关键词: AB, antibody ADC, apparent diffusion coefficient ADEM, acute demyelinating encephalomyelitis Brainstem encephalitis CLIPPERS, Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids CNS, cerebral nervous system CSF, cerebrospinal fluid Case report DW, diffusion weighted IVIG, intravenous immunoglobulin LETM, longitudinal extensive transverse myelitis LP, lumbal puncture MOG antibody encephalitis MOG, myelin oligodendrocyte glycoprotein MOG-AB, myelin oligodendrocyte glycoprotein antibody MOGAD, myelin oligodendrocyte glycoprotein antibody disease MRI, magnetic resonance imaging MS, multiple sclerosis NMOSD, neuromyelitis optica spectrum disorder OCB, oligoclonal bands T2w, T2 weighted ceT1w, contrast enhanced T1 weighted

来  源:   DOI:10.1016/j.ensci.2022.100432   PDF(Pubmed)

Abstract:
UNASSIGNED: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a relatively new entity of demyelinating diseases, clinically presenting with optic neuritis, transverse myelitis, or encephalic symptoms. Typical radiological features include demyelinating cerebral and spinal lesions, cortical involvement, leptomeningeal enhancement, or tumefactive lesions. Here we present a rare case of a young patient with extensive brain stem lesion on the MRI while exhibiting nystagmus, singultus and somnolence.
UNASSIGNED: A 30-year-old male patient presented initially with fever and impaired consciousness, but furthermore developed nystagmus, singultus and tetraparesis during the following week. Repeated MRI examinations revealed extensive brain stem edema with notable bilateral affection of the cerebellar peduncles and the pons. Antiviral and antibiotic treatment was changed to intravenous corticosteroids and immunoglobulins as soon as the diagnosis of MOGAD was established by testing serum and cerebrospinal fluid positive for MOG specific antibodies. MRI alterations vanished completely over time with a delayed, nearly complete clinical recovery of our patient.
UNASSIGNED: Brain stem affection in MOGAD is rare. However, in patients presenting with an unclear brain stem encephalitis the possibility of MOGAD should be considered and tested using MOG antibodies. In case of a positive testing treatment with steroids and immunoglobulins seems recommendable.
摘要:
未经授权:髓磷脂少突胶质细胞糖蛋白抗体病(MOGAD)是一种相对较新的脱髓鞘疾病,临床表现为视神经炎,横贯性脊髓炎,或脑症状。典型的放射学特征包括脱髓鞘脑和脊髓病变,皮质受累,软脑膜增强,或根瘤性病变。在这里,我们介绍了一个罕见的病例,一个年轻的患者,在MRI上有广泛的脑干病变,同时表现出眼球震颤,单身和嗜睡。
未经评估:一名30岁男性患者最初出现发热和意识障碍,但还发展了眼球震颤,在接下来的一周中,单例和四瘫。反复的MRI检查显示广泛的脑干水肿,小脑柄和脑桥的双侧病变明显。通过检测血清和脑脊液中MOG特异性抗体阳性,一旦确定MOGAD的诊断,就将抗病毒和抗生素治疗改为静脉注射皮质类固醇和免疫球蛋白。MRI改变随着时间的推移完全消失,我们的病人几乎完成了临床康复。
未经证实:MOGAD的脑干病变很少见。然而,对于患有不清楚脑干脑炎的患者,应考虑MOGAD的可能性,并使用MOG抗体进行检测.在阳性测试的情况下,用类固醇和免疫球蛋白治疗似乎是值得推荐的。
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