Structural and functional neuroimaging in human prion diseases

被引:9
作者
Ortega-Cubero, S. [1 ]
Luquin, M. R. [1 ]
Dominguez, I. [2 ]
Arbizu, J. [2 ]
Pagola, I. [1 ]
Carmona-Abellan, M. M. [1 ]
Riverol, M. [1 ]
机构
[1] Univ Navarra Clin, Dept Neurol, Navarra, Spain
[2] Univ Navarra Clin, Nucl Med Serv, Navarra, Spain
来源
NEUROLOGIA | 2013年 / 28卷 / 05期
关键词
Creutzfeldt-Jakob; PET; Prion; Prion diseases; MRI; SPECT; CREUTZFELDT-JAKOB-DISEASE; FATAL FAMILIAL INSOMNIA; MAGNETIC-RESONANCE SPECTROSCOPY; EMISSION COMPUTED-TOMOGRAPHY; STRAUSSLER-SCHEINKER-DISEASE; CLINICAL-DIAGNOSIS; STATUS EPILEPTICUS; IMAGING FINDINGS; PULVINAR SIGN; PROTEIN PRPC;
D O I
10.1016/j.nrl.2011.03.012
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Introduction: Prion diseases are neurodegenerative disorders resulting from the accumulation of a misfolded isoform of the cellular prion protein (PrPc). They can occur as acquired, sporadic, or hereditary forms. Although prion diseases show a wide range of phenotypic variations, pathological features and clinical evolution, they are all characterised by a common unfavourable course and a fatal outcome. Review summary: Some variants, such as kuru, have practically disappeared, while others, for example the variant Creutzfeldt-Jakob (vCJD) or those attributable to iatrogenic causes, are still in force and pose a challenge to current medicine. There are no definitive pre-mortem diagnostic tests, except for vCJD, where a tonsil biopsy detects 100% of the cases. For this reason, diagnostic criteria dependent on statistical probability have had to be created. These require complementary examinations, such as an electroencephalogram (EEG) or the detection of 14-3-3 protein in cerebrospinal fluid (CSF). Only the pulvinar sign in magnetic resonance imaging (MRI) has been included as a vCJD diagnostic criterion. The present review discusses neuroimaging findings for each type of prion disease in patients with a definitive histopathological diagnosis. Conclusions: The aim is to define the usefulness of these complementary examinations as a tool for the diagnosis of this family of neurodegenerative diseases. (C) 2011 Sociedad Espanola de Neurologia. Published by Elsevier Espana, S.L. All rights reserved.
引用
收藏
页码:299 / 308
页数:10
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