Early infantile epileptic encephalopathy: a case associated with hemimegalencephaly.

The authors report a case of early infantile epileptic encephalopathy (EIEE) associated with hemimegalencephaly. The etiological factors in previously published cases of EIEE are reviewed and attention is focused on the high proportion of cases associated with neuronal migration disorders. We suggest that idiopathic cases of EIEE would be due to such disorders.

Appropriately treating insomnia with triazolam.

Insomnia is a problem that extends beyond the nighttime. People who experience sleep disturbances complain that they stay awake for a long time before they fall asleep. They may wake up several times during the night and cannot return to sleep and/or they wake up early in the morning. As a result, they feel sleepy during the day and are less alert. Various forms of insomnia are described that require--as much as possible--an individualized treatment approach. Besides sleeping hygiene, benzodiazepines certainly occupy a place in the treatment of insomnia. Triazolam, a triazolobenzodiazepine, closely approaches the characteristics of an ideal hypnotic: pharmacological activity at the level of the receptors, moderate absorption, short-acting, and rapid elimination. It is effective and safe if prescribed correctly and at the appropriate dosage.

Time course of serum amyloid A response in myocardial infarction.

Plasma concentrations of serum amyloid A (SAA), high density lipoprotein (HDL) cholesterol, non-HDL cholesterol, and apolipoproteins (Apo) A-I and B were measured daily for 6 days in 10 patients following myocardial infarction (MI) and in 10 secular controls admitted to a coronary care unit. SAA concentrations peaked 3 days following MI (mean 47 mg/dl) and correlated with creatine kinase (CK) (r = 0.67, P less than 0.001). Non-HDL cholesterol and Apo B fell 15 and 18%, respectively, reached nadirs 3-4 days after MI and were inversely related to CK concentrations (P less than 0.01 for both). HDL cholesterol levels, in contrast, increased 15% and were significantly higher than baseline by day 3 when SAA concentrations were maximum. HDL cholesterol subsequently fell in parallel with SAA and had returned to baseline by day 6. Apo A-I declined throughout the 6 days of observation and was 13% lower than initial values on day 6 (P less than 0.05). The Apo A-I reduction was inversely related to both CK and SAA concentrations. There were no significant changes in any of the analytes in control subjects. We conclude that Apo A-I and possibly Apo B containing lipoproteins are negative acute phase reactants. HDL cholesterol is transiently elevated after MI despite decreasing Apo A-I levels and this may relate to incorporation of SAA into HDL particles.

Labia minora reduction in an iron-lung disabled woman.

Enlarged labia minora can be bothersome for functional, aesthetic, and social reasons. Reassurance that variation in size is normal is often all that is needed. However, there are women with legitimate complaints that make surgical reduction acceptable and necessary. Surgical intervention should not be ignored because of other debilitating medical problems. One such unusual case is presented here.

Cerebro-facio-thoracic dysplasia: report of three cases.

Three children, two girls and one boy, are described as suffering from a new syndrome, 'cerebro-facio-thoracic dysplasia'. The characteristic findings are mental retardation, characteristic facies, narrow forehead, bushy eyebrows with synophrys, hypertelorism, broad nose, wide philtrum, triangular-shaped mouth, short neck, marked maxillary hypoplasia, a low hairline (especially posteriorly in the midline on the neck), brachycephaly, calcified clinoid ligements, and multiple bony abnormalities in the upper thoracic vertebrae and sometimes in the cervical region, together with a variety of deformities of the upper ribs. It seems possible that the condition is hereditary and that it may be inherited as a result of an autosomal recessive trait.