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Dura mater graft-associated Creutzfeldt-Jakob disease: the first case in Korea

Hye Lim Kim1, Ju Young Do, Han Jeong Cho

  • 1Department of Neurology, Hallym University College of Medicine, Hallym University, Anyang, Korea.

Journal of Korean Medical Science
|November 9, 2011
PubMed

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.

Keywords:
Creutzfeldt-Jakob SyndromeDura Mater GraftIatrogenic Disease

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