title: Post-partum psychosis in adult GM2 gangliosidosis. A case report.
abstract: Adult hexosaminidase A deficiency is a form of GM2 gangliosidosis with autosomal recessive inheritance. Only 35 cases (mostly among Ashkenazic Jews) have been reported worldwide. Symptoms include, in a third of the cases, psychosis. A 27-year-old sufferer with no prior psychiatric history, developed a post-partum psychosis, with affective and hebephrenic components, 3 days following her first delivery. She responded to lithium within 10 days of initiating treatment; the full episode lasted 1 month. We conclude that lithium is the preferred treatment for psychosis in such adult patients, especially in light of possible long-term neurological deterioration caused by phenothiazines. Ashkenazic Jews with atypical neurological syndromes presenting with psychosis should be tested for hexosaminidase A deficiency.
authors.affiliation: Department of Psychiatry, Jerusalem Mental Health Center.
authors.forename: P
authors.initials: P
authors.lastname: Lichtenberg
authors.forename: R
authors.initials: R
authors.lastname: Navon
authors.forename: E
authors.initials: E
authors.lastname: Wertman
authors.forename: H
authors.initials: H
authors.lastname: Dasberg
authors.forename: B
authors.initials: B
authors.lastname: Lerer
mesh_terms: D000328:Adult; D005260:Female; D005678:G(M2) Ganglioside; D005733:Gangliosidoses / Q000150:complications / Q000523:psychology*; D054818:Hexosaminidase A; D006801:Humans; D008094:Lithium / Q000627:therapeutic use; D016651:Lithium Carbonate; D011247:Pregnancy; D011618:Psychotic Disorders / Q000188:drug therapy / Q000209:etiology*; D011644:Puerperal Disorders / Q000188:drug therapy / Q000209:etiology*; D001619:beta-N-Acetylhexosaminidases / Q000172:deficiency
chemical_list: D005678:G(M2) Ganglioside; D016651:Lithium Carbonate; D008094:Lithium; D054818:Hexosaminidase A; D001619:beta-N-Acetylhexosaminidases