Atypical form of dural graft associated Creutzfeldt-Jakob disease: report of a postmortem case with review of the literature

Abstract

(J Neurol Neurosurg Psychiatry 2001;70:696-699)

Autopsy case of Creutzfeldt–Jakob disease with Met/Val heterozygosity at codon 129 and type 1 protease‐resistant prion protein presenting some florid‐type plaques and many Kuru plaques in the cerebellum

“…The presence of florid‐type plaques and Kuru plaques was already pointed out in an atypical subtype of dura‐associated CJD cases 5–10 . The findings of our case were similar to this atypical subtype, which was reported to have type 1 PrPres 5,6 . Our case also showed type 1 PrPres on western immunoblots.…”

MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease

“…Dura mater grafts have been found as disease cause in 200 CJD patients worldwide [1]. Due to the long incubation periods ranging from 16 months to 31 years, DM-CJD cases are still detected nowadays [1,[3][4][5][6][7][8][9].…”

Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease

“…13 In addition, patient 3 showed myokymia, hyporeflexia in the lower extremities, and decreased nerve conduction velocities without conduction block, indicating the presence of peripheral neuropathy. Of the two patients with dCJD, 11 12 one (patient 5) had atypical clinicopathological features showing PrP plaque formation in the brain, 11 but had no evidence of peripheral neuropathy. Patient 6 presented classic clinicopathological manifestations of GSS102, including progressive ataxia and dementia, and also showed areflexia in the lower extremities and painful dysaesthesia.…”