May 1987

Adrenoleukodystrophy Carrier State Presenting as a Chronic Nonprogressive Spinal Cord Disorder

Author Affiliations

From the Edward Mallinckrodt Department of Pediatrics (Dr Noetzel) and the Department of Neurology and Neurological Surgery (Drs Noetzel and Landau), Washington University School of Medicine, St Louis, and the John F. Kennedy Institute and the Departments of Pediatrics and Neurology, The Johns Hopkins University School of Medicine, Baltimore (Dr Moser).

Arch Neurol. 1987;44(5):566-567. doi:10.1001/archneur.1987.00520170092031

• Females who are heterozygous for adrenoleukodystrophy (ALD) can be identified biochemically. Since most carriers for this disease are asymptomatic, their diagnosis usually occurs only after neurologically abnormal male relatives have been investigated. We describe a woman with a chronic nonprogressive spinal cord syndrome secondary to the ALD heterozygote state. The neurologic condition in this patient suggests that the diagnosis of ALD should be considered in women with evidence of white matter disease.