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[间脑综合征。病例报告及文献综述]

[Diencephalic syndrome. Case report and review of literature].

作者信息

Baracchini A, Chiaravalloti G, Del Pistoia M, Ceccarelli M

机构信息

Istituto di Clinica Pediatrica, Università degli Studi di Pisa.

出版信息

Minerva Pediatr. 1993 Oct;45(10):407-10.

PMID:8302225
Abstract

We report the case of a twenty-three month old, normally eating child, affected by organic macies and severe dystrophy. After the negative response of blood and laboratory examinations let us exclude a malabsorption syndrome, only the performance of neuro-radiologic exams showed evidence of a subthalamic tumor as the cause of a "Diencephalic syndrome". Diencephalic syndrome or Russel's syndrome is a diencephalic tumor induced disease, which sets in the first three years of life. The disease clinical markers are a severe emaciation with appetite preservation and absence or very scarce evidence of any telltale neurological sign. the tumoral histo-pathology most frequently shows a low grade of malignancy astrocytoma, whose eradication is very often difficult because of the particular anatomic site. Choice treatment includes an excisional biopsy associated to chemotherapy and to radiotherapy.

摘要

我们报告了一例23个月大、饮食正常但患有器质性消瘦和严重营养不良的儿童病例。在血液和实验室检查呈阴性反应,使我们排除吸收不良综合征后,只有神经放射学检查显示有丘脑下肿瘤,这是“间脑综合征”的病因。间脑综合征或拉塞尔综合征是一种由间脑肿瘤引发的疾病,发病于生命的头三年。该疾病的临床特征是严重消瘦但食欲保留,且没有或仅有非常罕见的明显神经体征。肿瘤组织病理学最常显示为低级别恶性星形细胞瘤,由于其特殊的解剖位置,根除往往很困难。首选治疗方法包括切除活检联合化疗和放疗。

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