Sporadic Creutzfeldt-Jakob disease(sCJD)is a prion-caused degenerative disease of the central nervous system,with the typical clinical manifestation of rapidly progressive dementia.The course of disease is less than 1 year in most patients and more than 2 years in only 2% to 3% patients.We reported a case of sCJD with expressive language disorder and slow progression in this paper.By summarizing the clinical manifestations and the electroencephalograhpy,MRI,and pathological features,we aimed to enrich the knowledge about the sCJD with slow progression.
Humans ; Creutzfeldt-Jakob Syndrome/pathology* ; Brain/pathology* ; Magnetic Resonance Imaging ; Central Nervous System/pathology*

Creutzfeldt-Jakob disease (CJD) is presumably caused by a slow infectious pathogen or prion. The principal clinical features of Creutzfeldt-Jakob disease are dementia, pyramidal and extrapyramidal symptoms and signs, cerebellar dysfunction, and myoclonus. The patient rapidly deteriorates, declines to a vegetative state, becomes comatous, and is ultimately dead within several months. The authors present a case of Creutzfeldt-Jakob disease, proved by clinical findings, typical serial EEG, and pathologic features.
Creutzfeldt-Jakob Syndrome/*diagnosis/pathology/radiography ; Electroencephalography ; Female ; Humans ; Middle Aged ; Tomography, X-Ray Computed

BACKGROUNDCreutzfeldt-Jakob disease (CJD), a rare disease, is uncharacterized by computed tomography (CT) and magnetic resonance imaging (MRI). This study was aimed to evaluate the diffusion-weighted MRI (DWI) manifestations of CJD and to discuss their diagnostic value.

METHODSThe findings of T(1)-weighted MRI (T(1)WI), T(2)-weighted MRI (T(2)WI), DWI and post-contrast MRI in 5 patients (3 patients with biopsy-proven CJD and 2 patients with clinically-proven CJD) were retrospectively analyzed in this study.

RESULTSFour out of the 5 patients had cerebral atrophy of various degrees. One patient showed symmetric high signal intensity at the bilateral globus pallidus and the head of the caudate nucleus, with very high signal in the cerebral cortex on the DWI. This patient only had symmetric slightly high signal at the bilateral globus pallidus and putamen on T(2)WI. One patient had high signal intensity at the basal ganglia and cerebral cortex on DWI, but abnormal T(2) signal intensity at the bilateral paraventricular white matter on MRI. Two patients presented with widely gyri-like high signal intensity at the cortex on DWI, but routine MRI showed bilateral paraventricular long T(2) signal intensity in 1 patient and no abnormal findings in another. No abnormalities were shown by both routine MRI and DWI in the last patient.

CONCLUSIONSDWI is more sensitive than its conventional counterpart in the depiction of CJD. DWI is more sensitive to detect cortical abnormal signal intensity in CJD not detected by T(2)WI.

Adult ; Aged ; Atrophy ; Brain ; pathology ; Creutzfeldt-Jakob Syndrome ; diagnosis ; pathology ; physiopathology ; Diffusion Magnetic Resonance Imaging ; methods ; Electroencephalography ; Female ; Humans ; Male ; Middle Aged

OBJECTIVE: To compare conventional and diffusion-weighted MR imaging in terms of their depiction of the abnormalities occurring in Creutzfeldt-Jakob disease. MATERIALS AND METHODS: We retrospectively analyzed the findings of conventional (T2-weighted and fluid-attenuated inversion recovery) and diffusion-weighted MR imaging in four patients with biopsy-proven Creutzfeldt-Jakob disease. The signal intensity of the lesion was classified by visual assessment as markedly high, slightly high, or isointense, relative to normal brain parenchyma. RESULTS: Both conventional and diffusion-weighted MR images demonstrated bilateral high signal intensity in the basal ganglia in all four patients. Cortical lesions were observed on diffusion-weighted MR images in all four, and on fluid-attenuated inversion recovery MR images in one, but in no patient on T2-weighted images. Conventional MR images showed slightly high signal intensity in all lesions, while diffusion-weighted images showed markedly high signal intensity in most. CONCLUSION: Diffusion-weighted MR imaging is more sensitive than its conventional counterpart in the depiction of Creutzfeldt-Jakob disease, and permits better detection of the lesion in both the cerebral cortices and basal ganglia.
Adult ; Aged ; Biopsy ; Comparative Study ; Creutzfeldt-Jakob Syndrome/diagnosis/*pathology ; Female ; Human ; Magnetic Resonance Imaging/*methods ; Male ; Middle Age ; Retrospective Studies ; Sensitivity and Specificity ; Support, Non-U.S. Gov't

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.
14-3-3 Proteins/cerebrospinal fluid ; Ataxia/diagnosis ; Brain/pathology ; Creutzfeldt-Jakob Syndrome/diagnosis/pathology/*transmission ; Dementia/diagnosis ; Dura Mater/*transplantation ; Female ; Humans ; Meningioma/surgery ; Middle Aged ; Paresthesia/diagnosis ; Prions/*analysis ; Republic of Korea ; Transplants

A 68-year-old man presented with a three-week history of rapidly progressive dementia, gait ataxia and myoclonus. Subsequent electroencephalography showed periodic sharp wave complexes, and cerebrospinal fluid assay revealed the presence of a 14-3-3 protein. A probable diagnosis of sporadic Creutzfeldt-Jakob disease was made, which was further supported by magnetic resonance (MR) imaging of the brain showing asymmetric signal abnormality in the cerebral cortices and basal ganglia. The aetiology, clinical features, diagnostic criteria, various MR imaging patterns and radiologic differential diagnosis of sporadic Creutzfeldt-Jakob disease are discussed in this article.
Aged ; Brain ; pathology ; Cerebral Cortex ; Cerebrospinal Fluid ; metabolism ; Creutzfeldt-Jakob Syndrome ; diagnostic imaging ; Dementia ; physiopathology ; Diagnosis, Differential ; Diffusion Magnetic Resonance Imaging ; Electroencephalography ; Humans ; Hypoxia-Ischemia, Brain ; diagnostic imaging ; Male ; Prion Diseases ; physiopathology