title: Neuropsychiatric aspects of adult-onset Tay-Sachs disease: two case reports with several new findings.
abstract: Deficiency of hexosaminidase A causes the GM2 gangliosidosis known as Tay-Sachs disease. It is now known that this condition has several late-onset variants that cause numerous neuropsychiatric disturbances. Early recognition is important because treatment with phenothiazines and heterocyclic antidepressants may worsen the course. The authors report two cases with several new findings, including prominent psychiatric symptoms without psychosis early in the course of the illness.
authors.affiliation: Department of Psychiatry, Columbia University College of Physicians and Surgeons, Columbia-Presbyterian Medical Center, New York, New York 10034.
authors.forename: G I
authors.initials: GI
authors.lastname: Hurowitz
authors.forename: J M
authors.initials: JM
authors.lastname: Silver
authors.forename: M F
authors.initials: MF
authors.lastname: Brin
authors.forename: D T
authors.initials: DT
authors.lastname: Williams
authors.forename: W G
authors.initials: WG
authors.lastname: Johnson
mesh_terms: D000328:Adult; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D002884:Chromosomes, Human, Pair 15; D005260:Female; D054818:Hexosaminidase A; D006801:Humans; D007585:Jews / Q000235:genetics; D008094:Lithium / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D001523:Mental Disorders / Q000209:etiology* / Q000235:genetics; D011433:Propranolol / Q000008:administration & dosage / Q000627:therapeutic use; D013661:Tay-Sachs Disease / Q000188:drug therapy / Q000201:enzymology* / Q000523:psychology*; D001619:beta-N-Acetylhexosaminidases / Q000032:analysis / Q000172:deficiency*
chemical_list: D008094:Lithium; D011433:Propranolol; D054818:Hexosaminidase A; D001619:beta-N-Acetylhexosaminidases