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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 24:825-828, May 2003
© 2003 American Society of Neuroradiology

PEDIATRICS

MR Imaging Features in Marinesco-Sjögren Syndrome: Severe Cerebellar Atrophy Is Not an Obligatory Finding

Anke Reinhold^a,b, Ianina Scheer^c, Rüdiger Lehmann^d, Luitgard M. Neumann^b, Theodor Michael^a, Raymonda Varon^b and Arpad von Moers^a

^a the Clinic for Pediatric Neurology, Charité Campus Mitte; Berlin, Germany
^b the Institute for Human Genetics, Charité Campus Mitte; Berlin, Germany
^c the Institute for Radiology, Abteilung Pädiatrische Radiologie, Charité Campus Mitte; Berlin, Germany
^d the Charité Campus Virchow; and the Institut für Radiologie, Abteilung Neuroradiologie, Charité Campus Mitte; Berlin, Germany

Address reprint requests to Dr Arpad von Moers, Klinik für Pädiatrie m.S. Neurologie, Charité Campus Virchow, Augustenburger Platz.1, 13353 Berlin, Germany

BACKGROUND AND PURPOSE: Cerebellar atrophy is considered the most prominent neuroradiologic finding in Marinesco-Sjögren syndrome (MSS). Our purpose was to investigate this neuroradiologic feature in a series of patients with MSS.

METHODS: Five patients with MSS (age range, 5–19 years) underwent native MR imaging of the brain. The findings were assessed with particular attention to the cerebellum and the supratentorial structures.

RESULTS: Only two patients had slight cerebellar atrophy; the cerebellum was normal in size and configuration in the other patients. Additional supratentorial findings were present in some of the patients, with an apparently small anterior pituitary gland in two and the absence of the posterior pituitary bright spot in three of the patients.

CONCLUSION: Cerebellar atrophy is not an obligatory finding in MSS, and almost normal cranial MR imaging results are compatible with the diagnosis. Morphologic changes of the pituitary gland seem to be common in patients with MSS and are not associated with endocrine dysfunction.

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				I. Harting, A. Blaschek, N. I. Wolf, A. Seitz, M. Haupt, H. H. Goebel, D. Rating, K. Sartor, and F. Ebinger T2-hyperintense cerebellar cortex in Marinesco-Sjogren syndrome Neurology, December 28, 2004; 63(12): 2448 - 2449. [Full Text] [PDF]