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The adrenoleukomyeloneuropathy complex

Expression in four generations

From the Departments of Neurology, Mayo Clinic, Rochester, MN (Dr. O'Neill), and the University of Michigan Medical Center, Ann Arbor, MI (Drs. Marmion and Feringa).

varying combinations of leukodystrophy, myeloneuropathy, peripheral neuropathy, and primary Addison disease were identified in 14 members of four generations of a kindred of 49 persons. Of these, 26 persons from three generations were evaluated clinically. We propose that adrenoleukomyeloneuropathy (ALMN) is a rare, progressive, presumably biochemical disorder of uncertain inheritance. ALMN is expressed clinically by dysfunction of the central nervous system, peripheral nervous system, and endocrine system. We stress that suspicion of this disorder be high in evaluating uncertain neurologic disorders, and that identification of one manifestation of this complex should alert the clinician to the possibility of a different expression of the same complex in other family members.

Address correspondence and reprint requests to Dr. O'Neill, Department of Neurology, Mayo Clinic, Rochester, MN 55901.

Funding for this project was possible through the Clinical Research Center, University of Michigan Medical Center (NIH Grant No. 5-M01-RR00042 ‘Multicategorical Clinical Research Center‘).

Presented at the thirty-first annual meeting of the American Academy of Neurology, Chicago, IL, April 1979.

Accepted for publication April 18, 1980.

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