The diencephalic syndrome (Russell syndrome) is a rare cause for failure to thrive in an infant or young child.


Causes of the diencephalic syndrome may include:

(1) glioma in the region of the optic chiasm or hypothalamus

(2) midline cerebellar astrocytoma

(3) suprasellar tumor (ependymoma, spongioblastoma)

(4) thalamic tumor


Clinical features may include:

(1) failure to thrive

(2) emaciation (cachexia) despite normal caloric intake

(3) hyperactivity/hyperkinesis

(4) hyperalertness

(5) euphoria

(6) headache

(7) nystagmus or strabismus

(8) visual field defect

(9) optic pallor

(10) proptosis

(11) vomiting

(12) no evidence of malabsorption


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