January 3, 2011
Diencephalic syndrome, also known as Russell syndrome, is characterized by a hypervigilant infant with preserved appetite but failure to thrive and marasmus. This syndrome has been described in association with tumors of the hypothalamic-optic chiasm region, mainly pilocytic astrocytoma due to hypothalamic dysfunction.
Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis of a hypothalamic/chiasmatic astrocytoma might be delayed. The astrocytomas associated with this syndrome have a tendency to be larger and more aggressive than other astrocytomas in young children.
Submitted by Rhadika Dhamija, MD
Dr. Dhamija reports no disclosures.
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