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一例MRI显示丘脑病变的早发性多发性硬化症病例。

An early-onset case of multiple sclerosis with thalamic lesions on MRI.

作者信息

Asai K, Inagaki M, Maegaki Y, Yamamoto T, Suzaki I, Ohta S

机构信息

Department of Pediatrics, Matsue Red Cross Hospital, Japan.

出版信息

Acta Paediatr Jpn. 1994 Aug;36(4):431-4. doi: 10.1111/j.1442-200x.1994.tb03217.x.

Abstract

We present here an early-onset case of multiple sclerosis (MS) with thalamic lesions. The patient first experienced an episode of ataxic gait at 2 years 3 months of age, with spontaneous remission within 1 month. At 5 years 4 months, she was admitted because of cerebellar ataxia, oculomotor restriction and feeding difficulty. Magnetic resonance imaging (MRI) showed multiple well-defined lesions in the white matter of the cerebellum, mid-brain, periventricle and right frontal lobe. Cerebrospinal fluid (CSF) showed a mild elevation of both immunoglobulin G (IgG) and myelin basic protein (MBP). Serum anti-myelin antibody was also positive, although leukocytosis and elevation of C-reactive protein were not found. Methylprednisolone pulse therapy relieved symptoms within 2 weeks and the abnormal MRI and CSF findings gradually improved. At 6 years 6 months of age, she incurred a third episode of cerebellar ataxia and disturbance of consciousness. Magnetic resonance imaging revealed recurrence and extension of the previous lesions as well as new lesions in the thalamus and internal capsule. CSF IgG and MBP level showed a higher elevation than in the second episode. The combination of the cerebellar, brain-stem, cerebral and thalamic lesions with remission and exacerbation, supported by MRI and CSF findings, allowed the diagnosis of clinically definite MS to be made. This is one of the youngest cases of MS yet described, with the first attack occurring at 27 months of age. In addition, this case is unique for the involvement of the gray matter in the thalamus.

摘要

我们在此报告一例患有丘脑病变的早发性多发性硬化症(MS)病例。该患者在2岁3个月大时首次出现共济失调步态发作,1个月内自发缓解。5岁4个月时,她因小脑共济失调、眼球运动受限和进食困难入院。磁共振成像(MRI)显示小脑、中脑、脑室周围和右额叶白质中有多个边界清晰的病变。脑脊液(CSF)显示免疫球蛋白G(IgG)和髓鞘碱性蛋白(MBP)均轻度升高。血清抗髓鞘抗体也呈阳性,尽管未发现白细胞增多和C反应蛋白升高。甲基强的松龙冲击疗法在2周内缓解了症状,MRI和脑脊液的异常表现逐渐改善。6岁6个月时,她出现了第三次小脑共济失调和意识障碍发作。磁共振成像显示先前病变复发并扩大,丘脑和内囊出现新病变。脑脊液IgG和MBP水平比第二次发作时升高得更高。小脑、脑干、大脑和丘脑病变伴缓解和加重,并有MRI和脑脊液检查结果支持,从而确诊为临床确诊的MS。这是迄今报道的最年轻的MS病例之一,首次发作发生在27个月大时。此外,该病例因丘脑灰质受累而独特。

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