Authors: Yasushi Iwasaki Mari Yoshida Yoshio Hashizume Tetsuyuki Kitamoto Gen Sobue
Publish Date: 2006/07/18
Volume: 112, Issue: 5, Pages: 561-571
Abstract
We analyzed neuropathologic features of 23 Japanese patients with sporadic Creutzfeldt–Jakob disease sCJD by means of prion protein PrP immunolabeling associated with codon 129 polymorphism of the PrP gene and western blot analysis of proteaseresistant PrP PrP type Clinical features particularly age at onset disease duration periodic synchronous discharge and presence of myoclonus were also analyzed This study included 11 cases of subacute spongiform encephalopathy SSE 10 cases of panencephalopathic PEtype sCJD and two cases of thalamictype sCJD classified according to cerebral pathology findings According to PrP gene polymorphism and PrP type 18 cases were classified as MM1type two as MV1type two as MM2type and one as MM1 + 2type sCJD SSE and PEtype sCJD showed similar clinical features with the exception of disease duration codon 129 polymorphism and PrP type Thalamictype sCJD showed different clinical features and PrP type We suggest that SSE and PEtype sCJD comprise the sCJD subtype and that PEtype sCJD is a prolonged pathologic phenotype of SSE When we compare our results with those from a series of Caucasian sCJD patients the percentages of codon 129 polymorphisms differed as did classification based on PrP gene polymorphism and PrP type our series included many PEtype sCJD cases and disease duration was relatively long and MM2type cases showed clinicopathologic variability
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