The Diencephalic Syndrome of Russell: A Case Report

Roka, Y. B.; Paudel, G.; Bidur, K. C.; Munakomi, S.
January 2010
Journal of Nepal Paediatric Society;2010, Vol. 30 Issue 1, p60
Academic Journal
Case Study
Diencephalic Syndrome (DS) is also known as Russells Syndrome. This is associated with marked emaciation, locomotor hyperactivity, vomiting, and absence of obvious neurological signs and loss of subcutaneous fat. A 15-month old child who presented with hyperactivity, loss of weight and failure to thrive since bi rth is reported. On Computed Tomography he had a large supra sellar mass with extension into the third ventricle causing gross hydrocephalus. He underwent biventricular shunting followed by microscopic near total excision of the tumor. The histopathology revealed it to be fibrillary meningioma. Although DS is uncommon it must be kept as a differential diagnosis in all children who fail to grow despite adequate intake.


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