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长节段横贯性脊髓炎:同一种疾病,不同结局——病例系列

Longitudinally Extensive Transverse Myelitis: One Disease, Variable Outcomes-A Case Series.

作者信息

Chandrasekar Shyam, John Joseph, Satapathy Amit Kumar

机构信息

Department of Pediatrics, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.

出版信息

J Neurosci Rural Pract. 2022 Mar 11;13(2):339-342. doi: 10.1055/s-0042-1743211. eCollection 2022 Apr.

Abstract

Longitudinal extensive transverse myelitis (LETM) is a rare form of widespread inflammation of the spinal cord causing T2 hyperintensity in spinal magnetic resonance imaging (MRI) extending across three or more vertebral segments. It is an acute onset of sensory, motor, and autonomic dysfunction of variable etiology with a likely poor outcome. We present a case series of three cases wherein children between the ages of 4 and 13 years had diverse symptoms from gradual painless loss of vision in both eyes with headache, vomiting and seizure, and a normal central nervous system examination except involvement of the optic nerve to another child with abdominal pain, urinary retention and constipation for 3 days with exaggerated DTR, and patchy sensory loss without any definite sensory level, and to the third child with fever and weakness of lower limbs, hypotonia and grade 1-2/5 power in lower limbs and normal upper limb power. Contrast-enhanced MRI spine of all children showed long segment T2 hyperintensity with variable involvement of the brain. The first two children were treated with pulsed dose methylprednisolone, and the last child received intravenous immunoglobulin followed by methylprednisolone. All were followed with oral prednisolone. LETM has a varied presentation with different etiologies. Antineuromyelitis optica immunoglobulin G (IgG) antibody (Aquaporin-4 IgG) and antimyelin oligodendrocyte glycoprotein antibody are strongly recommended though they may not be locally available or not affordable. Early and aggressive immunomodulatory therapy may help faster recovery, as did with two of our three children.

摘要

长节段横贯性脊髓炎(LETM)是一种罕见的脊髓广泛炎症形式,在脊髓磁共振成像(MRI)中导致T2高信号,跨越三个或更多椎体节段。它是一种病因多样的急性感觉、运动和自主神经功能障碍,预后可能较差。我们报告了一组三例病例,其中4至13岁的儿童有不同症状,从双眼逐渐无痛性视力丧失伴头痛、呕吐和癫痫发作,除视神经受累外中枢神经系统检查正常,到另一名儿童出现腹痛、尿潴留和便秘3天,腱反射亢进,感觉减退呈斑片状且无明确感觉平面,再到第三名儿童出现发热和下肢无力、肌张力减低以及下肢肌力1 - 2/5级而上肢肌力正常。所有儿童的脊柱增强MRI均显示长节段T2高信号,脑部受累情况各异。前两名儿童接受了脉冲剂量甲泼尼龙治疗,最后一名儿童接受了静脉注射免疫球蛋白,随后使用甲泼尼龙。所有患儿均接受口服泼尼松龙随访。LETM有多种不同病因的表现形式。强烈推荐检测抗视神经脊髓炎免疫球蛋白G(IgG)抗体(水通道蛋白4 IgG)和抗髓鞘少突胶质细胞糖蛋白抗体,尽管它们可能在当地无法获取或价格昂贵。早期积极的免疫调节治疗可能有助于更快恢复,我们的三个孩子中有两个就是如此。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e071/9187388/e7de7a5e5952/10-1055-s-0042-1743211-i2151707-1.jpg

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