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AJNR - American Journal of Neuroradiology

Published ahead of print on October 22, 2008
doi: 10.3174/ajnr.A1282

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American Journal of Neuroradiology 30:386-388, February 2009
© 2009 American Society of Neuroradiology

Case Report
BRAIN

Demonstration of Cerebellar Atrophy in Neuroacanthocytosis of 2 Siblings

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

^a Department of Radiology, Kinki University School of Medicine, Osaka-Sayama, Japan
^b Department of Radiology, 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.