Gharagozloo A M, Poe L B, Collins G H
Department of Radiology, State University of New York, Syracuse 13210.
Radiology. 1994 Jun;191(3):817-9. doi: 10.1148/radiology.191.3.8184071.
To report a case of a rare demyelinating disease called Baló concentric sclerosis.
A 32-year-old woman had left hemiparesis that progressed to hemiplegia, hemihypesthesia, and left hemianopsia. Laboratory evaluation was notable only for a mildly elevated erythrocyte sedimentation rate and an elevated level of myelin basic protein in cerebrospinal fluid.
The antemortem diagnosis was made on the basis of magnetic resonance (MR) imaging of the brain, which showed numerous areas of concentric demyelination alternating with "spared" white matter. With the exception of a temporary improvement with high-dosage intravenous steroid therapy, the clinical course was monophasic and relentless, leading to death approximately 1 year after the onset of symptoms. Pathologic examination helped confirm the diagnosis.
Because MR imaging shows alternating ringlike lesions involving the deep and superficial white matter, which correspond to pathologic findings, it may play a central role in antemortem diagnosis of this rare disease, leaving more invasive diagnostic procedures for evaluation of equivocal cases.
报告一例名为巴洛同心性硬化的罕见脱髓鞘疾病病例。
一名32岁女性出现左侧偏瘫，进而发展为半身不遂、偏身感觉减退和左侧偏盲。实验室检查仅发现红细胞沉降率轻度升高以及脑脊液中髓鞘碱性蛋白水平升高。
根据脑部磁共振成像做出生前诊断，该成像显示多个同心性脱髓鞘区域与“未受累”白质交替出现。除了高剂量静脉注射类固醇治疗后有短暂改善外，临床病程呈单相且进展迅速，症状出现约1年后导致死亡。病理检查有助于确诊。
由于磁共振成像显示累及深部和浅部白质的交替环状病变，与病理结果相符，因此在这种罕见疾病的生前诊断中可能起核心作用，对于疑难病例则留用更具侵入性的诊断程序进行评估。
