TY - JOUR
T1 - Dentatorubro-pallidoluysian degeneration
T2 - Clinical, neuro-ophthalmologic, biochemical, and pathologic studies on autosomal dominant form
AU - Goto, Ikuo
AU - Tobimatsu, Shozo
AU - Ohta, Michiya
AU - Hosokawa, Shinichi
AU - Shibasaki, Hiroshi
AU - Kuroiwa, Yoshigoro
PY - 1982/12
Y1 - 1982/12
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0020334692&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0020334692&partnerID=8YFLogxK
U2 - 10.1212/wnl.32.12.1395
DO - 10.1212/wnl.32.12.1395
M3 - Article
C2 - 6890648
AN - SCOPUS:0020334692
VL - 32
SP - 1395
EP - 1399
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 12
ER -