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Brain Advance Access first published online on October 12, 2006
This version published online on October 24, 2006
Brain, doi:10.1093/brain/awl243
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© 2006 The Author(s) This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commerical License (http://creativecommons.org/licenses/by-nc/2.0/uk/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received May 5, 2006
Revised July 12, 2006
Accepted August 4, 2006

Article

Striosomes and mood dysfunction in Huntington's disease

Lynette J. Tippett 1 *, Henry J. Waldvogel 2, Sally J. Thomas 1, Virginia M. Hogg 1, Willeke van Roon-Mom 2, Beth J. Synek 3, Ann M. Graybiel 4, and Richard L. M. Faull 2

1 Department of Psychology, The University of Auckland, Auckland, New Zealand
2 Department of Anatomy with Radiology, The University of Auckland, Auckland, New Zealand
3 Department of Forensic Pathology, Auckland City Hospital, Auckland, New Zealand
4 Department of Brain and Cognitive Sciences and the McGovern Institute for Brain Research, Massachusetts Institute of Technology, Cambridge, MA, USA

* To whom correspondence should be addressed.
Lynette J. Tippett, E-mail: l.tippett{at}auckland.ac.nz


  Abstract

Variable phenotype is common in neurological disorders with single-gene inheritance patterns. In Huntington's disease, mood and cognitive symptoms are variably co-expressed with motor symptoms. There is also variable degeneration of neurons in the two major neurochemical compartments of the striatum, the striosomes and the extrastriosomal matrix. To determine whether the phenotypic variability in Huntington's disease is related to this compartmental organization, we carried out a double-blind study in which we used GABAA receptor immunohistochemistry to analyse the status of striosomes and matrix in the brains of 35 Huntington's disease cases and 13 control cases, and collected detailed data on the clinical symptomatology expressed by the patients from family members and records. We report here a significant association between pronounced mood dysfunction in Huntington's disease patients and differential loss of the GABAA receptor marker in striosomes of the striatum. This association held for both clinical onset and end-stage assessments of symptoms. The cases with accentuated striosome abnormality further exhibited later onset age, lower disease grade and lower CAG repeat length in the HD gene. We found no independent association, however, between CAG repeat length or age of onset and mood dysfunction. We suggest that variation in clinical symptomatology in Huntington's disease is associated with variation in the relative abnormality of GABAA receptor expression in the striosome and matrix compartments of the striatum, and that striosome-related circuits may modulate mood functioning.

Keywords: striosomes, striatum; neurological disorder; Huntington's disease; mood symptoms.
This new version of the article corrects several incorrect listings of "Huntingdon's disease" to the correct "Huntington's disease". The publisher apologizes for the original error.
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