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Five Cases of Patients with Creutzfeldt-Jakob Disease and Literature Review

Author: LvYuan
Tutor: LiMingXin
School: Shandong University
Course: Clinical
Keywords: Creutzfeldt-Jakob disease (CJD) cognitive disorder diagnostic criteria electroencephalogram (EEG) magnetic resonance imaging (MRI) cerebrospinalfluid (CSF) neuron specific enolase (NSE) 14-3-3protein
CLC: R742
Type: Master's thesis
Year: 2013
Downloads: 32
Quote: 0
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Abstract


Objectives:The aim of this study is to investigate the clinical manifestation, laboratory examination, electroencephalogram characteristics, neuropathological lesion and imaging findings of Creutzfeldt-Jakob disease (CJD).Materials and Methods:Analyze the5cases with Creutzfeldt-Jakob disease (CJD) that has been diagnosed in the department of neurology, Qilu hospital. The clinical features of Creutzfeldt-Jakob disease were analyzed, including the etiology, pathogenesis, clinical manifestation, electroencephalogram characteristics, cranial MRI, diagnostic criteria and prognosis.Result:Five patients had no obvious predisposing causes before the occurring of symptoms. The symptom that appeared above all often is a rapidly progressive cognitive disorder. These five patients had different degree of cognitive disorder. Four patients suffered from aphasia, apraxia, agnosia, agraphia, alexia, acalculia, hypomnesis, unorientation and disorders of judgement while the other one patient only had agraphia and acalculia. Their clinical manifestations were various, including conscious disturbance, myoclonus, visual symptoms, pyramidal dysfunction, extrapyramidal dysfunction, akinetic mutism, cerebellar disturbance and autonomic dysfunction. Value of neuron specific enolase (NSE) of four patients all increased while the other one didn’t have this test. Diffusion-weighted MRI of these patients showed a hyperintensity in cerebral cortex (frontal lobe, parietal lobe, temporal lobe, and occipital lobe) and basal ganglia. Their EEG showed a periodic sharp wave without a wave. Other ordinary investigations of the five patients didn’t have abnormal results. After being diagnosed with CJD, all of them discharged from hospital. Then we followed these patients up later, two of them lost to follow up and the other three died soon. Median survival time is5months.Conclusion:Patients with CJD always developed in subacute onset without obvious predisposing causes. The symptom that appeared above all often is a rapidly progressive cognitive disorder. Clinical manifestations of CJD were various and related to lesion locations. The definitive diagnosis of CJD needs a neuropathology test. However, clinical diagnosis depends on medical history, typical clinical features, special laboratory examination, electroencephalogram characteristics and MRI findings. There is still no effective therapeutic method for CJD. This kind of disease is always fatal and median survival time is5months.

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