Diencephalic syndrome

Diencephalic syndrome, diencephalic syndrome of emaciation or Russell's syndrome is a rare neurological disorder seen in infants and children and characterised by failure to thrive and severe emaciation despite normal or slightly decreased caloric intake, locomotor hyperactivity, and euphoria. Less commonly it may involve skin pallor without anaemia, hypoglycaemia, and hypotension. The syndrome is a rare but potentially fatal cause of failure to thrive in children. Failure to thrive presents on average at 7 months of age. Of note the syndrome is not associated with developmental delay. There may be associated hydrocephalus.