Background: Superficial siderosis (SS) of the central nervous system is a rare and heterogeneous condition due to deposition of hemosiderin on the surface of the brain and spinal cord. The usually progressive clinical course is characterized by a combination of hearing loss, cerebellar ataxia, and myelopathy. There is no known treatment for SS, but the iron chelator deferiprone (DFP) has been proposed as a potentially useful treatment.
View Article and Find Full Text PDFObjective: To evaluate the long-term effect of Deferiprone (DFP) in reducing brain iron overload and improving neurological manifestations in patients with NBIA.
Methods: 6 NBIA patients (5 with genetically confirmed PKAN), received DFP solution at 15 mg/kg po bid. They were assessed by UPDRS/III and UDRS scales and blinded video rating, performed at baseline and every six months.
Deferiprone was shown to reverse iron deposition in Friedreich's ataxia. This multi-center, unblinded, single-arm pilot study evaluated safety and efficacy of deferiprone for reducing cerebral iron accumulation in neurodegeneration with brain iron accumulation. Four patients with genetically-confirmed pantothenate kinase-associated neurodegeneration, and 2 with parkinsonism and focal dystonia, but inconclusive genetic tests, received 15 mg/kg deferiprone bid.
View Article and Find Full Text PDFAfter nine months' experience with helical CT (Elscint Twin), we describe an epicrisis in 13 patients with intracranial aneurysms, 12 of whom were referred to us by the emergency room for suspect subarachnoid hemorrhage. After standard CT scan confirming subarachnoid hemorrhage, patients underwent angio-CT with display of the sac responsible for bleeding in all cases. All patients subsequently underwent digital angiography which confirmed the angio-CT findings and in three cases disclosed associated malformations.
View Article and Find Full Text PDFAn application of intravenous radionuclide angiography as an adjunct to SPECT with 99mTc HM-PAO is presented. From the angiographic sequence a two-dimensional parametric image is generated computing, pixel by pixel, the center of gravity (COG) of the local time curves. This index is independent of blood flow and reflects the relation between transmitted and extravasated activity.
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