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Neuropsychiatric Aspects of the Adult Variant of Tay-Sachs Disease

Received August 13, 1996; revised January 10, 1997; accepted January 13, 1997. From the Departments of Psychiatry and Medicine, Division of Neurology, and Biomedical Sciences, Division of Neuroscience, McMaster University Medical Centre, Hamilton, Ontario, Canada. Address correspondence to Dr. MacQueen, Department of Psychiatry, McMaster University, 1200 Main Street West, Hamilton, Ontario, L8N 3Z5, Canada.

Tay-Sachs disease (a G_M2 gangliosidosis) is an inherited neuronal storage disease that can affect individuals across the age spectrum. Psychosis is reported in 30% to 50% of adult-onset patients, and many are misdiagnosed with schizophrenia. Mood disorders are present in more than 25% and cognitive impairment in more than 20%. Treatment of psychosis with neuroleptics may not have a favorable risk/benefit ratio, but treatment with benzodiazepines or electroconvulsive therapy may be efficacious. Metabolic diseases such as gangliosidosis are probably under-recognized as causes of neuropsychiatric illness. Increased awareness of these disorders will lead to accurate diagnosis, appropriate treatment selection, and genetic counseling.

Key Words: Tay-Sachs Disease; Depression; Psychosis; Diagnosis; Gangliosidosis

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