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(Radiology. 1999;213:400-406.)
© RSNA, 1999

Neuroradiology

Cree Leukoencephalopathy: Neuroimaging Findings1

Ibrahim A. Alorainy, MD, Yves G. Patenaude, MD, Augustin M. O'Gorman, MD, Deborah N. Black, MD and Kathleen Meagher-Villemure, MD

1 From the Department of Medical Imaging, Montreal Children's Hospital, Quebec, Canada (I.A.A., Y.G.P., A.M.O.); the Department of Neurology, Central Vermont Hospital, Montpelier (D.N.B.); and the Institut Universitaire de Pathologie, Lausanne, Switzerland (K.M.V.). Received November 9, 1998; revision requested December 29; revision received February 2, 1999; accepted April 29. Address reprint requests to I.A.A., Department of Radiology, College of Medicine, King Saud University, P.O. Box 9047, Riyadh 11413, Saudi Arabia.

PURPOSE: To describe the computed tomographic (CT) and magnetic resonance (MR) imaging findings in Cree leukoencephalopathy.

MATERIALS AND METHODS: The authors retrospectively reviewed the medical records and neuroimaging studies in 12 infants with Cree leukoencephalopathy (CT in 12 infants, MR in six). The diagnosis was established clinically in six patients and at autopsy in the other six.

RESULTS: At CT, extensive, diffuse, and symmetric hypoattenuation was seen in the cerebral and cerebellar white matter in all 12 patients. Hypoattenuation was also seen in the corpus callosum in 11 (92%), internal capsule in 10 (83%), globus pallidus in nine (75%), brainstem in nine (75%), and thalamus in four (33%). The caudate nucleus and putamen were spared. On T2-weighted MR images in six patients, the cerebral and cerebellar white matter, including the subcortical arcuate fibers, was hyperintense as were the internal capsule, corpus callosum, corticospinal tracts, and globus pallidus. The thalamus was affected in four (67%) patients, pons in five (83%), and medulla in four (33%). The caudate nucleus and putamen were not affected.

CONCLUSION: Cree leukoencephalopathy causes striking symmetric and diffuse involvement of the cerebral and cerebellar white matter and brainstem with sparing of the caudate nucleus and putamen.

Index terms: Brain, CT, 10.1211 • Brain, diseases, 10.872 • Brain, MR, 10.12141 • Infants, central nervous system, 10.872 • Infants, newborn, central nervous system, 10.872




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