Disease Information for Diencephalic syndrome

(also called Russell"s syndrome), a rare cause of failure to thrive in infancy and early childhood, first described by Russell in 1950; It is characterized by profound emaciation, normal caloric intake, absence of subcutaneous fat, locomotor hyperactivity, alertness and euphoria; It

commonly occurs in association with chiasmatic and hypothalamic astrocytoma but may be seen in association with other tumours in the suprasellar region including benign lesions such as epidermoid cyst; Posterior fossa tumours may also produce this syndrome; Tumours in children with diencephalic syndrome tend to present earlier and are larger tumours than those without the syndrome;

---[GE Health/Medcyclopaedia website 2006]--------

Diencephalic Syndrome; Diencephalic Syndrome of Childhood;

Diencephalic Syndrome of Emaciation; Paramedian diencephalic Syndrome ; Russell"s Diencephalic Cachexia;

Russell"s Syndrome; Russell"s diencephalic syndrome; Diencephalic cachexia, diencephalia infantilis, diencephalic syndrome of emaciation, diencephalic syndrome of infancy; The diencephalic syndrome is a very rare neurological disorder characterized by failure to thrive, abnormal thinness (emaciation), amnesia, intense sleepiness, unusual eye position and sometimes blindness; It is normally seen in infancy or early childhood but some cases have been reported in older children and even adults; Diencephalic syndrome is usually caused by a brain tumor such as a low-grade glioma or astrocytoma------[NORD website 2006]-----------------------------

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