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 Citation Map 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 Takahashi, H. Articles by Ikuta, F. Search for Related Content PubMed PubMed Citation Articles by Takahashi, H. Articles by Ikuta, F.

Hereditary dentatorubral-pallidoluysian atrophy

Clinical and pathologic variants in a family

From the Department of Pathology (Drs. Takahashi, Ohama, Takeda, Nakashima, Makifuchi, and Ikuta), Brain Research Institute, and the Department of Psychiatry (Dr. Naito), School of Medicine, Niigata University, Niigata, Japan.

We describe a family showing dentatorubral-pallidoluysian atrophy. Three patients appeared through three successive generations and displayed a wide variety of clinical pictures. The male proband with onset in childhood showed progressive myoclonus epilepsy syndrome. The father experienced cerebellar ataxia, myoclonus, and mild dementia starting in middle age; the paternal grandmother had progressive symptoms of cerebellar ataxia, choreiform movements, and dementia, but neither myoclonus nor epilepsy in senescence. Neuropathologic examination of two patients, the proband and the paternal grandmother, revealed combined degeneration of the dentatorubral and pallidoluysian systems and obvious degeneration involving the striatum in the proband and the cerebellar cortex in the grandmother. The present study indicates that this disease can include many clinical and pathologic variants even in the same family.

Address correspondence and reprint requests to Dr. Takahashi, Department of Pathology, Brain Research Institute, Niigata University, Asahimachi 1, Niigata 951, Japan.

Received September 16,1987. Accepted for publication in final form December 1,1987.

Supported in part by a research grant for CNS degenerative disease from the Ministry of Health and Welfare of Japan.

This article has been cited by other articles:

				E. Munoz, J. Campdelacreu, I. Ferrer, M. J. Rey, A. Cardozo, B. Gomez, and E. Tolosa Severe Cerebral White Matter Involvement in a Case of Dentatorubropallidoluysian Atrophy Studied at Autopsy Arch Neurol, June 1, 2004; 61(6): 946 - 949. [Abstract] [Full Text] [PDF]

				E L Sampson, J D Warren, and M N Rossor Young onset dementia Postgrad. Med. J., March 1, 2004; 80(941): 125 - 139. [Abstract] [Full Text] [PDF]

				I-H. Lee, B.-W. Soong, Y.-C. Lu, and Y.-C. Chang Dentatorubropallidoluysian Atrophy in Chinese Arch Neurol, November 1, 2001; 58(11): 1905 - 1908. [Abstract] [Full Text] [PDF]

				Y. Takeuchi, H. Matsushita, H. Sakai, H. Kawano, and M. Ochi Developmental Changes in Cerebrospinal Fluid Concentrations of Monoamine-Related Substances in Patients With Dentatorubral-Pallidoluysian Atrophy J Child Neurol, February 1, 2001; 16(2): 79 - 82. [Abstract] [PDF]

				E Munoz, M Mila, A Sanchez, P Latorre, A Ariza, M Codina, F Ballesta, and E Tolosa Dentatorubropallidoluysian atrophy in a Spanish family: a clinical, radiological, pathological, and genetic study J. Neurol. Neurosurg. Psychiatry, December 1, 1999; 67(6): 811 - 814. [Abstract] [Full Text] [PDF]

				S.H. Subramony Topical Review: Clinical Aspects of Hereditary Ataxias J Child Neurol, September 1, 1995; 10(5): 353 - 362. [Abstract] [PDF]

				W. E. Reichman and J. L. Cummings Diagnosis of Rare Dementia Syndromes: An Algorithmic Approach J Geriatr Psychiatry Neurol, April 1, 1990; 3(2): 73 - 84. [Abstract] [PDF]