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AJNR - American Journal of Neuroradiology

Published ahead of print on March 11, 2009
doi: 10.3174/ajnr.A1508

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American Journal of Neuroradiology 30:1325-1327, August 2009
© 2009 American Society of Neuroradiology

Case Report
PEDIATRICS

MR Imaging Findings in 2 Cases of Late Infantile GM1 Gangliosidosis

E. De Grandis^a, M. Di Rocco^b, A. Pessagno^a, E. Veneselli^a and A. Rossi^c

^a Division of Child Neuropsychiatry, Gaslini Institute, University of Genoa, Genoa, Italy
^b Second Unit of Pediatrics, Gaslini Institute, Genoa, Italy
^c Department of Pediatric Neuroradiology, Gaslini Institute, Genoa, Italy

Please address correspondence to Elisa De Grandis, MD, Division of Child Neuropsychiatry, G Gaslini Institute, University of Genoa, Largo Gaslini 5, 16148 Genova Quarto, Italy; e-mail: elisadegrandis{at}yahoo.it

SUMMARY: Late infantile GM1 gangliosidosis is a rare lysosomal disorder characterized by mental deterioration and progressive spastic, cerebellar, and extrapyramidal signs, without facial dysmorphisms and organomegaly. Neuroimaging findings have been reported in only a few cases. Here we report on predominant globus pallidus MR signal-intensity abnormalities in 2 patients with the late infantile form of GM1 gangliosidosis.