Clinical characteristics of Baló's concentric sclerosis: analysis of 12 cases
10.3760/cma.j.issn.1006-7876.2011.02.010
- VernacularTitle:同心圆硬化12例临床及影像学分析
- Author:
Changqing LI
;
Xiaokun QI
;
Jianguo LIU
;
Bin XIONG
;
Feng QIU
- Publication Type:Journal Article
- Keywords:
Diffuse cerebral sclerosis of Schilder;
Cognition disorders;
Magnetic resonance imaging
- From:
Chinese Journal of Neurology
2011;44(2):113-116
- CountryChina
- Language:Chinese
-
Abstract:
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.
