Neuropsychiatric aspects of the adult variant of Tay-Sachs disease.

J Neuropsychiatry Clin Neurosci. 1998 Winter;10(1):10-9.  MacQueen GM, Rosebush PI, Mazurek MF.

Source Department of Psychiatry, McMaster University Medical Centre, Hamilton, Ontario, Canada.

Abstract:  Tay-Sachs disease (a GM2 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. PMID: 9547461 [PubMed – indexed for MEDLINE]

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