HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Goto, I. Articles by Kuroiwa, Y. Search for Related Content PubMed PubMed Citation Articles by Goto, I. Articles by Kuroiwa, Y.

Dentatorubro-pallidoluysian degeneration

Clinical, neuro-ophthalmologic, biochemical, and pathologic studies on autosomal dominant form

Department of Neurology (Drs. Goto, Tobimatsu, Hosokawa, Shibasaki and Kuroiwa) Department of Neurology and Department of Neuropathology (Dr. Ohta), Neurological Institute, Kyushu University, Fukuoka, Japan.

Four cases in two generations of a Japanese family are described as affected by an autosomal dominant-inherited cerebellar ataxia, pyramidal and extrapyramidal signs, and abnormal ocular movements, resembling Machado-Joseph disease.

The neuro-ophthalmologic examination suggests the involvement of multiple motor control systems.

The neuropathologic examination of one case showed neuronal loss in the subthalamic nuclei, pallidum, dentate nuclei of cerebellum, and red nuclei, which is distinct from Machado-Joseph disease.

Address correspondence and reprint requests to Dr. Go to, Department of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University 60, 3–11 Maidashi, Higashi-ku, Fukuoka, Japan 812.

Accepted for publication October 29, 1981.

This article has been cited by other articles:

				T. W. Farmer, M. S. Wingfield, S. A. Lynch, F. S. Vogel, C. Hulette, B. Katchinoff, and P. L. Jacobson Ataxia, Chorea, Seizures, and Dementia: Pathologic Features of a Newly Defined Familial Disorder Arch Neurol, July 1, 1989; 46(7): 774 - 779. [Abstract] [PDF]

				J. Kitamura, Y. Kubuki, K. Tsuruta, T. Kurihara, and S. Matsukura A New Family With Joseph Disease in Japan: Homovanillic Acid, Magnetic Resonance, and Sleep Apnea Studies Arch Neurol, April 1, 1989; 46(4): 425 - 428. [Abstract] [PDF]