Neuropsychiatric aspects of the adult variant of Tay-Sachs disease

J Neuropsychiatry Clin Neurosci. Winter 1998;10(1):10-9. doi: 10.1176/jnp.10.1.10.


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.

Publication types

  • Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Male
  • Tay-Sachs Disease / genetics
  • Tay-Sachs Disease / physiopathology*
  • Tay-Sachs Disease / psychology*