The syndrome usually occurs in an infant or child, but rarely may occur in an adult.
Features:
(1) emaciation despite normal caloric intake and normal appetite, with markedly reduced body fat (failure to thrive in infant or young child)
(2) motor hyperactivity, irritability, restlessness or tremor
(3) cheerful to euphoric
(4) alert
(5) skin pallor without anemia
(6) profuse sweating or heat intolerance
(7) erratic temperature control
(8) nystagmus
(9) visual field defect
(10) focal neurologic findings are often absent
Associated lesions are usually low grade, slow growing tumors:
(1) chiasmatic or hypothalamic glioma
(2) thalamic tumors
(3) astrocytoma of midline cerebellum or fourth ventricle
(4) suprasellar tumors (ependymoma, spongioblastoma, craniopharyngioma)
(5) rarely vascular aneurysm
