Brain abnormalities in neuromyelitis optica
Brain abnormalities in neuromyelitis optica
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DOI：
10.1001/archneur.63.3.390
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发表时间：
2006-03-01
影响因子：
--
通讯作者：
Weinshenker, BG
中科院分区：
文献类型：
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作者：
Pittock, SJ;Lennon, VA;Weinshenker, BG
背景：视神经脊髓炎（NMO）是一种严重的脱髓鞘疾病，主要是由于其倾向于选择性地影响视神经和脊髓，导致失明和瘫痪的反复发作。当代诊断标准要求没有视神经或脊髓以外的临床疾病。然而，我们经常遇到有明确诊断为NMO的患者，他们要么无症状，要么有症状性脑损伤，这表明NMO的诊断标准应该修改。目的：探讨NMO患者的MRI表现。设计：观察性、回顾性病例系列患者：我们通过临床生物标本数据库确定患有或疑似NMO的患者。我们纳入了以下患者：(1)满足1999年Wingerchuk等人的NMO标准，除了视神经和脊髓以外没有症状的绝对标准和发病时脑MRI正常的支持性标准；(2)有MRI证据表明脊髓病变延伸3个或更多节段（区分NMO和MS的最具体的非血清学特征）；(3)在梅奥诊所进行神经学和脑MRI评估。主要结局指标：磁共振图像分为正常或异常，包括非特异性、多发性硬化症样或非典型异常。我们评估脑病变是否有症状，并分析单个脑活检标本的神经病理特征。结果：60例患者（53例女性，88%）符合纳入标准。发病时平均+/- SD年龄为37.2 +/- 18.4岁，平均SD随访时间为6.0 +/- 5.6年。41例（68%）患者检出视神经脊髓炎igg。36例（60%）患者发现脑MRI病变。大多数是非特异性的，但6例（10%）患者有多发性硬化样病变，通常无症状。另有5例（8%）患者，多为儿童，间脑、脑干或大脑病变，不典型多发性硬化症。出现时，脑受累症状不明显，但1例患者处于昏迷状态并有较大的脑损伤。结论：无症状性脑损伤在NMO中很常见，有症状性脑损伤不能排除NMO的诊断。这些观察结果证明修订NMO的诊断标准是合理的，以允许大脑受累。
Background: Neuromyelitis optica (NMO) is a severe demyelinating disease defined principally by its tendency to selectively affect optic nerves and the spinal cord causing recurrent attacks of blindness and paralysis. Contemporary diagnostic criteria require absence of clinical disease outside the optic nerve or spinal cord. We have, however, frequently encountered patients with a well-established diagnosis of NMO in whom either asymptomatic or symptomatic brain lesions develop suggesting that the diagnostic criteria for NMO should be revised.Objective: To describe the magnetic resonance image (MRI) brain findings in NMO.Design: Observational, retrospective case seriesPatients: We ascertained patients through a clinical biospecimens database of individuals with definite or suspected NMO. We included patients who (1) satisfied the 1999 criteria of Wingerchuk et al for NMO except for the absolute criterion of lacking symptoms beyond the optic nerve and spinal cord and the supportive criterion of having a normal brain MRI at onset; (2) had MRI evidence of a spinal cord lesion extending 3 vertebral segments or more (the most specific nonserological feature to differentiate NMO from MS); and (3) were evaluated neurologically and by brain MRI at the Mayo Clinic.Main Outcome Measures: Magnetic resonance images were classified as normal or as abnormal with either nonspecific, multiple sclerosis-like or atypical abnormalities. We evaluated whether brain lesions were symptomatic and analyzed the neuropathologic features of a single brain biopsy specimen.Results: Sixty patients (53 women [88%]) fulfilled these inclusion criteria. The mean +/- SD age at onset was 37.2 +/- 18.4 years and the mean SD duration of follow-up was 6.0 +/- 5.6 years. Neuromyelitis optica-IgG was detected in 41 patients (68%). Brain MRI lesions were detected in 36 patients (60%). Most were nonspecific, but 6 patients (10%) had multiple sclerosis-like lesions, usually asymptomatic. Another 5 patients (8%), mostly children, had diencephalic, brainstem or cerebral lesions, atypical for multiple sclerosis. When present, Symptoms of brain involvement were subtle, except in I patient who was comatose and had large cerebral lesions.Conclusions: Asymptomatic brain lesions are common in NMO, and symptomatic brain lesions do not exclude the diagnosis of NMO. These observations justify revision of diagnostic criteria for NMO to allow for brain involvement.