Iron in the Hallervorden-Spatz syndrome.

摘要: The dark discoloration of globus pallidus and substantia nigra pars reticularis in the Hallervorden-Spatz syndrome is due to accumulation iron. Routine iron stains detect metal mostly microglia macrophages, but scattered neurons are also reactive. Axonal spheroids characteristic disease, many these expansions give a positive reaction. Globus normally rich iron, additional "storage" has often been considered essential factor pathogenesis syndrome. However, other equally iron-rich structures, such as red nucleus dentate nucleus, remain unaffected. In normal reticularis, double-label immunofluorescence microscopy ferritin, an indirect marker cellular localization, phosphorylated neurofilament protein reveal close proximity ferritin-reactive microglial oligodendroglial processes tightly packed axons. It proposed that primary axonal disorder allows seepage into axoplasm. Iron may contribute probably should be viewed epiphenomenon. Pallidal nigral excess not unique syndrome, some previously reported postmortem examinations actually represent pallidonigroluysian atrophy.