André B. Rietman

Epilepsy in children with tuberous sclerosis complex: Chance of remission and response to antiepileptic drugs.

To describe treatment and outcome of epilepsy in children with tuberous sclerosis complex (TSC).

Treatment of intractable epilepsy in tuberous sclerosis complex with everolimus is not yet evidence-based.

Dr Liu and colleagues propose an interesting hypothesis in response to our finding that pepper consumption is associated with a reduced risk of Parkinson disease. Although we had examined this association because tobacco family (Solanaceae) edibles contain nicotine, which appears neuroprotective in animal models, we noted that other shared constituents of tobacco and peppers might also be relevant. Among the candidates we mentioned were anatabine and capsaicin. Like nicotine, they readily cross the blood–brain barrier and then presumably reach dopaminergic neurons in the substantia nigra. A thorough examination will indeed include other possible agents. These include tobacco mosaic virus (TMV) as these authors suggest, although our data do not fully support it. Edible cucumbers and other Cucurbitaceae for which we have data (cantaloupe, zucchini, and yellow winter squash) are susceptible to TMV, but their combined consumption is not associated with Parkinson disease (relative risk 5 1.00, 95% confidence interval 5 0.72–1.38 per time per day, adjusted for Solanaceae, other vegetables, age, sex, race/ethnicity, tobacco use, and caffeine, not shown). In addition, the smoking–TMV antibody association recently observed by Liu et al was present in African Americans but not Caucasians, and overall, 30% of the smokers but none of the nonsmokers worked with tobacco products. Even in the absence of confounding by occupational TMV exposure, it is not yet clear that these intriguing results apply to our largely Caucasian population (or pepper eaters compared to noneaters). We look forward to continued research by this group and others to explore this and the many other possible alternative hypotheses for the inverse association between Parkinson disease and both tobacco use and consumption of peppers.

Emotional and behavioral problems in children and adolescents with neurofibromatosis type 1

To assess emotional and behavioral problems in children and adolescents with neurofibromatosis type 1,parents of 183 individuals aged 10.8 ± 3.1 years (range 6‐17) completed the Child Behavior Checklist (CBCL). Also, 173 teachers completed the Teachers Report Form (TRF), and 88 adolescents (children from 11 to 17 years) completed the Youth Self‐Report (YSR). According to parental ratings, 32% scored in the clinical range (above the 90th percentile). This percentage was much lower when rated by teachers or adolescents themselves. Scores from all informants on scales for Somatic complaints, Social problems, and Attention problems were significantly different from normative scores. Attentional problems were associated with lower verbal IQ, male gender, younger age, and ADHD‐symptoms. Disease‐related factors did not predict behavioral problems scores. Substantial emotional and behavioral problems were reported by parents, teachers, and to a lesser extent by adolescents with NF1 themselves. Possibly, a positive illusory bias affects the observation of behavioral problems by adolescents with NF1.

Development of emotional and behavioral problems in neurofibromatosis type 1 during young childhood

This retrospective longitudinal study in young children with neurofibromatosis type 1 (NF1) aimed to identify if, and how early problems in behavior, intelligence, and language development are associated with later behavioral problems. At the first assessment at preschool age, we evaluated language skills, intelligence, and emotional and behavioral problems as reported by parents. The second assessment at school‐age we evaluated intelligence, and emotional and behavioral problems as reported by parents and teachers. Association of baseline assessments with secondary assessment was evaluated using multivariable linear regression analysis. Of the 61 patients (25 males, 36 females; mean age 4;5 years [SD 1;1 years]) with NF1 who had a first assessment, 38 children (21 males, 17 females; mean age 7;11 years [SD 2;1 years]) had a second assessment after a mean period of 3;5 years. Longitudinal data on behavioral problems were collected for 23 of these children. Intelligence and language development were not associated with internalizing problems. Parent‐rated internalizing behavioral problems significantly increased with age in this subgroup. Baseline internalizing problems predicted later internalizing problems (adjusted R2 = 0.33, p = 0.003). The presence of these problems at pre‐school age may be predictive of internalizing problems at a later age.