Vessel wall enhancement in the diagnosis and management of primary angiitis of the central nervous system in children.

We describe two cases of primary angiitis of the central nervous system in children (cPACNS) diagnosed by vessel wall contrast enhancement on magnetic resonance imaging (MRI). Both patients developed acute cerebral infarction after fever and malaise. In patient 1, a 7-month-old boy, MRI revealed extensive cerebral infarction in the right middle cerebral artery (MCA) area and stenosis at the M1 portion of the right MCA. Oral glucocorticoid therapy was initiated. Vessel wall enhancement was ameliorated 3months after onset, and stenosis was mostly restored. Patient 2, a 5-year-old boy, suffered from cerebral infarction in the left MCA area, and stenosis was identified in the left internal carotid artery, left MCA, and left posterior cerebral artery. Although vessel wall enhancement was reduced after glucocorticoid therapy, vessel wall enhancement of left MCA re-emerged, accompanied by increased erythrocyte sedimentation rate (ESR) and, decreased cerebral blood flow (CBF) in the affected hemisphere. Intravenous methylprednisolone therapy followed by oral glucocorticoid and mycophenolate mofetil resulted in resolution of these findings. Vessel wall enhancement is a promising finding in the diagnosis of cPACNS. Disease flares occur rarely in medium-to-large vessel cPACNS during dose tapering. Vessel wall enhancement, ESR, and CBF may be useful for the assessment of the activity of angiitis.

Early predictors of status epilepticus-associated mortality and morbidity in children.

BACKGROUND: Early predictors of status epilepticus (SE)-associated mortality and morbidity have not been systematically studied in children, considerably impeding the identification of patients at risk. OBJECTIVES: To determine reliable early predictors of SE-associated mortality and morbidity and identify the etiology of SE-associated sequelae in Japanese children. METHODS: We conducted a prospective multicenter study of clinical findings and initial laboratory data acquired at SE onset, and assessed outcomes at the last follow-up examination. In-hospital death during the acute period and neurological sequelae were classified as poor outcomes. RESULTS: Of the 201 children who experienced their first SE episode, 16 exhibited poor outcome that was most commonly associated with acute encephalopathy. Univariate analysis revealed that the following were associated with poor outcomes: young age (⩽24 months); seizure duration >90 min; seizure intractability (failure of the second anticonvulsive drug); biphasic seizures; abnormal blood glucose levels (<61 or >250 mg/dL); serum aspartate aminotransferase (AST) ⩾56 U/L; and C-reactive protein (CRP) levels >2.00 mg/dL. Multivariate analysis revealed that young age, seizure intractability, abnormal blood glucose levels, and elevated AST and CRP levels were statistically significant. CONCLUSIONS: Young age and seizure intractability were highly predictive of poor outcomes in pediatric SE. Moreover, abnormal blood glucose levels and elevated AST and CRP levels were predictors that might be closely associated with the etiology, especially acute encephalopathy and severe bacterial infection (sepsis and meningitis) in Japanese children.

Fat areas on the extremities in normal weight and overweight children and adolescents: Comparison between age-related and weight-related changes in adiposity.

The purpose of the study was to compare changes in fat distribution in association with obesity and puberty in adolescent boys and girls. Fat areas at the ulnar, triceps, thigh, and calf regions were measured in normal weight prepubertal children (P) and adolescents (N), and overweight adolescents (O). There were significant differences in fat areas at the four sites between N and O of both sexes, especially in the proximal extremities (triceps, thigh) in boys. On the other hand, fat areas on the extremities of N boys and girls were not significantly different from those of P children except for thigh fat area in girls. It is suggested that pubertal maturation in girls, but not in boys, is associated with increased adiposity on the legs, particularly in the proximal regions, and that there are sex differences between changes in fat distribution associated with puberty and obesity. © 1996 Wiley-Liss, Inc.

Usefulness of a stature-based standard of skinfold thickness, especially for short children.

Skinfold thicknessess (SFT) were measured at ulnar, triceps, subscapular and suprailiac sites in 730 boys and 724 girls (age 3-12 years) whose stature ranged from 100 to 150 cm and whose weight was within ±20% of the average. Means and standard deviation (SD) were calculated after logarithmic transformation of the original skinfold readings to demonstrate stature-based standards of SFT in Japanese children. The means of SFT exhibited nadirs (boys/ girls: ulnar 5.1/5.9 mm, triceps 7.9/9.5 mm, subscapular 4.9/6.1 mm, suprailiac 4.5/6.2 mm) in subjects 110-115 cm tall except for ulnar SFT in girls. SFT values increased as children increased in stature. Standard deviations of SFT at the four sites in short children (staturte < mean -1 SD) were estimated using the stature-based standard as well as an age-based standard. Susms of the SDs assessed by the age-based standard were significantly smaller than those assessed by the stature-based standard in boys (P < 0.05) and girls (P < 0.01) with short stature, suggesting that SFT in short children was falsely understimated by the age-based standard. Thus, the stature-based standard is beneficial for the assessment of SFT, especially in children whose stature is below the mean --1 SD. © 1995 Wiley-Liss, Inc.