AJDRAJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 2009;30:386.

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American Journal of Neuroradiology
DOI 10.3174/ajnr.A1282

BRAIN

Demonstration of Cerebellar Atrophy in Neuroacanthocytosis of 2 Siblings

T. Katsube, T. Shimono, R. Ashikaga, M. Hosono, H. Kitagaki and T. Murakami

From the Department of Radiology (T.K., T.S., R.A., M.H., T.M.), Kinki University School of Medicine, Osaka-Sayama, Japan; and Department of Radiology (H.K.), Shimane University School of Medicine, Izumo City, Shimane-Ken, Japan.

Please address correspondence to Takashi Katsube, MD, Kinki University School of Medicine, 377-2, Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan; e-mail: katsube{at}radiol.med.kindai.ac.jp

SUMMARY: Neuroacanthocytosis is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis in the peripheral blood. Clinical manifestations of this disorder resemble those of Huntington disease (HD). Neuroimaging features of neuroacanthocytosis are atrophy and signal intensity change of the striata on MR imaging, as in HD. We report herein the cases of 2 siblings with neuroacanthocytosis showing cerebellar atrophy as well as atrophy and signal intensity changes of striata.






Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X