BENIGN INTRACRANIAL hypertension has been reported as a complication of prolonged steroid therapy by a number of authors. The following case report describes the occurrence of the syndrome in a child with congenital adrenal hyperplasia who had been treated with steroids for nine years before she manifested the syndrome of pseudotumor cerebri. From this report and a review of the literature, it would appear that patients with the syndrome of pseudotumor cerebri following steroid therapy should be treated in a conservative fashion.
Report of a CaseA 10-year-old white girl (NCBH 23 47 60) was diagnosed at 10 days of age as having congenital adrenal hyperplasia of the salt-losing type. She was admitted to the North Carolina Baptist Hospital with a history of intermittent vomiting of five days' duration. One sibling with enlarged genitalia died at 14 days of age.Physical examination revealed a female infant with an enlarged clitoris. Laboratory studies were diagnostic of congenital adrenal hyperplasia. She responded to treatment and was dis¬ charged on a regimen of 25 mg cortisone ace¬ tate (Cortone) intramuscularly (IM) weekly, 25 mg desoxycorticosterone trimethylacetate (Doca) IM monthly, and a daily addition of 1 gm NaCl to the formula. Because of the NC 27103 (Dr. Kelsey). difficulty in regulation of her serum electro¬ lytes, changes were made in the therapeutic program; pertinent data concerning her subse¬ quent course are summarized in the Table. Severe generalized headaches and four days of vomiting resulted in her most recent admis¬ sion to the North Carolina Baptist Hospital when she was 9 years old. Physical examina¬ tion revealed a moderately obese female with a temperature of 101 F (38.3 C), pulse rate of 90 beats per minute, respirations, 16, and blood pressure, 110/50. Pertinent findings were an enlarged clitoris, bilateral papilledema and bi¬ lateral Babinski responses. X-ray films of the chest were normal, and those of the skull re¬ vealed prominent saggital and coronal sutures. The serum electrolytes, serum urea nitrogen, blood sugar and hemogram were all normal. Urinary 17-ketosteroids (KS) were 17.5 mg/24 hr. The morning after admission the child de¬ veloped a left abducens palsy. The cortisone acetate dose was increased to 75 mg per day. A brain scan using Technetium was normal. The electroencephalogram showed bilateral diffuse slow-wave activity. A ventriculogram revealed a fluctuating pressure of 90 to 120 mm and a nor¬ mal ventricular system except for the fourth ventricle, which was not well visualized. The ventricular fluid protein was 19 mg/100 ce. A right subtemporal decompression was per¬ formed and at surgery the dura was tight and nonpulsatile. She tolerated this procedure well. During surgery and postoperatively she was given 100 mg hydrocortisone (Solu-Cortef) in saline intravenously. Her dosage of oral corti¬ sone was gradually tapered over the next four days and she was discharged on cortisone, 12.5 mg every 12 hours, fludrocortisone (Florinef), 0.1 mg every 12 hours, and salt tablets...