同心圆硬化12例临床及影像学分析

Clinical characteristics of Baló＇s concentric sclerosis： analysis of 12 cases

目的 探讨同心圆硬化的临床表现、影像学特征及其诊断价值.方法 回顾12例同心圆硬化患者（门诊2例,住院10例）的临床表现、实验室检查结果、影像学特点及病理结果,并对患者进行随访.结果 12例患者（男4例,女8例）发病年龄8～54岁（平均36.8岁）,多以淡漠少语、反应迟钝起病（7例）,病程中其他临床表现以智能减退（10例）、肢体活动障碍（8例）、大小便失禁（7例）、肌力减退（7例）、锥体束征阳性（6例）较多见.8例患者行脑脊液髓鞘碱性蛋白检查,其中7例增高.6例行脑活体组织检查,均可见分层脱髓鞘改变,有炎性细胞浸润.病灶数量1例为单发,11例为多发.4例患者行头颅CT检查,病灶均呈低密度影,增强后无强化.MRI检查均可见圆或类圆形影像,增强扫描8例可见环状或半环状强化,易被误诊为脑肿瘤;3例呈同心圆样强化.结论 同心圆硬化常亚急性或慢性起病,首发症状多为认知功能障碍,易被误诊,头颅MRI有特征表现,有诊断价值,激素治疗有效,预后良好,呈良性临床过程.

Objective To explore the clinical features, imaging and their diagnosis vaule of Baló＇s concentric sclerosis （BCS）. Methods The clinical manifestation, laboratory examination, neuroimaging characteristics, pathology and follow-up of 12 （2 outpatients and 10 inpatients） cases of BCS were analyzed retrospectively. Results The onset age of 12 patients （male 4, female 8） ranged from 8 to 54 years old （mean 36.8 years）. Unconcerned and slowness response were common in the disease onset （7 cases）.Other symptoms and signs such as hypophrenia （ 10 cases）, limitation of limb or hand movement （8 cases）,urinary and fecal incontinence （7 cases）, hypo-myodynamia （7 cases）, positive pyramidal sign （6 cases）were not rare in course of disease. Eight cases underwent the examination of cerebrospinal fluid myelin basic protein, which increased in 7 cases. Demyelinating change and perivascular inflammatory infiltration were shown in 6 brain biopsies. The involvements of multi-lesions were found in 11 cases. Four patients underwent CT scanning and all the cases showed with hypodense lesions and couldn＇ t be enhanced. On MRI, all cases appeared round or round-like in shape. Enhanced lesions showed ring-like or half-ting-like shape （8 cases） and concentric ring （3 cases）. It＇s easy to be misdiagnosed as brain tumor （8 cases）.Conclusions BCS is a kind of demyelinating disease of subacute or chronic onset accompanied with cognitive disorder being the most common initiating symptoms. It＇s prone to be misdiagnosed. Diagnosis depends on MRI and corticosteroid treatment is effective. BCS has a good prognosis and presents with benign clinical course.