What is Diencephalic Syndrome?

January 5, 2011

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E-Pearl of the Week

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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.

Reference

  1. Poussaint TY, Barnes PD, Nichols K et al. Diencephalic syndrome: Clinical features and imaging findings. Am J Neuroradiol 1997; 18: 1499-1505

Submitted by Rhadika Dhamija, MD

Dr. Dhamija reports no disclosures.

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