Prader-Willi syndrome and central nervous system calcifications: chance or fundamentally related findings?
The central nervous system of a 16 8/12-year-old boy with Prader-Willi syndrome is described. Extensive calcifications of the central nervous system were observed at autopsy as grittiness of the brain slices and verified by X-ray and microscopy. The encrustations were particularly localized in the leptomeninges, the first and second layer of the cerebral and cerebellar cortex and along the ventricular system. We suggest that the alterations are nonspecific and perhaps secondary to a fundamental metabolic disorder in the central nervous system.
Reske-Nielsen
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Department of Neuropathology and Radiology R, University Hospital, Aarhus, Denmark.
Lund
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D000293:Adolescent; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000473:pathology*; D002114:Calcinosis / Q000473:pathology*; D006801:Humans; D008297:Male; D009046:Motor Neurons / Q000473:pathology; D009474:Neurons / Q000473:pathology; D011218:Prader-Willi Syndrome / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology; D013118:Spinal Cord Diseases / Q000473:pathology*