Locus coeruleus involvement in Huntington's disease
R. M. Zweig, C. A. Ross, J. C. Hedreen, C. Peyser, J. E. Cardillo, S. E. Folstein and D. L. Price
Department of Internal Medicine, Veterans Affairs Medical Center, Reno, NV 89520.
Numbers and areas of neuronal profiles from sections of brain stem at
specific anatomic levels of the locus coeruleus and the dorsal raphe
nucleus were measured in 33 patients with Huntington's disease and in 23
age-matched control subjects. Results from the Huntington's disease cases
were correlated with severity of neostriatal atrophy and with
systematically collected quantitative clinical data. Among the patients
with Huntington's disease, lower locus coeruleus neuronal counts, reduced
neuronal areas, and reduced locus coeruleus length (distance between
rostral and caudal levels) were associated with features of advanced
disease, including severity of neostriatal atrophy, severity of dementia,
duration of illness, and severity of motor impairment and activities of
daily living impairment. By contrast, there was no evidence of neuronal
pathology within the dorsal raphe nucleus in Huntington's disease.
Pathologic changes in the locus coeruleus may relate to some of the
clinical manifestations of Huntington's disease.
