Tohoku J. Exp. Med., 2001, 195 (2)

Case Report

Two Cases of Japanese CADASIL with Corpus Callosum Lesion

KAORI IWATSUKI, TETSURO MURAKAMI, YASUHIRO MANABE, HISASHI NARAI,
HITOSHI WARITA, TAKESHI HAYASHI and KOJI ABE

Department of Neurology, Graduate School of Medicine and Dentistry,
Okayama University, Okayama 700-8558

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is a rare hereditary stroke disease. In the present study, a Japanese CADASIL family was first reported with missense mutation of Arg141Cys of Notch3 and a unique lesion of corpus callosum. Upon neuropsychological examination, our case 1 showed only right-handed constructional apraxia associated with corpus callosum lesion. No other callosal disconnection signs were present. Sagittal T2 weighted image of case 1 showed multiple small lesions along with the pericallosal branches from the truncus to the posterior part of the splenium in the corpus callosum. Although detailed mapping of the corpus callosum for functional fractionation in humans remains incomplete, the constructional apraxia on the right may be related to callosal dysfunction from the truncus to the posterior part of the splenium in the corpus callosum.

Keywords —— CADASIL; corpus callosum; constructional apraxia

© 2001 Tohoku University Medical Press

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Tohoku J. Exp. Med., 2001, 195, 135-140

Address for reprints: Yasuhiro Manabe, M.D., Department of Neurology, Graduate School of Medicine and Dentistry, Okayama University, 2-5-1 Shikata-cho, Okayama 700-8558, Japan.

e-mail: manabe@cc.okayama-u.ac.jp