Journal
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
Volume 70, Issue 5, Pages 696-699Publisher
BRITISH MED JOURNAL PUBL GROUP
DOI: 10.1136/jnnp.70.5.696
Keywords
cadaveric dura mater graft; Creutzfeldt-Jakob disease; prion protein; plaque
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A postmortem case of an atypical form of dural graft associated Creutzfeldt-Jakob disease (CJD) is described. A 42 gear old man developed progressive spastic paresis 163 months after a cadaveric dura mater graft. He presented with no myroclonus and very late occurrence of periodic synchronous discharges on EEG. The prion protein (PrP) gene was homozygous for methionine at the polymorphic codon 129. Neuropathological examination disclosed plaque-like PrP deposits with atypical distribution elf synaptic PrP accumulations in the brain. This patient represents an atypical form of dural graft associated CJD characterised by unusual clinicopathological features.
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