2):156-160.

Abstract
• Brain-stem auditory evoked potentials (BAEPs) were recorded in 23 children who had signs of brain-stem or cerebellar dysfunction. In patients with brain-stem gliomas, BAEPs were abnormal in all except one, in whom involvement of the brain-stem auditory pathway was limited to the midbrain tectum. The BAEPs were normal in neuronal ceroid lipofuscinosis, but abnormal bilaterally in inheritable leukoencephalopathies. All patients with Leigh's encephalopathy had BAEP abnormalities; in two, abnormalities occurred before the appearance of lesions on computed tomographic scan. Patients with Friedreich's ataxia and giant axonal dystrophy had abnormal BAEPs, but the test was normal in a child with similar neurologic findings with vitamin E deficiency. Patients with diffuse metabolic encephalopathies had variable findings. Thus, BAEP abnormalities are nonspecific for various disease processes but are frequently seen in neoplastic and neurodegenerative diseases, with primary white matter or extensive brain-stem involvement.

Author Affiliations
From the Department of Neurology, University of California at San Francisco.

Footnotes
Accepted for publication Feb 22, 1984.

Read at a meeting of the American Electroen-cephalography Society, New Orleans, Oct 21, 1983.

Reprint requests to Department of Neurology, Room M-794, University of California at Davis, 4301 X St, Sacramento, CA 95817 (Dr Davis).

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