Effect of corticotrophin on hypothalamic syndrome in children.

Bauer (1954), in reviewing 60 cases of hypothalamic syndrome reported at all ages in the literature, named the principal signs in descending order of frequency as precocious puberty, hypogonadism, diabetes insipidus, psychic disturbances, somnolence, obesity, thermodysregulation, emaciation, convulsions, sphincter loss, bulimia, anorexia and dyshydrosis. Some of the above signs are clearly due to abnormal secretion by the pituitary gland, and those specifically resulting from a deficiency of adrenocorticotrophic hormone might be expected to respond to replacement therapy since the value of corticotrophin (or of cortisone) is well established in the prevention and treatment of hypopituitary coma (Caughey, 1958). The pituitary gland is under the control of the hypothalamus and it is not clear whether certain signs are due purely to hormone deficiency or to direct interference with the hypothalamic vegetative centres; this applies to disturbance of appetite and body weight, mental alertness and body temperature. Damage to hypothalamic centres by an astrocytoma causes emaciation and increased appetite in the syndrome described by Russell (Russell, 1957; Kagan, 1958); these symptoms do not respond to corticotrophin in spite of other evidence of depression of pituitary function. Damage to the vegetative centres probably also explains the failure to respond to corticotrophin or cortisone in some cases of collapse following operation in the hypothalamic region (Northfield, 1955). We now report four cases of hypothalamic syndrome in whom a stuporose state responded well to treatment with corticotrophin, which suggests that the underlying cause might be anterior pituitary deficiency rather than damage to the vegetative centres. Anorexia and wasting, which accompanied the stuporose state in two of the cases, also responded to corticotrophin. A space-occupying lesion at the base of the brain was accompanied in all these cases by an endocrine disturbance, in three cases by diabetes insipidus and in one by precocious puberty. The four cases came under the care of Mr. Wylie McKissock at The Hospital for Sick Children, Great Ormond Street.