Periodic limb movements during sleep in children with narcolepsy.

STUDY OBJECTIVES: In adults with narcolepsy, periodic limb movements of sleep (PLMS) occur more frequently than in control population, and presence of increased PLMS is associated with greater sleep disruption and shorter mean sleep latency. This study was performed to determine whether PLMS are common in children with narcolepsy, and whether the presence of PLMS is associated with greater sleep disruption. DESIGN: Demographic and polysomnographic information were collected from consecutive patients diagnosed with narcolepsy identified retrospectively by diagnosis-based search. Descriptive data were compiled, and sleep characteristics of children with and without PLMS were compared. SETTING: Sleep disorders center in a children's hospital. PATIENTS: 44 patients, 6-19 years old (mean 13 years, SD 3.57), were identified. Twenty-eight were African American. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Four patients had a PLMS index (PLMI) ≥ 5/h (considered abnormal in literature). Sixteen (36%) had "any PLMS" (PLMI > 0/h). The mean PLMI was 1.3/h (SD 2.5). Sleep was significantly more disrupted, and the mean sleep latency was shorter in patients with "any PLMS" as compared to those with no PLMS. There was no correlation between the PLMI and other diagnostic criteria for narcolepsy. "Any PLMS" were present equally in children of African American and Caucasian heritage, 35.7% vs. 37.5%. CONCLUSIONS: As in adults, children with PLMS and narcolepsy have more sleep disruption and shorter mean sleep latencies than those with narcolepsy but without PLMS. Our findings also suggest that the use of adult criteria for diagnosis of "significant" PLMS in children may not be sufficiently sensitive.

Pontine tegmental cap dysplasia with a 2q13 microdeletion involving the NPHP1 gene: insights into malformations of the mid-hindbrain.

The case of a young man with multiple brain and somatic anomalies that presented diagnostic difficulties, is discussed in this report. A majority of his features were suggestive of Joubert syndrome--although it was felt that he did not fully meet diagnostic criteria. The subsequent evaluations included a magnetic resonance image of the brain, that was found to be consistent with pontine tegmental cap dysplasia. Chromosomal microarray studies showed a 2q13 deletion. A gene associated with Joubert syndrome, NPHP1, is within this region. This case highlights several important aspects of the diagnosis and nosology of malformations of the mid-hind brain.

Functional MRI and Wada determination of language lateralization: a case of crossed dominance.

The Wada test has historically been the conventional procedure for determining language lateralization before neurosurgery. However, functional magnetic resonance imaging (fMRI) offers a less invasive alternative to the Wada procedure. Research indicates that the two techniques used together may provide comparable, and sometimes complementary, information that results in improved prediction of postsurgical language ability. We present a case in which use of fMRI in conjunction with Wada testing provided complementary information about language lateralization before neurosurgical resection of a mesial temporal subependymoma for seizure control in a patient with schizencephaly.

Anxiety in children with epilepsy.

Although the prevalence is unknown, affective disorders are more common in children with epilepsy than in healthy controls. The purpose of the present study was to examine the occurrence of anxiety in children and adolescents with epilepsy and to determine factors associated with elevation of these symptoms. Children and adolescents (n=101) between the ages of 6 and 16 years were given the Revised Children's Manifest Anxiety Scale (RCMAS). Mild to moderate symptoms of anxiety were reported by 23% of the patients. Based on regression analysis, factors significantly associated with increased anxiety included the presence of comorbid learning or behavioral difficulties, ethnicity, and polytherapy. Results suggest the need to monitor children and adolescents with epilepsy for affective symptoms in order to provide appropriate interventions.

Parental anxiety and quality of life in children with epilepsy.

Parental beliefs and attitudes concerning epilepsy may significantly impact adjustment and quality of life for both the child and family. The purpose of the present study was to examine the relationship between parental anxiety and quality of life in pediatric patients with ongoing epilepsy. Subjects were parents (n=200) of children between the ages of 6 and 16 years who had been diagnosed and treated for epilepsy for at least 1 year. Parents were given quality of life and anxiety questionnaires during the child's clinic visit. A stepwise regression analysis suggested that severity of comorbid conditions, parental anxiety, seizure control, and number of medications were significantly associated with quality of life for these children. Parents with increased anxiety whose children had poorly controlled seizures and a comorbid disability were found to have diminished quality of life.

Screening for autoantibodies in children with opsoclonus-myoclonus-ataxia.

Various paraneoplastic autoantibodies have been linked to discrete neurologic syndromes and tumors in adults, but little is known about their incidence in children. We report a cross-sectional study of known paraneoplastic antibodies in 59 children with opsoclonus-myoclonus-ataxia, 86% of whom were moderately or severely symptomatic, and 68% of whom had relapsed at the time of testing. This total number of patients includes 18 children with low-stage neuroblastoma (tested after tumor resection), six of whom had never been treated with immunosuppressants. All were seronegative for anti-Hu, anti-Ri, and anti-Yo, the three paraneoplastic antibodies most associated with opsoclonus-myoclonus or ataxia in adults. These data contrast with reports of anti-Hu-positive sera in children with high-stage tumors and suggest that anti-Hu, anti-Ri, and anti-Yo do not explain relapses in pediatric opsoclonus-myoclonus-ataxia. They underscore the need to search for unique autoantibodies, as well as cellular mechanisms of pediatric paraneoplastic disease.

The course of inattentive and hyperactive-impulsive symptoms in children with new onset seizures.

The purpose of the present study was to determine the prevalence of inattentive and hyperactive-impulsive symptoms in children with newly diagnosed epilepsy, explore the course of these symptoms over time, and examine factors associated with change in these symptoms. Parents of children (n=42) were administered the Attention Deficit Disorder Evaluation Scale-Home Version (ADDES-HV) at the time of diagnosis. The ADDES-HV was readministered after the child's seizures were controlled. Prior to initiation of anticonvulsant therapy, 31% of the children were rated as having clinically significant problems with inattention and 31% with hyperactivity-impulsivity. At follow-up, 27% had elevated symptoms of inattention and 24% had hyperactivity-impulsivity. Analysis of this change indicated that children with a normal MRI were more likely to have decreased hyperactive-impulsive behaviors following control of their seizures. Results suggest the need for assessment and monitoring of attention in children with epilepsy.

Symptom differences in children with absence seizures versus inattention.

Objective. Differentiation between the diagnoses of absence seizures and Attention Deficit Hyperactivity Disorder (ADHD), Predominantly Inattentive Type, is frequently confounded by similarities in symptom presentation. The purpose of the present study was to determine symptoms that would distinguish between the disorders.Methods. Prior to diagnosis, parents of children with absence seizures (n=17) or ADHD, Predominantly Inattentive Type (n=26), were administered the Attention Deficit Disorder Evaluation Scale-Home Version (ADDES-HV). A statistical model was developed based on age, gender, race, and items from the Inattentive Scale of the ADDES-HV.Results. Two items, "does not complete homework" and "does not remain on task," correctly classified 40 of 43 children. Children with absence seizures were rated by their parents as having a low rate of occurrence of these behaviors.Conclusion. Lack of sustained attention distinguished between the groups and was much more prevalent in children with ADHD, Predominantly Inattentive Type.

Factors Associated with Academic Achievement in Children with Controlled Epilepsy.

Children with epilepsy are at risk for academic underachievement. Multiple etiologies for this academic vulnerability have been suggested by past research including lower self-esteem, inattention, memory inefficiency, and lower socioeconomic status. The present study assessed 65 children (mean age = 10 years, 5 months) with well-controlled epilepsy on the four primary factors, as well as academic achievement and intelligence. A stepwise regression analysis was employed with academic achievement as the dependent variable and measures of self-esteem, attention, memory, and socioeconomic status as independent variables. When intelligence was controlled, attention was the only variable associated with achievement scores. Seizure variables including seizure type and duration of epilepsy were not associated with differences in academic performance. Findings support the importance of measuring attention skills in children with epilepsy and suggest that reduced auditory attention skills may be associated with decreased academic performance in these children.