Replacement treatment with 10 to 15 mg/m2/24 hours of hydrocortisone is indicated, with supplementation during stress at three times the maintenance dosage or the use of intramuscular hydrocortisone. The dose is titrated to allow a normal growth rate. Mineralocorticoid replacement with fludrocortisone is monitored by plasma renin activity and serum sodium and potassium determinations.
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Classic clinical manifestations of Cushing syndrome in children include progressive central or generalized obesity, marked failure of longitudinal growth, hirsutism, weakness, a nuchal fat pad, acne, striae, hypertension, and often hyper-pigmentation when ACTH is elevated. The most frequent cause is exogenous administration in the context of numerous conditions requiring long-term pharmacologic doses of glucocorticoids. Endogenous causes include adrenal adenoma, carcinoma, nodular adrenal hyperplasia, an ACTH-secreting pituitary microadenoma resulting in bilateral adrenal hyperplasia (Cushing disease), or an extremely rare ACTH-secreting tumor. Diagnostic tests include 24-hour urinary cortisol excretion, low-dose dexamethasone suppression test, high-dose dexamethasone suppression test (helps distinguish Cushing syndrome from Cushing disease), and late evening salivary cortisol sampling.
Treatment of Cushing syndrome is directed to the etiology and may include excision of autonomous adrenal, pituitary, or ectopic ACTH-secreting tumors. Rarely adrenalectomy is needed to control the symptoms. Parenteral glucocorticoid therapy is necessary during and immediately after surgical treatment to avoid acute adrenal insufficiency.
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A neurologic evaluation, including history, physical examination, and judicious use of ancillary studies, allows the localization and diagnosis of nervous system pathology. The process andinterpretation of the neurologic examination varies with age; thenewborn is unique with many transient and primitive reflex patterns, whereas the examination of adolescents and adults is similar. Careful evaluation of social, cognitive, language, fine motorand gross motor skills, and their age appropriateness is key.