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暴发性多发性硬化症(MS)。

Fulminant multiple sclerosis (MS).

机构信息

Rasoul-e-Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

Neurol Sci. 2011 Oct;32(5):953-7. doi: 10.1007/s10072-011-0723-0. Epub 2011 Aug 24.

DOI:10.1007/s10072-011-0723-0
PMID:21863271
Abstract

Fulminant multiple sclerosis (MS) is the most malignant form of MS which usually leads to death in few weeks. Although it can be accompanied by optic neuritis (ON), but long interval between ON and the grave onset has not been reported. Fulminant MS usually occurs as the first onset and previous ON is not common. We report a rare case of 30-year-old woman with a history of ON 1 year ago. The rapid deterioration to vegetative state followed by a seizure and previous ON differentiated this case from previous reported cases. The differential diagnosis of a rapidly progressive leucoencephalopathy in this patient includes inflammatory, vascular, infectious and toxic disorders. Regarding the previous history of optic neuritis with positive brain MRI (multiple T2 hyperintense lesions in white matter) in our patient, multiple sclerosis is the most probable diagnosis. More effective treatments, such as plasmapheresis and cyclophosphamide, that influenced on the prognosis of some previous patients could be useful, but should be performed before progressive brain atrophy emerges.

摘要

暴发性多发性硬化症(MS)是 MS 最恶性的形式,通常在数周内导致死亡。尽管它可能伴有视神经炎(ON),但 ON 和严重发作之间的长间隔时间尚未报道。暴发性 MS 通常作为首发,以前的 ON 并不常见。我们报告了一例罕见的 30 岁女性病例,该患者 1 年前有 ON 病史。从意识清醒状态迅速恶化到植物人状态,随后出现癫痫发作和以前的 ON,这与以前报道的病例不同。该患者迅速进展性脑白质病的鉴别诊断包括炎症、血管、感染和中毒性疾病。关于我们患者以前的视神经炎病史和阳性脑 MRI(脑白质内多个 T2 高信号病变),多发性硬化症是最可能的诊断。一些以前的患者有效的治疗方法,如血浆置换和环磷酰胺,可能会影响预后,但应在进行性脑萎缩出现之前进行。

相似文献

1
Fulminant multiple sclerosis (MS).暴发性多发性硬化症(MS)。
Neurol Sci. 2011 Oct;32(5):953-7. doi: 10.1007/s10072-011-0723-0. Epub 2011 Aug 24.
2
Pediatric optic neuritis: brain MRI abnormalities and risk of multiple sclerosis.小儿视神经炎:脑部磁共振成像异常与多发性硬化症风险
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Permeability of the blood-brain barrier predicts conversion from optic neuritis to multiple sclerosis.血脑屏障的通透性可预测视神经炎向多发性硬化症的转化。
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The role of NMR imaging in the assessment of multiple sclerosis and isolated neurological lesions. A quantitative study.核磁共振成像在多发性硬化症和孤立性神经病变评估中的作用。一项定量研究。
Brain. 1987 Dec;110 ( Pt 6):1579-616. doi: 10.1093/brain/110.6.1579.
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Assessing structure and function of the afferent visual pathway in multiple sclerosis and associated optic neuritis.评估多发性硬化症及相关视神经炎中传入性视觉通路的结构和功能。
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Optic neuritis in multiple sclerosis.多发性硬化症中的视神经炎。
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Fulminant multiple sclerosis versus autoimmune encephalitis: A case report.暴发性多发性硬化症与自身免疫性脑炎:一例报告。
Clin Case Rep. 2024 Feb 9;12(2):e8508. doi: 10.1002/ccr3.8508. eCollection 2024 Feb.
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Multiple Sclerosis: Inflammatory and Neuroglial Aspects.多发性硬化症:炎症与神经胶质方面
Curr Issues Mol Biol. 2023 Feb 8;45(2):1443-1470. doi: 10.3390/cimb45020094.
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How Repair-or-Dispose Decisions Under Stress Can Initiate Disease Progression.压力下的修复或处置决策如何引发疾病进展。

本文引用的文献

1
Acute disseminated encephalomyelitis.急性播散性脑脊髓炎
Neurol Sci. 2008 Sep;29 Suppl 2:S286-8. doi: 10.1007/s10072-008-0966-6.
2
Idiopathic inflammatory-demyelinating diseases of the central nervous system.中枢神经系统特发性炎性脱髓鞘疾病
Neuroradiology. 2007 May;49(5):393-409. doi: 10.1007/s00234-007-0216-2. Epub 2007 Feb 28.
3
MRI and the diagnosis of multiple sclerosis: expanding the concept of "no better explanation".磁共振成像与多发性硬化症的诊断:拓展“无更好解释”的概念
iScience. 2020 Oct 22;23(11):101701. doi: 10.1016/j.isci.2020.101701. eCollection 2020 Nov 20.
4
Unprovoked seizures in multiple sclerosis: Why are they rare?多发性硬化症中的无诱因发作:为什么它们很少见?
Brain Behav. 2017 May 24;7(7):e00726. doi: 10.1002/brb3.726. eCollection 2017 Jul.
Lancet Neurol. 2006 Oct;5(10):841-52. doi: 10.1016/S1474-4422(06)70572-5.
4
Treatment with interferon beta-1b delays conversion to clinically definite and McDonald MS in patients with clinically isolated syndromes.使用β-1b干扰素治疗可延缓临床孤立综合征患者转变为临床确诊的多发性硬化症及符合麦克唐纳标准的多发性硬化症。
Neurology. 2006 Oct 10;67(7):1242-9. doi: 10.1212/01.wnl.0000237641.33768.8d. Epub 2006 Aug 16.
5
Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria".多发性硬化症的诊断标准:对“麦克唐纳标准”的2005年修订版。
Ann Neurol. 2005 Dec;58(6):840-6. doi: 10.1002/ana.20703.
6
Postinfectious inflammatory disorders: subgroups based on prospective follow-up.感染后炎症性疾病:基于前瞻性随访的亚组分析
Neurology. 2005 Oct 11;65(7):1057-65. doi: 10.1212/01.wnl.0000179302.93960.ad.
7
Acute disseminated encephalomyelitis or multiple sclerosis: can the initial presentation help in establishing a correct diagnosis?急性播散性脑脊髓炎或多发性硬化症:初始表现能否有助于确立正确诊断?
Arch Dis Child. 2005 Jun;90(6):636-9. doi: 10.1136/adc.2004.062935.
8
Marburg type and Balò's concentric sclerosis: rare and acute variants of multiple sclerosis.马尔堡型和巴洛同心性硬化:多发性硬化症的罕见急性变异型。
Neurol Sci. 2004 Nov;25 Suppl 4:S361-3. doi: 10.1007/s10072-004-0341-1.
9
Attack-related severity: a key factor in understanding the spectrum of idiopathic inflammatory demyelinating disorders.与发作相关的严重程度:理解特发性炎性脱髓鞘疾病谱系的关键因素。
J Neurol Sci. 2004 Oct 15;225(1-2):71-8. doi: 10.1016/j.jns.2004.07.003.
10
First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability.儿童期急性中枢神经系统炎性脱髓鞘首发:多发性硬化和残疾的预后因素
J Pediatr. 2004 Feb;144(2):246-52. doi: 10.1016/j.jpeds.2003.10.056.