The Diencephalic Syndrome of Russell: A Case Report.
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Date
2010-01
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Abstract
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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Keywords
Childhood tumors, glioma, hydrocephalus, russells syndrome, suprasellar mass
Citation
Roka Y B, Paudel G, Bidur K C, Munakomi S. The Diencephalic Syndrome of Russell: A Case Report. Journal of Nepal Paediatric Society. 2010 Jan-Apr; 30(1): 60-63.