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四例伴有同侧大脑半球萎缩的异位松果体瘤(作者译)

[Four cases of ectopic pinealoma with ipsilateral cerebral hemiatrophy (author's transl)].

作者信息

Kwak R, Suzuki J

出版信息

No Shinkei Geka. 1975 Sep;3(9):731-8.

PMID:1239686
Abstract

The 6 cases of brain tumor with ipsilateral cerebral hemiatrophy in which 4 cases were experienced in our institute and 2 cases were found in literature were studied in this paper. The specific character which was common in 6 cases were observed in their clinical course and the findings of clinical examinations. The histopathological study was proceeded with a autopsy case to observe the correlation between brain tumor and the ipsilateral cerebral hemiatrophy. As the result of it, we discussed the mechanism of appearance of the ipsilateral cerebral hemiatrophy due to brain tumor in the thalamic region. 1) All six cases were very young in which the age of onset was between 8 to 14 years old, 11 years 8 months old in average. 2) Their clinical course was relatively chronic. The period from onset to first admission was between 1 year 2 months to 4 years, 2 years and 1 month in average. 3) The declining of school work and hemiparesis were recognized as the primary sign of their onset. Main symptoms were hemiparesis, dementia, character and emotional change, and abnormal behavior, but sign of increased intracranial pressure was not observed. 4) The findings of carotid angiogram and pneumoence-phalogram showed ipsilateral hemiatrophy on the tumor side. 5) The brain tumor localized in the thalamic region and its surroundings which was common with all six cases. 6) Histopathological diagnosis was pinealoma, and 3 autopsy cases were ectopic pinealoma and the other 3 cases were suspected too as ectopic pinealoma. 7) In our autopsy case, ipsilateral cortical and subcortical atrophy with ectopic pinealoma was observed. As the pathological findins, degeneration and destruction of ganglion cells, demyelination in the subcortex and damage of axon were observed. These findings suggested that the ipsilateral cerebral hemiatrophy was induced by Waller's and retrograde degeneration as the result of the secondary damages of the thalamic ganglion cells and the afferent and efferent nerve fibers, due to invasive tumor into thalamic region.

摘要

本文对6例伴有同侧大脑半球萎缩的脑肿瘤病例进行了研究,其中4例为我院收治,2例来自文献报道。观察了这6例病例在临床病程及各项临床检查结果中所共有的特征。对1例尸检病例进行了组织病理学研究,以观察脑肿瘤与同侧大脑半球萎缩之间的相关性。据此,我们探讨了丘脑区脑肿瘤导致同侧大脑半球萎缩的发生机制。1) 所有6例患者均非常年轻,发病年龄在8至14岁之间,平均为11岁8个月。2) 其临床病程相对缓慢。从发病到首次入院的时间在1年2个月至4年之间,平均为2年1个月。3) 学业成绩下降和偏瘫被认为是其发病的主要表现。主要症状为偏瘫、痴呆、性格和情绪改变以及行为异常,但未观察到颅内压升高的迹象。4) 颈动脉血管造影和气脑造影结果显示肿瘤侧同侧半球萎缩。5) 脑肿瘤均位于丘脑区及其周围,这是所有6例病例的共同特征。6) 组织病理学诊断为松果体瘤,3例尸检病例为异位松果体瘤,另外3例也疑似为异位松果体瘤。7) 在我们的尸检病例中,观察到伴有异位松果体瘤的同侧皮质和皮质下萎缩。作为病理表现,观察到神经节细胞的变性和破坏、皮质下脱髓鞘以及轴突损伤。这些发现表明,由于肿瘤侵入丘脑区,丘脑神经节细胞以及传入和传出神经纤维受到继发性损害,导致同侧大脑半球萎缩是由华勒氏变性和逆行性变性引起的。

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