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Huntington's disease as a model for mood disorders. Clues from neuropathology and neurochemistry

C E Peyser1, S E Folstein

  • 1Department of Psychiatry, Johns Hopkins University School of Medicine, Baltimore, MD 21205.

Molecular and Chemical Neuropathology
|March 1, 1990
PubMed

Insights

Huntington's disease (HD) linked to mood disorders suggests brain pathophysiology. Examining the glutamate system in HD may reveal insights into idiopathic affective disorders.

Area of Science:

  • Neuroscience
  • Genetics
  • Psychiatry

Background:

  • Huntington's disease (HD) is an inherited neurodegenerative disorder.
  • Affective disorders (AfD) occur in 38% of HD patients, sometimes preceding neurological symptoms.
  • HD's clear neuropathology makes it a model for studying idiopathic AfD.

Purpose of the Study:

  • To explore the link between HD pathophysiology and AfD.
  • To investigate the role of the neostriatum and glutamate system in HD-related AfD.
  • To propose the HD model for understanding idiopathic AfD.

Main Methods:

  • Review of HD neuropathology, focusing on caudate atrophy and limbic system connections.
  • Examination of HD neurochemistry, particularly the excitotoxin hypothesis involving glutamate and NMDA receptors.
  • Analysis of preliminary neuropathological differences in HD patients with and without AfD.

Main Results:

  • Striking atrophy and neuronal loss in the HD neostriatum, especially the dorsal medial caudate, are implicated in AfD.
  • The excitotoxin hypothesis, involving glutamate and NMDA receptor dysfunction, is central to HD neurochemistry.
  • AfD in HD appears linked to specific brain pathophysiology rather than being a mere reaction to illness.

Conclusions:

  • Dysfunction of the dorsal medial caudate may underlie AfD in Huntington's disease.
  • The glutamate system, particularly NMDA receptors, warrants investigation in idiopathic affective disorders using the HD model.
  • HD serves as a valuable model for elucidating the biological underpinnings of idiopathic AfD.

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