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July 1982

Multiple Lipomas (Krabbe's Disease) With Hypothalamic Calcifications

Author Affiliations

Servicio de Neurocirugía Clínica Puerta de Hierro San Martín de Porres, 4 Madrid-35, Spain

Arch Neurol. 1982;39(7):456. doi:10.1001/archneur.1982.00510190074032

To the Editor.—  In 1944, Krabbe and Bartels described a disorder called "multiple circumscribed lipomatosis" characterized by the development of symmetrical lipomas in the arms, upper part of the chest, pelvis, and buttocks.1 This disease usually manifests itself around the age of 30 years and is not apparently hereditary or familial.2 Krabbe's disease has a slow, progressive, and painless course, and may be associated with anomalies of the skin, as pigmented nevi or fibromas, and also with diverse malformations and mental disturbances.3 These associations favor including Krabbe's lipomatosis within the large group of phakomatoses.4 Nevertheless, general somatic, including endocrine and neurologic examination, usually shows nothing remarkable.5We describe a patient with Krabbe's disease in whom radiologic studies, including a computed tomographic (CT) scan, showed multiple hypothalamic calcifications.

Report of a Case.—  A 27-year-old woman was seen after intracranial calcifications had been found in plain roentgenograms

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