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当代阿耳茨海默病研究

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ISSN (Print): 1567-2050
ISSN (Online): 1875-5828

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Case Report

原发性干燥综合征伴快速进行性痴呆：一例

卷 19, 期 6, 2022

发表于: 18 July, 2022

页: [479 - 484] 页: 6

弟呕挨: 10.2174/1567205019666220627094707

价格: $65

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摘要

背景：快速进展性痴呆症（RPD）是指在数周至数月的时间内进展缓慢的痴呆症。原发性干燥综合征（pSS）是一种自身免疫性疾病，可影响任何器官系统，并可能表现为广泛的临床特征，可能模仿过多的医疗条件，在罕见情况下，可能表现为RPD。我们描述了一个独特的pSS病例，其中快速进行性痴呆（RPD）是第一个疾病表现，患者的放射学和脑电图结果与Creutzfeldt-Jakob病（CJD）相符。 病例介绍：在这里，我们报告了一名58岁的女性，她在入院前的6个月内出现了认知障碍迅速恶化。脑MRI和EEG提示CJD。然而，CSF 14-3-3和tau/phospho-tau比值在正常范围内，因此考虑了替代诊断。血液测试对阳性的抗核抗体、抗ENA和抗SSA具有重要意义，嘴唇活检与pSS一致。患者开始静脉注射类固醇，然后口服泼尼松，这防止了病情进一步恶化。 结论：这一罕见病例扩大了pSS的神经表现范围，强调了在RPD的鉴别诊断中考虑pSS的重要性，以避免误诊并及时提供适当的治疗。

关键词: 干燥综合征、克雅氏病、神经认知障碍、痴呆症、病例报告、神经系统自身免疫性疾病、脑电图源成像。

« Previous
[1]
Jin JO, Yu QT. Cell-associated cytokines in the pathogenesis of Sjögren's syndrome. J Clin Cell Immunol 2013; S(9): 11742.
[2]
Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009; 132(Pt 10): 2659-68.
[3]
Shiboski CH, Shiboski SC, Seror R, et al. American college of Rheumatology/European league against rheumatism classification criteria for primary Sjögren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol 2017; 69(1): 35-45.
[4]
Geschwind MD. Rapidly progressive dementia. Continuum (Minneapolis, Minn) 2016; 22(2 Dementia): 510-37.
[5]
Zerr I, Pocchiari M, Collins S, et al. Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease. Neurology 2000; 55(6): 811-5.
[6]
Wieser HG, Schindler K, Zumsteg D. EEG in Creutzfeldt-Jakob disease. Clin Neurophysiol 2006; 117(5): 935-51.
[7]
Muayqil T, Gronseth G, Camicioli R. Evidence-based guideline: Diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: Report of the guideline development subcommittee of the American Academy of Neurology. Neurology 2012; 79(14): 1499-506.
[8]
Young GS, Geschwind MD, Fischbein NJ, et al. Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: High sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol 2005; 26(6): 1551-62.
[9]
Meissner B, Kallenberg K, Sanchez-Juan P, et al. MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology 2009; 72(23): 1994-2001.
[10]
Atarashi R, Satoh K, Sano K, et al. Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion. Nat Med 2011; 17(2): 175-8.
[11]
Margaretten M. Neurologic Manifestations of Primary Sjögren Syndrome. Rheum Dis Clin North Am 2017; 43(4): 519-29.
[12]
Delalande S, de Seze J, Fauchais AL, et al. Neurologic manifestations in primary Sjogren syndrome: A study of 82 patients. Medicine (Baltimore) 2004; 83(5): 280-91.
[13]
Carvajal Alegria G, Guellec D, Mariette X, et al. Epidemiology of neurological manifestations in Sjogren’s syndrome: Data from the French ASSESS Cohort. RMD Open 2016; 2(1): e000179.
[14]
Massara A, Bonazza S, Castellino G, et al. Central nervous system involvement in Sjögren’s syndrome: Unusual, but not unremarkable--clinical, serological characteristics and outcomes in a large cohort of Italian patients. Rheumatology (Oxford) 2010; 49(8): 1540-9.
[15]
Morreale M, Marchione P, Giacomini P, et al. Neurological involvement in primary Sjögren syndrome: A focus on central nervous system. PLoS One 2014; 9(1): e84605.
[16]
Moreira I, Teixeira F, Martins Silva A, Vasconcelos C, Farinha F, Santos E. Frequent involvement of central nervous system in primary Sjögren syndrome. Rheumatol Int 2015; 35(2): 289-94.
[17]
Matsuo K, Saburi M, Ishikawa H, et al. Sjögren syndrome presenting with encephalopathy mimicking Creutzfeldt-Jakob disease. J Neurol Sci 2013; 326(1-2): 100-3.
[18]
Bourahoui A, De Seze J, Guttierez R, et al. CSF isoelectrofocusing in a large cohort of MS and other neurological diseases. Eur J Neurol 2004; 11(8): 525-9.
[19]
Pars K, Pul R, Schwenkenbecher P, et al. Cerebrospinal fluid findings in neurological diseases associated with Sjögren’s syndrome. Eur Neurol 2017; 77(1-2): 91-102.
[20]
Alexander EL, Lijewski JE, Jerdan MS, Alexander GE. Evidence of an immunopathogenic basis for central nervous system disease in primary Sjogren’s syndrome. Arthritis Rheum 1986; 29(10): 1223-31.
[21]
Alexander EL, Ranzenbach MR, Kumar AJ, et al. Anti-Ro(SS-A) autoantibodies in central nervous system disease associated with Sjogren’s syndrome (CNS-SS): Clinical, neuroimaging, and angiographic correlates. Neurology 1994; 44(5): 899-908.
[22]
Del Papa N, Vitali C. Management of primary Sjogren’s syndrome: Recent developments and new classification criteria. Ther Adv Musculoskelet Dis 2018; 10(2): 39-54.
[23]
Pascual-Marqui RD, Michel CM, Lehmann D. Low resolution electromagnetic tomography: A new method for localizing electrical activity in the brain. Int J Psychophysiol 1994; 18(1): 49-65.
[24]
Brunet D, Murray MM, Michel CM. Spatiotemporal analysis of multichannel EEG: CARTOOL. Comput Intell Neurosci 2011; 2011: 813870.
[25]
Nemtsas P, Birot G, Pittau F, et al. Source localization of ictal epileptic activity based on high-density scalp EEG data. Epilepsia 2017; 58(6): 1027-36.

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