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Hallervorden spatz syndrome the contribution of ct and nmr imaging to diagnosis



Hallervorden spatz syndrome the contribution of ct and nmr imaging to diagnosis



Giornale di Neuropsichiatria dell'Eta Evolutiva 11(1): 35-40



Hallervorden-Spatz syndrome (HSS) is a rare condition characterized by onset during the first two decades of life with progressive spasticity, dystonia and dementia; pigmentary degeneration of retina may be an additional finding. Pathological changes mainly involve globus pallidus and substantial nigra: iron staining pigments, focal axonal swellings and demyelination are observed in subcortical nuclei. No diagnostic biochemical test is available and diagnosis is usually made post-mortem. We present a case, with early onset, analyzed by CT and NMR. Symmetrical areas of increased density (CT) and respectively decreased signal intensity on both proton density weighed and T2-weighted images (NMR) in the globus pallidus support the diagnosis of HSS, as recently demonstrated by other reports. We conclude that it is now possible to diagnose HSS while the patient is alive, by clinical and neuroradiological findings, as recently suggested by others Authors.

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