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Case Reports
. 2009 Feb;30(2):386-8.
doi: 10.3174/ajnr.A1282. Epub 2008 Oct 22.

Demonstration of cerebellar atrophy in neuroacanthocytosis of 2 siblings

T Katsube  1 , T Shimono, R Ashikaga, M Hosono, H Kitagaki, T Murakami
Affiliations
Case Reports

Demonstration of cerebellar atrophy in neuroacanthocytosis of 2 siblings

T Katsube et al. AJNR Am J Neuroradiol. 2009 Feb.

Abstract

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.

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Figures

Fig 1.
Fig 1.
Case 1. T2-weighted MR imaging examination of the brain. A, Axial image showing atrophy of bilateral striata and mildly increased signal intensity from the putamen (arrow). B, Sagittal image showing atrophy in the superior cerebellum (arrowhead).
Fig 2.
Fig 2.
Case 2. T2-weighted MR imaging examination of the brain. Atrophy of bilateral striata, mildly increased signal intensity of the putamen (arrow, A) and cerebellar atrophy (arrowhead, B) are apparent, as in case 1.
See this image and copyright information in PMC

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