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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
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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.
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).