Neuropathological Classification of Huntington's Disease (original) (raw)

Journal Article

,

Jean-Paul Vonsattel, M.D.

C. S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology Laboratories,

Massachusetts General Hospital

Department of Neurology-Neuropathology,

Harvard Medical School

, Boston, Massachusetts

Ralph Lowell Laboratories, Mailman Research Center,

McLean Hospital and Harvard Medical School

, Belmont, Massachusetts

Search for other works by this author on:

,

Department of Neurology,

Boston University Medical School and Massachusetts General Hospital

, Harvard Medical School, Boston, Massachusetts

Search for other works by this author on:

,

Ralph Lowell Laboratories, Mailman Research Center,

McLean Hospital and Harvard Medical School

, Belmont, Massachusetts

Search for other works by this author on:

,

C. S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology Laboratories,

Massachusetts General Hospital

Department of Neurology-Neuropathology,

Harvard Medical School

, Boston, Massachusetts

Search for other works by this author on:

,

Ralph Lowell Laboratories, Mailman Research Center,

McLean Hospital and Harvard Medical School

, Belmont, Massachusetts

Search for other works by this author on:

Edward P. Richardson, Jr., M.D.

C. S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology Laboratories,

Massachusetts General Hospital

Department of Neurology-Neuropathology,

Harvard Medical School

, Boston, Massachusetts

Correspondence to: Dr. Edward P. Richardson, Jr., Neuropathology Laboratory, Massachusetts General Hospital, Boston, MA 02114.

Search for other works by this author on:

Supported in part by NINCDS Grant 16367 (Huntington's Disease Center Without Walls) (RHM, EDB, EPR), NIMH/NINCDS 31862 and Hereditary Disease Foundation (Brain Tissue Resource Center) (TJS), The Fonds National Suisse de la Recherche Scientifique, Switzerland (JPV), the Alexander von Humboldt-Stiftung, Bonn, Federal Republic of Germany (EPR, during sabbatical leave at the Neuropathological Institute, Free University of Berlin, Director: Prof. J. Cervós-Navarro), and the Massachusetts Huntington Disease Foundation of America (RHM).

Author Notes

Published:

01 November 1985

• PDF
• Split View
• • Article contents
  • Figures & tables
  • Video
  • Audio
  • Supplementary Data
• Cite

Cite

Jean-Paul Vonsattel, Richard H. Myers, Thomas J. Stevens, Robert J. Ferrante, Edward D. Bird, Edward P. Richardson, Neuropathological Classification of Huntington's Disease, Journal of Neuropathology & Experimental Neurology, Volume 44, Issue 6, November 1985, Pages 559–577, https://doi.org/10.1097/00005072-198511000-00003
Close

Navbar Search Filter Mobile Enter search term Search

Abstract

In postmortem brain specimens from 163 clinically diagnosed cases of Huntington's disease (HD) the striatum exhibited marked variation in the severity of neuropathological involvement. A system for grading this severity was established by macroscopic and microscopic criteria, resulting in five grades (0–4) designated in ascending order of severity. The grade correlates closely with the extent of clinical disability as assessed by a rating scale. In five cases of clinically diagnosed HD there were no discernible neuropathological abnormalities (grade 0), suggesting that the anatomical changes lag behind the development of clinical abnormalities. In eight cases, neuropathological changes could only be recognized microscopically (grade 1). The earliest changes were seen in the medial paraventricular portions of the caudate nucleus (CN), in the tail of the CN, and in the dorsal part of the putamen. Counts of neurons in the CN reveal that 50% are lost in grade 1 and that 95% are lost in grade 4; astrocytes are greatly increased in grades 2–4. These studies indicate that analyses of the CN in grade 4 would reflect mainly its astrocytic composition with a component of remote neurons projecting to the striatum. Because of the relative preservation of the lateral half of the head of the CN in grades 1–2, these regions would reflect early cellular and biochemical changes in HD.

This content is only available as a PDF.

Author notes

Supported in part by NINCDS Grant 16367 (Huntington's Disease Center Without Walls) (RHM, EDB, EPR), NIMH/NINCDS 31862 and Hereditary Disease Foundation (Brain Tissue Resource Center) (TJS), The Fonds National Suisse de la Recherche Scientifique, Switzerland (JPV), the Alexander von Humboldt-Stiftung, Bonn, Federal Republic of Germany (EPR, during sabbatical leave at the Neuropathological Institute, Free University of Berlin, Director: Prof. J. Cervós-Navarro), and the Massachusetts Huntington Disease Foundation of America (RHM).

© 1985 by the American Association of Neuropathologists

Topic:

• huntington's disease
• astrocytes
• autopsy
• caudate nucleus
• corpus striatum
• neurons
• putamen
• brain
• disability

Citations

Views

Altmetric

Metrics

Total Views 11,785

3,300 Pageviews

8,485 PDF Downloads

Since 12/1/2016

×

Email alerts

Citing articles via

• Latest

• Most Read

• Most Cited

More from Oxford Academic