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Clinical spectrum of inflammatory central nervous system demyelinating disorders associated with antibodies against myelin oligodendrocyte glycoprotein.
Neurochemistry international ( IF 4.4 ) Pub Date : 2018-10-23 , DOI: 10.1016/j.neuint.2018.10.016
Tetsuya Akaishi 1 , Douglas Kazutoshi Sato 2 , Toshiyuki Takahashi 3 , Ichiro Nakashima 4
Affiliation  

Immunoglobulin G (IgG) antibodies against myelin oligodendrocyte glycoprotein (MOG) are detected in the serum of some patients with demyelinating diseases. These patients are known to show repeated clinical episodes of inflammatory demyelinating attacks in the central nervous system. Although the associated pathogenicity and mechanism of inflammatory demyelination remains inconclusive, it is known that patients with MOG-IgG antibodies have a different clinical spectrum from those with other demyelinating diseases, such as multiple sclerosis. Based on our database of 85 MOG-IgG positive (+) cases, the most frequently associated clinical episodes were isolated optic neuritis (67.5%), encephalitis (26.5%), and myelitis (19.3%). Optic neuritis in MOG-IgG (+) disease usually involves the long segment of optic nerves and sometimes happens bilaterally, but visual acuity usually recovers with proper treatment in the acute phase. Brain and brainstem lesions usually present vague and focal appearances with irregular margins, typically in subcortical or brainstem regions, but occasionally in the cortex or corpus callosum. Due to these characteristics, MOG-IgG (+) cases with brain or brainstem lesions are sometimes diagnosed with acute disseminated encephalomyelitis, meningitis, or symptomatic epilepsy. The myelitis in MOG-IgG (+) typically shows longitudinally extensive lesions as seen in neuromyelitis optica spectrum disorders. Acute treatment to reduce attack-related disability is recommended in MOG-IgG (+) disease, and long-term immunosuppression may be considered in patients with a high frequency of relapses and/or high risk of neurological disability.

中文翻译:

与抗髓磷脂少突胶质细胞糖蛋白抗体相关的炎症性中枢神经系统脱髓鞘疾病的临床范围。

在一些患有脱髓鞘疾病的患者的血清中检测到了针对髓磷脂少突胶质细胞糖蛋白(MOG)的免疫球蛋白G(IgG)抗体。已知这些患者在中枢神经系统中反复出现炎性脱髓鞘性发作的临床发作。尽管相关的致病性和炎症性脱髓鞘的机制尚无定论,但已知具有MOG-IgG抗体的患者与其他具有脱髓鞘性疾病(例如多发性硬化症)的患者具有不同的临床范围。根据我们对85例MOG-IgG阳性(+)病例的数据库,最常见的临床发作是单纯性视神经炎(67.5%),脑炎(26.5%)和脊髓炎(19.3%)。MOG-IgG(+)疾病中的视神经炎通常累及视神经的长段,有时发生在双侧,但是视力通常在急性期通过适当的治疗即可恢复。脑部和脑干病变通常表现为模糊不清和局灶性外观,边缘不规则,通常出现在皮层下或脑干区域,但偶尔出现在皮层或lesions体中。由于这些特征,有时会诊断出患有脑或脑干病变的MOG-IgG(+)病例为急性播散性脑脊髓炎,脑膜炎或有症状的癫痫病。如在视神经脊髓炎光谱疾病中所见,MOG-IgG(+)中的脊髓炎通常显示出纵向广泛的病变。在MOG-IgG(+)疾病中,建议采取急性治疗以减少与发作相关的残疾,对于复发频率高和/或神经系统残疾风险高的患者,可以考虑长期免疫抑制。
更新日期:2018-10-23
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