title: Dura mater graft-associated Creutzfeldt-Jakob disease: the first case in Korea.
abstract: Since 1987, dura mater graft-associated iatrogenic Creutzfeldt-Jakob disease (dCJD) has been reported in many countries. We report the first case of dCJD in Korea. A 54-yr-old woman, who underwent resection of the meningioma in the left frontal region and received a dura mater graft 23 yr ago presented with dysesthesia followed by psychiatric symptoms and ataxia. Her neurological symptoms rapidly progressed to such an extent that she exhibited myoclonus, dementia, and pyramidal and extrapyramidal signs within 8 weeks. The 14-3-3 protein was detected in her cerebrospinal fluid; however, an electroencephalogram did not reveal characteristic positive sharp wave complexes. Diffusion-weighted magnetic resonance images, obtained serially over 64 days, revealed the rapid progression of areas of high signal intensity in the caudate nucleus and cingulate gyrus to widespread areas of high signal intensity in the cortex and basal ganglia. Pathological examination of brain biopsy specimens confirmed the presence of spongiform changes and deposition of prion protein in the neurons and neuropils.
authors: Department of Neurology, Hallym University College of Medicine, Hallym University, Anyang, Korea.
authors: Hye Lim
authors: HL
authors: Kim
authors: Ju Young
authors: JY
authors: Do
authors: Han Jeong
authors: HJ
authors: Cho
authors: Yong-Chul
authors: YC
authors: Jeon
authors: Seok Joo
authors: SJ
authors: Park
authors: Hyeo Il
authors: HI
authors: Ma
authors: Jun Ho
authors: JH
authors: Song
authors: Yul
authors: Y
authors: Lee
authors: Hyun
authors: H
authors: Choi
authors: Kyung Chan
authors: KC
authors: Choi
authors: Yong Sun
authors: YS
authors: Kim
authors: Inga
authors: I
authors: Zerr
authors: Kai
authors: K
authors: Kallenberg
authors: Yun Joong
authors: YJ
authors: Kim
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D001259:Ataxia / Q000175:diagnosis; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000473:pathology / Q000635:transmission*; D003704:Dementia / Q000175:diagnosis; D004388:Dura Mater / Q000637:transplantation*; D005260:Female; D006801:Humans; D008579:Meningioma / Q000601:surgery; D008875:Middle Aged; D010292:Paresthesia / Q000175:diagnosis; D011328:Prions / Q000032:analysis*; D056910:Republic of Korea; D019737:Transplants
chemical_list: D048948:14-3-3 Proteins; D011328:Prions