Baracchini A, Chiaravalloti G, Del Pistoia M, Ceccarelli M
Istituto di Clinica Pediatrica, Università degli Studi di Pisa.
Minerva Pediatr. 1993 Oct;45(10):407-10.
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.
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。
