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This is a case report cf late onset 'Fay-Sachs disease diagnosed in a 14-year-old male non-Jewish adolescent who presented in a psy chotic and catatonic state. The objective is to emphasize that Toy-Sachs disease can present with psychiatric symptoms, with a variety of phenotypes from infancy to adulthood, and can offec! individuals other than Ashkenazi J ews. Amphiphilic drugs, includingphenothiazine and tricyclic antidepressants, mayworsen the courseof the illness. Resistant catatonia can respond to an extended trial ofhig]: dose lorazepam,

Late Onset Tay-Sachs Disease Presenting as a Brief Psychotic Disorder with Catatonia: A Case Report and Review of the Literature