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[与多发性硬化症病变相关的脑淋巴瘤]

[Cerebral lymphoma associated with lesions of multiple sclerosis].

作者信息

Gherardi R, Salama J, Gray F, Kemeny J L, Delaporte P, Poirier J, Cambier J

出版信息

Rev Neurol (Paris). 1985;141(6-7):456-63.

PMID:4089407
Abstract

A 24 year-old man experienced a left retrobulbar neuritis which improved completely after 2 months of non-steroid antiinflammatory therapy. One month after the end of the treatment he developed a Korsakoff-like amnestic syndrome. Three months later he complained of horizontal diplopia. A CT Scan showed a diffuse enhancement of the periventricular areas, corpus callosum and fornix. Diplopia and CT scan abnormalities disappeared after the administration of tetracosactide. Subsequently a progressive worsening of the neurological condition developed, including a 1 1/2 syndrome of Fisher. In C.S.F. proteins ranged from 35 to 66 mg/dl, gammaglobulins from 4 to 5 per cent, cells from 2.2 to 6.8 per mm3 without abnormal cells. Rounded areas of enhancement were observed on CT scan in pons and right occipital lobe. Usual biological tests, abdominal echography and lymphography were normal. Death occurred 15 months after the onset of symptoms. Neuropathological examination showed: 1) a cerebral lymphoma of probable B origin with distinct masses in right occipital lobe and pontine tegmentum and a more diffuse perivascular infiltration on the left side in the amygdaloid nucleus, fourth temporal gyrus, sublenticular area, hypothalamus and in the right internal capsule; 2) multiple small clear-cut foci of demyelination with myelin-axonal dissociation bilaterally in the optic pathways, periventricular regions, corona radiata, cerebral and cerebellar white matter, sublenticular areas, temporal lobes, splenium of the corpus callosum and fornices with secondary atrophy of the mamillary bodies. Both recent and old plaques were observed. Inflammatory perivascular cuffing, when present, consisted of small nontumoral lymphocytes.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

一名24岁男性经历了左侧球后视神经炎,在接受2个月的非甾体抗炎治疗后完全康复。治疗结束1个月后,他出现了科萨科夫样遗忘综合征。3个月后,他抱怨有水平性复视。CT扫描显示脑室周围区域、胼胝体和穹窿弥漫性强化。给予二十四肽促皮质素后,复视和CT扫描异常消失。随后,神经状况逐渐恶化,包括费希尔1 1/2综合征。脑脊液蛋白含量为35至66mg/dl,γ球蛋白为4%至5%,细胞数为每立方毫米2.2至6.8个,无异常细胞。CT扫描显示脑桥和右枕叶有圆形强化区域。常规生物学检查、腹部超声检查和淋巴造影均正常。症状出现15个月后死亡。神经病理学检查显示:1)可能起源于B细胞的脑淋巴瘤,在右枕叶和脑桥被盖有明显肿块,左侧杏仁核、颞叶第四回、豆状核下区、下丘脑和右内囊有更弥漫的血管周围浸润;2)双侧视神经通路、脑室周围区域、放射冠、大脑和小脑白质、豆状核下区、颞叶、胼胝体压部和穹窿有多个小而清晰的脱髓鞘病灶,伴有髓鞘-轴突分离,乳头体继发性萎缩。观察到近期和陈旧性斑块。血管周围有炎症性套袖时,由小的非肿瘤性淋巴细胞组成。(摘要截取自250字)

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