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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 28:1606-1608, September 2007
DOI 10.3174/ajnr.A0603
© 2007 American Society of Neuroradiology

Case Report
PEDIATRICS

Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay: A Report of MR Imaging in 5 Patients

M.-H. Martin^a, J.-P. Bouchard^b, M. Sylvain^c, O. St-Onge^d and S. Truchon^d

^a Laval University, Québec City, Québec, Canada
^b Department of Neurological Sciences, C.H.A.-Hôpital de l'Enfant Jésus, Québec City, Québec, Canada
^c Department of Neurology (Pediatric), C.H.U.Q.-C.H.U.L., Québec City, Québec, Canada
^d Department of Radiology, C.H.U.Q.-C.H.U.L., Québec City, Québec, Canada

Please address correspondence to Marie-Helene Martin, MD, 1095-602 de l'Amérique-Française, Québec, PQ G1R 5P9, Canada; e-mail: mariehmartin{at}hotmail.com, mariehmartin{at}sympatico.ca

SUMMARY: We present findings on MR imaging in 5 patients with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). In the literature, early atrophy of the superior vermis as well as progressive atrophy of the cerebellar hemispheres and cervical cord was described. We found linear hypointensity on T2 and T2 fluid-attenuated inversion recovery–weighted images in the pons in all of our 5 patients.

This article has been cited by other articles:

				P. Van Damme, P. Demaerel, W. Spileers, and W. Robberecht Autosomal recessive spastic ataxia of Charlevoix-Saguenay Neurology, May 19, 2009; 72(20): 1790 - 1790. [Full Text] [PDF]