NR AWOR
AU Targlio,S.; Buffa,C.; Migheli,A.; Debandi,M.G.; Attuoni,M.; Dolce,D.; Atzori,C.; Imperiale,D.
TI Multifocal "necrotizing" leukoencephalopathy mimicking Creutzfeldt-Jakob disease
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions DIA-51
PT Konferenz-Poster
AB A multifocal "necrotizing" leukoencephalopathy (MNL) mainly involving the white matter of the pons and characterized by microscopic foci of necrosis have been reported in immunosuppressed and critically-ill patients. A 64-year-old woman with history of chronic respiratory failure of restrictive type was referred to our intensive care unit because of severe hyponatremia (110 mEq/l) and recurrent seizures. At admission, coma without focal neurologic signs was evident. EEG background was in theta range with frequent spike-wave discharges. In spite of adequate correction of sodium levels and systemic antiepileptic treatment, no clinical improvement was observed. CSF analysis disclosed the presence of 14-3-3 protein and very high levels of tau (10.000 pg/ml). A presumptive diagnosis of Creutzfeldt-Jakob disease (CJD) was made. A brain MRI highlighted high signals on DW and T2 scans involving left posterior thalamus and parietal cortex. A second CSF analysis (performed 4 weeks later) confirmed the presence of 14-3-3 protein and greatly raised tau levels (15.000 pg/ml). After about two months the patient died from multiple organ failure. Biochemical examination of frozen brain tissue failed to detect PrPres. Neuropathological examination ruled out a diagnosis of CJD. Multiple microscopic foci of necrosis involving the white matter tracts at the basis of the pons were present on histologic examination. No calcified areas were evident. A presumptive diagnosis of multifocal leukoencephalopathy was made. The case described appears to fulfil the pathologic criteria for MNL in immunosuppressed and critically-ill patients. To our knowledge there are no other reports of MNL mimicking CJD in its clinical, EEG, MRI and CSF aspects.
AD Centro Diagnosi Osservazione Malattie Prioniche D.O.M.P. - A.S.L. 3 Torino Address: Ospedale Maria Vittoria Via Cibrario 72 - 10144 Torino Phone 0114393845; fax 0114393503. E-mail: imperiale@asl3.to.it
SP englisch
PO Italien