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From: TSS ()
Subject: MM2-thalamic-type sporadic Creutzfeldt-Jakob disease with widespread neocortical pathology
Date: October 21, 2006 at 1:30 pm PST

##################### Bovine Spongiform Encephalopathy #####################

MM2-thalamic-type sporadic Creutzfeldt-Jakob disease with widespread neocortical pathology


Journal Acta Neuropathologica
Publisher Springer Berlin / Heidelberg
ISSN 0001-6322 (Print) 1432-0533 (Online)
Subject Medicine
Issue Volume 112, Number 4 / October, 2006
Category Case Report
DOI 10.1007/s00401-006-0131-3
Pages 503-511
Online Date Thursday, September 07, 2006

Case Report
MM2-thalamic-type sporadic Creutzfeldt-Jakob disease with widespread neocortical pathology
Kazunori Hirose1, Yasushi Iwasaki2 , Masayuki Izumi1, Mari Yoshida3, Yoshio Hashizume3, Tetsuyuki Kitamoto4 and Ko Sahashi1

(1) Department of Neurology, Aichi Medical University School of Medicine, 21 Karimata Yazako Nagakute-cho, Aichi-gun, Aichi 480-1195, Japan
(2) Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan
(3) Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Aichi, Japan
(4) Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, 2-1 Seiryo-cho, Aoba-ku, Sendai 980-8575, Japan

Received: 7 June 2006 Revised: 31 July 2006 Accepted: 5 August 2006 Published online: 7 September 2006

Abstract We report an autopsy case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) with widespread cerebral neocortical pathology. Initial symptoms were progressive insomnia and mental disturbance. Magnetic resonance imaging revealed no high-signal intensity lesions on diffusion-weighted images and later showed gradually progressive cerebral atrophy. Periodic synchronous discharges and myoclonus were not observed. Upon neuropathologic examination, widespread cerebral neocortical involvement with fine vacuole-type spongiform change was observed. Severe degeneration with almost complete neuronal loss, tissue rarefaction, numerous fat-laden macrophages and hypertrophic astrocytosis of the medial thalamic nucleus was evident. The inferior olivary nucleus showed severe involvement with neuronal loss and hypertrophic astrocytosis. In the cerebellar cortex, moderate depletion of Purkinje neurons was evident, with no spongiform change in the molecular layer and no neuronal loss in the granule cell layer. Immunohistochemistry for prion protein (PrP) revealed widespread synaptic-type deposits with some primitive plaque-type deposits in the cerebral neocortex, basal ganglia and cerebellar cortex. PrP deposition was also observed in the brainstem, particularly the tegmentum, substantia nigra and pontine nucleus, and spinal cord, particularly the posterior horn. In the medial thalamus and inferior olivary nucleus, PrP deposition was sparse. Analysis of the PrP gene showed no mutation but did show methionine homozygosity at polymorphic codon 129. Western blot analysis of protease-resistant PrP indicated the presence of type 2 PrP. We believe that this patient suffered from MM2-thalamic-type sCJD (sporadic fatal insomnia) with widespread cerebral neocortical pathology due to prolonged disease duration. The present case showed different patterns of spongiform degeneration and PrP deposition in the cerebral neocortex than those in previously reported MM2-thalamic-type sCJD cases.


http://www.springerlink.com/content/ax541436156751v2/


TSS

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