Nystagmus in the Diagnosis of Russell Diencephalic Syndrome

Abstract

Russell diencephalic syndrome is a condition in which infants become emaciated in the setting of a decreased or normal caloric intake as the result of a hypothalamic astrocytoma. The diagnosis may be delayed if providers initially attribute the symptoms to a behavioral disorder. The detection of nystagmus, which is present in many patients, may be a critical diagnostic clue. The authors describe two patients in whom the discovery of nystagmus months after the onset of emaciation led to the diagnosis of Russell diencephalic syndrome. [ J Pediatr Ophthalmol Strabismus . 2019;56:e79–e83.]