NR AXZD
AU Yamada,M.; Noguchi-Shinohara,M.; Hamaguchi,T.; Kitamoto,T.; Sato,T.; Nakamura,Y.; Mizusawa,H.; CJD Surveillance Committee, Japan
TI Plaque and Non-plaque Types of Dura Mater Graft-associated Creutzfeldt-Jakob Disease: Clinicopathological and Molecular Analysis
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Epidemiology, Risk Assessment and Transmission P04.11
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
Background: A total of 129 patients with dura mater graft-associated Creutzfeldt-
Jakob disease (dCJD) have been identified in Japan, accounting for the greater part of the world dCJD cases. A subset of patients with dCJD demonstrates atypical clinical features and plaque formation in the brain (plaque type).
Objective: To elucidate the frequency and features of plaque type dCJD in comparison with the non-plaque type.
Methods: We analyzed clinicopathological findings and molecular features of prion protein (PrP) of 66 patients who had been prospectively registered as having dCJD by the CJD Surveillance Committee, Japan.
Results: (1) Analysis of pathologically confirmed dCJD patients (n=23) demonstrated plaque type dCJD in 11 patients (48%). In contrast to the non-plaque type with classical CJD features, the plaque type commonly presented with ataxic gait as an initial manifestation, relatively slow progression, and no or late occurrence of periodic sharp-wave complexes (PSWCs) on EEG. MRI, especially diffusion-weighted images, and CSF 14-3-3 protein and neuron specific enolase showed high diagnostic sensitivities for plaque as well as non-plaque types. The gender, age, or sites of dura mater grafting did not differ between the plaque and non-plaque types. In both the plaque and non-plaque types, PrP codons 129 and 219 were Met/Met and Glu/Glu, respectively, except for a non-plaque type patient with a Glu/Lys at codon 219. Both the plaque and non-plaque types presented with type 1 PrPres on Western blot. (2) Analysis of clinically diagnosed dCJD patients (n=34) demonstrated that 7 patients (21%) had atypical clinical features without PSWCs, probably corresponding to plaque type dCJD.
Conclusion: The frequency of the plaque type is apparently higher than previously recognized. About one-third of total dCJD patients may be the plaque type. For the clinical diagnosis of the plaque type, MRI and CSF markers would be useful, in addition to the core features, i.e., onset with ataxic gait disturbance, relatively slow progression, and no or late occurrence of PSWCs on EEG. The plaque and non-plaque types did not differ for the recipient-related factors such as PrP genotype; for the dura mater graft-related factors, there was no information on the donors of the dura mater product (Lyodura) available at all.
AD M. Yamada, M. Noguchi-Shinohara, T. Hamaguchi, Kanazawa University Graduate School of Medical Science, Japan; T. Kitamoto, Tohoku University Graduate School of Medicine, Japan; T. Sato, Higashi-Yamato Hospital, Japan; Y. Nakamura, Jichi Medical University, Japan; H. Mizusawa, Graduate School, Tokyo Medical and Dental University, Japan; CJD Surveillance Committee, Japan
SP englisch
PO Schottland