Isotretinoin for acne in Apert syndrome.

Apert syndrome is a congenital disorder consisting primarily of deformities of the skull, hands, and feet. These individuals are also prone to severe acne, particularly involving the upper arms and forearms. Conventional therapy is often ineffective. We report the successful use of isotretinoin for acne in a patient with Apert syndrome.

McCune-Albright syndrome with multiple bilateral café au lait spots.

A 7-week-old infant developed hyperpigmented lesions at 5 days of age that gradually progressed to sharply demarcated, medium-brown macules of unusual configuration involving the neck, trunk, buttocks, upper arms, and right upper thigh. A biopsy specimen of a representative lesion showed changes of epidermal melanosis consistent with café au lait spot. At age 15 months the patient developed a limp. Roentgenographic evaluation showed widespread, bilateral changes of fibrous dysplasia, most severe in the right pelvis, femur, and tibia. At the present time he has no evidence of precocious puberty or other endocrinopathies. Despite the absence of endocrine abnormalities, these findings are consistent with a diagnosis of McCune-Albright syndrome with extensive bony and cutaneous lesions.

Cerebral blood flow and red cell delivery in normal subjects and in multiple sclerosis.

Regional cerebral blood flow (rCBF) was determined in 77 normal females and 53 normal males of different ages and in 26 men and 45 women with multiple sclerosis by the inhalation of radioactive Xe133 method. In the normal subjects the CBF was relatively high in the teens and fell, at first rapidly and then slowly in both sexes with age. During adult life the flow in females was significantly higher than in males. The delivery of packed red cells (RCD) was determined by multiplying the CBF by the percentage concentration of red cells (HCT). The RCD for both sexes was nearly the same. In the patients with multiple sclerosis there occurred a progressive generalized decrease in CBF and in RCD with age which was significantly greater than observed in normal subjects. The rate of decrease in CBF and RCD correlated directly with the rate of progress of the disease.

The usefulness of electroencephalography in curarized newborns.

Curare and other muscle relaxants are being used with increasing frequency in the management of infants who are mechanically ventilated for severe respiratory distress. Neurological evaluation of these paralyzed newborns is difficult and neonatal convulsions may go unrecognized. The present study includes 5 curarized infants in whom neurological impairment was suspected because of complicated pregnancies and perinatal asphyxia. All showed very abnormal EEGs with ongoing paroxysmal activity in the absence of clinical seizures or a severely depressed record. In two of these infants an improvement in the values of the blood-gases coincided with the administration of diazepam. In curarized infants the electroencephalogram is of importance in the evaluation of the background activity, the detection of convulsive brain activity, and in the management of anticonvulsive therapy.