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Laboratoire de Médecine Expérimentale, INSERM U-289 (Drs. Juncos, Hirsch, Malessa, and Agid), and Laboratoire de Neuropathologie R. Escourolle (Dr. Duyckaerts), Hôpital de la Salpêtrière, Paris, France; the Department of Neurology (Dr. Juncos), Emory University School of Medicine, Atlanta, GA; and the University of Texas Health Science Center (Dr. Hersh), Dallas, TX.
Using an antibody against choline acetyltransferase (ChAT), mesencephalic cholinergic cell nuclei were studied in autopsy material from 3 cases of progressive supranuclear palsy (PSP) and 4 controls. ChAT-immunoreactive neurons were quantified in sections that spanned the rostrocaudal extent of each nucleus. In PSP, there was a significant decrease in the number of neurons with detectable immunoreactivity for ChAT in and adjacent to the central gray substance in the following nuclei: the nucleus of Edinger-Westphal (69%); the rostra1 interstitial nucleus of the medial longitudinal fasciculus (97%); the interstitial nucleus of Cajal (78%). A cell loss was also evident in a group of neurons found in the deep layers of the superior colliculus (93%). In contrast, the estimated number of ChAT-immunoreactive cell bodies in cranial nerves III and IV, in the mesencephalic reticular formation, and in the parabigeminal nucleus was not different from that of controls. The results are compatible with the notion that, in PSP, there is a regionally selective destruction of cholinergic neurons.
Address correspondence and reprint requests to Dr. J. L. Juncos, Emory University School of Medicine, Neurology, PO Drawer V, Atlanta, GA 30322.
Supported by INSERM and NIH Grant AG0.5893. J.L.J. was supported by a grant from the Fondation pour la Recherche Médicale.
Received February 16, 1990. Accepted for publication in final form June 21. 1990.
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