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Balós concentric sclerosis

机译:巴罗斯同心性硬化症

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A previously healthy 13-year-old boy presented with left arm and face weakness. Brain MRI (figure 1, axial Tl postcontrast) revealed an enhancing mass within the right centrum semio-vale, and a second non-enhancing lesion in the left occipital region (not shown). Lumbar puncture revealed five oligoclo-nal bands, giving a working diagnosis of a demyelinating disorder. Intravenous methylprednisolone was given, and his symptoms nearly resolved. Within days, he returned with complete left arm and leg.hemiparesis. Repeat MRI (figure 2, axial Tl postcontrast) revealed a lesion with concentric rings and peripheral enhancement. Based on his clinical picture, oligoclonal bands in the cerebrospinal fluid and characteristic imaging findings, a diagnosis of Balos concentric sclerosis was given. Balos concentric sclerosis is a rare demyelinating disease considered to be a variant of multiple sclerosis. It is an unusual entity in the paediatric population. Typical MRI changes consist of concentric rings or a whorled appearance.1 The characteristic pathological findings are alternating rings of myelin preservation or remyelination and myelin loss, consistent with demyelination, involving the cerebral hemispheres, cerebellum, brain stem, spinal cord and optic chiasm.2 Diagnosis is made via MRI and with the observance of oligoclonal bands in the cerebrospinal fluid.
机译:先前健康的13岁男孩表现出左臂和面部无力。脑部MRI(图1,轴向T1造影剂造影后)显示右中半瓣内肿块增大,左枕骨区域出现第二个非增强性病变(未显示)。腰椎穿刺显示有五个寡克隆带,可确诊为脱髓鞘疾病。静脉注射甲基泼尼松龙,他的症状几乎消失。几天之内,他的左臂和腿部完全偏瘫返回。重复MRI(图2,轴向T1造影后),发现同心环和周围增强的病变。根据他的临床照片,脑脊液中的寡克隆条带和特征性影像学表现,诊断为Balos同心性硬化症。 Balos同心性硬化症是一种罕见的脱髓鞘疾病,被认为是多发性硬化症的一种变体。它在儿科人群中是不寻常的实体。 MRI的典型变化包括同心环或扭曲的外观。1病理特征是髓鞘保存或再髓鞘化和髓鞘丢失交替发生,与脱髓鞘相一致,涉及脑半球,小脑,脑干,脊髓和视交叉2。通过MRI进行诊断,并观察脑脊液中的寡克隆带。

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