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The Neuropsychiatry of Adult-Onset Adrenoleukodystrophy

Received August 27, 1998; revised February 16, 1999; accepted February 22, 1999. From the Departments of Psychiatry and Behavioural Neurosciences, and Medicine (Neurology), McMaster University Medical Centre, Hamilton, Ontario, Canada. Address correspondence to Dr. Rosebush, Department of Psychiatry, MUMC, 1200 Main St W, Hamilton, ON L8N 3Z5, Canada; e-mail: Rosebush{at}fhs.mcmaster.ca

Adrenoleukodystrophy is an inherited X-linked peroxisomal disorder that preferentially affects the adrenal cortex, testes, and brain and may occur at almost any age. Psychiatric symptomatology is present in many of the adult-onset cases reported in the literature and may be one of the earliest manifestations of the disease. The majority of patients with psychiatric disturbances have signs and symptoms typical of mania. Psychosis and cognitive impairment may also be prominent. Metabolic diseases such as adrenoleukodystrophy are probably underrecognized as a cause of psychiatric illness. Increased awareness of these disorders will lead to accurate diagnosis, appropriate treatment selection, and genetic counseling.

Key Words: Adrenoleukodystrophy, Adult-Onset • Genetic Disorders

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