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.

Keywords: Creutzfeldt-Jakob Syndrome; Dura Mater Graft; Iatrogenic Disease.

Fig. 1

Serial DWIs taken during the course of dCJD. The first DWI (left column), taken almost 6 weeks after her first neurological symptoms, revealedslightly increased signal intensity in the cingulate gyrus and the caudate nucleus. In the second DWI (middle column), taken 10 weeks after her first neurological symptoms, high signal intensity in both insulae, the caudate nucleus, and the medial frontal cortical region became obvious. In the third DWI (right column), taken 12 weeks after her first neurological symptoms, extensive high signal intensity was observed in the both basal ganglia and the cortex.

Fig. 2

Microscopic findings. (A) Neuronal loss and spongiform change (hematoxylin and eosin stain). (B) Astrocytosis detected using an anti-GFAP antibody. (C) Deposition of prion protein monoclonal antibody (1C5) for prion protein (arrow).