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From The Russell H. Morgan Department of Radiology and Radiological Science (Drs. Nagae-Poetscher, Horská, and Barker), Johns Hopkins University School of Medicine, Baltimore, MD; Kennedy Krieger Institute (Drs. Nagae-Poetscher, Bibat, Fatemi, and Naidu), Baltimore, MD; UCLA, MRRC, and BRI (Dr. Philippart), Los Angeles, CA; and Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (Drs. Rosemberg, Lacerda, Costa, Kok, and Costa Leite), São Paulo, Brazil.
Address correspondence and reprint requests to Dr. S. Naidu, 707 North Broadway, Baltimore, MD 21205; e-mail: Naidu{at}kennedykrieger.org
We describe three cases of the rare syndrome of leukoencephalopathy, brain calcifications, and cysts. Conventional MRI, proton spectroscopy, and diffusion-weighted imaging yielded additional information on the disease. Imaging findings favor increased water content rather than a demyelinating process in the pathophysiology of this disease. Clinical features of Coats disease and consanguinity were also encountered.
Received June 26, 2003. Accepted in final form November 10, 2003.
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