The Diencephalic Syndrome of Infancy

Intestinal Abnormalities with Central Nervous System Malignancy

“…For example , as many as 75% of infants with brain tum or s have signific ant vomi ting (14,15) . In addition, stools in these children have a we ll-recognized tenden cy to be looser th an usua l, although no defects of fat or ca rbohydra te absorption ca n be regul arl y demon strat ed (11,13,(16)(17)(18)(19)(20). The cases presented here are illustrati ve.…”

“…Dela y in di agn osi s appears to occ ur for at least two reason s. In so me, abnor malities suc h as nyst agmu s and pa pillede ma that may indica te the CNS as the source of the problem are not present (13)(14)(15). In addi tio n, th e gastrointestinal tract ma y erroneou sly appear to be the more likel y source of growth failure (13,16,17). For example , as many as 75% of infants with brain tum or s have signific ant vomi ting (14,15) .…”

“…In Ca se 1, t he abn ormal bar ium co ntras t study co mbi ned with the presen ce of increased stoo l fat prompted small bowel biop sy, and th e abnormalities see n were initially the onl y o nes lo c alizing th e illness to a s pecific o rga n sys te m. Con sequentl y, this infant was tre ated transiently with a gluten and milk prot ein-free diet. In fact, glute n-free diets appear to be instituted in infants with CNS neoplasm s with some frequ ency, even whe n there is no evidence of malab sorption (11,17). Th e infant re presented in Case 2 also demon strated the mild small bowel abnormalities th at can re sult from sta rvation, and only after obtaining a second CT scan was th e corre ct diagnosis mad e.…”

Hypothalamic Symptomatology and its Relationship to Diencephalic Tumor in Childhood

“…The associated vomiting is usually too inconstant to explain the fat loss. Attempts to explain the losses on the basis of excessive caloric expenditure from hyperkinetic behavior do not satisfy all the cases since this feature is not always present [11]. Spec ulation exists that the decrease in subcutaneous fat is related to a hypoth alamic mechanism possibly involving a fat mobilizing substance as yet un identified.…”

An Uncommon and Often Overlooked Cause of Failure to Thrive: Diencephalic Syndrome

“…Diencephalic syndrome (DS) is a complex of signs and symptoms related to hypothalamic dysfunction. Since the first description of the association between severe FTT and brain tumors of the anterior hypothalamus, many cases have been reported (1–5). This syndrome is almost exclusively seen in infants and young children, and most patients have a space‐occupying lesion of the hypothalamic–optic chiasm region, mainly of low‐grade glioma (4).…”