What is Diencephalic Syndrome?

January 5, 2011


E-Pearl of the Week

Brought to you by the Resident and Fellow Section of the journal Neurology®.

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.


  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.

For more clinical pearls and other articles of interest to neurology trainees, visit www.neurology.org and click on the link to the Resident and Fellow Pages. Click here to visit the E-Pearl of the Week Archive.

Listen to this week's Neurology® Podcast.