Da Motta L A, Garcia Filho O V, Horta M P, Lima B O, Pratesi R, Farage Filho M
Unidade de Neurocirurgia do Hospital de Base do Distrito Federal, Brasília, Brasil.
Arq Neuropsiquiatr. 1990 Mar;48(1):107-12. doi: 10.1590/s0004-282x1990000100016.
The case of a male child with Russel's syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th months of life, when he started losing weight. By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nystagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/1) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).
本文报告了一例因位于间脑区域的毛细胞型星形细胞瘤导致Russel综合征的男童病例。诊断于16月龄时作出，但症状始于4月龄，当时他开始体重减轻。入院时体重6150克，身高74厘米，面容消瘦，无皮下脂肪，烦躁不安，并有颅内高压症状。存在会聚性斜视、左眼垂直性眼球震颤和双侧视乳头水肿。腱反射亢进，并有痉挛性四肢轻瘫。内分泌评估显示生长激素（GH，23ng/ml）、促甲状腺激素（TSH，6.2mUI/1）和催乳素（26ng/ml）基础值升高。前两种激素对促甲状腺激素释放激素（TRH）的急性试验无反应，而催乳素反应不佳。他接受了直线加速器放射治疗（总剂量4000拉德）和手术，但由于肿瘤体积大，手术未能将其完全切除。9个月后，患儿情况良好，体重显著增加（2500克）。
