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CT and MRI in maple syrup urine disease

From the Departmenta of Pediatric Neurology (Dm. Uziel and Nnrdocci) and Neuroradiology (Dr. Savoinrdo), Istituto Neurologico "C. Besta." Milan, Italy.

We describe a patient with a variant form of maple syrup urine disease who had unusual CT and MRI features that raised the suspicion of a metabolic disease. There were low density and abnormal signal in the white matter and pallida. Sponginess in these areas is the likely explanation for these findings.

Address correspondence and reprint requests to Dr. Uziel, Department of Pediatric Neurology, Istituto Neurologico "C. Besta," Via Celoria 11. 20133 Milan, Italy.

Received April 15, 1987. Accepted for publication in final form June 25, 1987.

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