Neuropsychiatric aspects of adult-onset Tay-Sachs disease: two case reports with several new findings

G I Hurowitz; J M Silver; M F Brin; D T Williams; W G Johnson

doi:10.1176/jnp.5.1.30

Neuropsychiatric aspects of adult-onset Tay-Sachs disease: two case reports with several new findings

J Neuropsychiatry Clin Neurosci. 1993 Winter;5(1):30-6. doi: 10.1176/jnp.5.1.30.

Authors

G I Hurowitz¹, J M Silver, M F Brin, D T Williams, W G Johnson

Affiliation

¹ Department of Psychiatry, Columbia University College of Physicians and Surgeons, Columbia-Presbyterian Medical Center, New York, New York 10034.

PMID: 8428133
DOI: 10.1176/jnp.5.1.30

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.

Publication types

Case Reports

MeSH terms

Adult
Chromosome Aberrations / genetics
Chromosome Disorders
Chromosomes, Human, Pair 15
Female
Hexosaminidase A
Humans
Jews / genetics
Lithium / administration & dosage
Lithium / therapeutic use
Male
Mental Disorders / etiology*
Mental Disorders / genetics
Propranolol / administration & dosage
Propranolol / therapeutic use
Tay-Sachs Disease / drug therapy
Tay-Sachs Disease / enzymology*
Tay-Sachs Disease / psychology*
beta-N-Acetylhexosaminidases / analysis
beta-N-Acetylhexosaminidases / deficiency*

Substances

Lithium
Propranolol
Hexosaminidase A
beta-N-Acetylhexosaminidases