Castaigne P, Escourolle R, Chain F, Foncin J F, Gray F, Sauron B, Duyckaerts C
Rev Neurol (Paris). 1984;140(8-9):479-87.
A clinico-pathological case of concentric sclerosis (Baló type) is reported. A 30-year-old man experienced dizziness, nausea and vomiting. Twelve days later he developed gait disturbances. Neurological examination showed broad based gait, brisk tendon reflexes, bilateral extensor plantar responses, right hemihypoesthesia, cerebellar dysmetria, and a left lateral gaze palsy. CSF examination showed, 520 mg p. 100 ml protein, 7500 red blood cells, 31 lymphocytes and 9 polymorphonuclear leukocytes/mm3, 18 p. 100 gammaglobulin. Three CT scans were performed and showed a round hypodensity in the parieto-occipital white matter with contrast enhancement on one occasion, and several other hypodensities in the contralateral parieto-occipital white matter and in both frontal lobes. 23 days after the onset of the disease, the patient became comatose. A cerebral biopsy was obtained from one of the frontal lesions. He died from aspiration bronchopneumonia 2 months after the first signs. Neuropathological examination showed numerous concentric zones of demyelination which involved the white matter of both hemispheres, brain stem, and cerebellum. On light microscopy sudanophilic myelin breakdown products were numerous in the bands of demyelinisation. Astrocytic proliferation was marked, with frequent Rosenthal fibers. Edema was noted in some lesions. Myelin-axonal dissociation was obvious, but some axonal swelling were observed. Electron microscopy demonstrated the integrity of oligodendrocytes and of blood vessels and confirmed the prominent alterations of the astrocytes. Fifteen similar cases of the literature have been reviewed. The present case seems to be the first one with CT scan examination and electron microscopic study of a brain biopsy. The nosological situation of Baló's disease among the inflammatory demyelinating diseases of the group of MS is discussed.
报告了一例同心性硬化（巴洛型）的临床病理病例。一名30岁男性出现头晕、恶心和呕吐。12天后出现步态障碍。神经系统检查显示步态基底增宽、腱反射亢进、双侧巴氏征阳性、右侧半身感觉减退、小脑辨距不良以及左侧凝视麻痹。脑脊液检查显示，蛋白100ml中含520mg，红细胞7500个，淋巴细胞31个，多形核白细胞9个/mm³，γ球蛋白100中含18。进行了三次CT扫描，显示顶枕白质有一个圆形低密度区，有一次出现对比增强，对侧顶枕白质和双侧额叶还有其他几个低密度区。疾病发作23天后，患者昏迷。从额叶病变之一处进行了脑活检。首次出现症状2个月后，患者死于吸入性支气管肺炎。神经病理学检查显示有许多同心性脱髓鞘带，累及双侧大脑半球、脑干和小脑的白质。光镜下，脱髓鞘带中有大量嗜苏丹性髓鞘崩解产物。星形细胞增生明显，可见频繁的罗森塔尔纤维。一些病变中可见水肿。髓鞘-轴突解离明显，但观察到一些轴突肿胀。电子显微镜显示少突胶质细胞和血管完整，并证实了星形细胞的显著改变。回顾了文献中15例类似病例。本病例似乎是首例进行CT扫描检查和脑活检电子显微镜研究的病例。讨论了巴洛病在多发性硬化症组炎性脱髓鞘疾病中的分类情况。
