MR imaging of Hallervorden-Spatz disease.
Hallervorden-Spatz disease (HSD) is a movement disorder with neuropathology including abnormal iron storage, disordered myelination, and loss of brain substance. Cranial magnetic resonance (MR) imaging of two patients with HSD provided good quality imaging of these patients' atrophic brain stems and cerebella, regions partially degraded by beam-hardening artifact on X-ray CT. Magnetic resonance also demonstrated signal aberrations in the lentiform nuclei and surrounding white matter consistent with iron storage or disordered myelination. However, quantification of relaxation times of these lesions was not specific for either process. The diagnosis of HSD remains one of exclusion, with MR providing helpful but not definitive information.
D000293:Adolescent; D001480:Basal Ganglia Diseases / Q000175:diagnosis*; D001933:Brain Stem / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology*; D006801:Humans; D007501:Iron / Q000378:metabolism; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis* / Q000378:metabolism
D007501:Iron