Serial neuropsychological testing before and after hemispherectomy in a child with electrical status epilepticus in slow wave sleep.

Electrical status epilepticus in slow wave sleep (ESES) is typically characterized by neuropsychological deterioration to varying extents. While the syndrome is self-limiting for some, for others it has a swiftly declining course that requires aggressive neurosurgical intervention. Here, we present a patient with ESES secondary to a large left middle cerebral artery stroke in utero who experienced behavioral and neuropsychological deterioration that rapidly progressed. Collectively, her neuropsychological status, EEGs, MRIs, and nature of her ESES warranted a left hemispherectomy to prevent further decline. The patient was seen for four neuropsychological evaluations. Over time, neuropsychological test scores did not adequately capture her degree of impairment and change. Behavioral observations during evaluations and parental reports played a critical role in determining her initial decline and mild post-surgical improvement. For all practitioners, this report illustrates one extreme of ESES. It also illustrates, particularly for neurologists and neurosurgeons, the importance of behavioral observations relative to test performances.

A cross-sectional cohort study of post-concussive symptoms and their relationships with depressive symptoms in youth with and without concussion.

OBJECTIVES: To evaluate the frequency and severity of post-concussive symptoms in youth with a history of concussion relative to youth without concussion who had another medical diagnosis, as well as compare the correlations between post-concussive and depressive symptoms between groups. We hypothesized comparable symptom reporting and correlations in each group. METHODS: A total of 564 youth ages 8-18 years were assessed regarding post-concussive symptoms. A subset of youth (n = 360) were compared on correlations between post-concussive and depressive symptoms. Non-parametric statistics were used for most analyses. RESULTS: Youth with concussion reported a comparable number of post-concussive and depressive symptoms as youth with another medical condition without concussion. However, those with concussion reported greater post-concussive symptom severity (but small effect sizes). Relationships between post-concussive and depressive symptoms were comparable for both groups, but for those who sustained a concussion, the correlation was significantly stronger for females than males. CONCLUSIONS: This study further demonstrates that post-concussive symptoms are nonspecific and provide little functional utility. Post-concussive and depressive symptoms are strongly correlated, particularly in females with concussion. Psychiatric comorbidities and other medical diagnoses should be assessed pre-injury because both affect interpretation of post-concussive symptom reports.

Confirmatory factor analysis of the Behavior Rating Inventory of Executive Functioning (BRIEF) in children and adolescents with ADHD.

The Behavior Rating Inventory of Executive Functioning (BRIEF) is a parent report measure designed to assess executive skills in everyday life. The present study employed a confirmatory factor analysis (CFA) to evaluate three alternative models of the factor structure of the BRIEF. Given the executive functioning difficulties that commonly co-occur with attention-deficit/hyperactivity disorder (ADHD), the participants included 181 children and adolescents with a diagnosis of ADHD. The results indicated that an oblique two-factor model, in which the Monitor subscale loaded on both factors (i.e., Behavioral Regulation, Metacognition) and measurement errors for the Monitor and Inhibit subscales were allowed to correlate, provided an acceptable goodness-of-fit to the data. This two-factor model is consistent with previous research indicating that the Monitor subscale reflects two dimensions (i.e., monitoring of task-related activities and monitoring of personal behavioral activities) and thus loads on multiple factors. These findings support the clinical relevance of the BRIEF in children with ADHD, as well as the multidimensional nature of executive functioning.

Neurobehavioral abnormalities in first-degree relatives of individuals with autism.

CONTEXT: Studying sensorimotor and neurocognitive impairments in unaffected family members of individuals with autism may help identify familial pathophysiological mechanisms associated with the disorder. OBJECTIVE: To determine whether atypical sensorimotor or neurocognitive characteristics associated with autism are present in first-degree relatives of individuals with autism. DESIGN: Case-control comparison of neurobehavioral functions. SETTING: University medical center. PARTICIPANTS: Fifty-seven first-degree relatives of individuals with autism and 40 age-, sex-, and IQ-matched healthy control participants (aged 8-54 years). MAIN OUTCOME MEASURES: Oculomotor tests of sensorimotor responses (saccades and smooth pursuit); procedural learning and response inhibition; neuropsychological tests of motor, memory, and executive functions; and psychological measures of social behavior, communication skills, and obsessive-compulsive behaviors. RESULTS: On eye movement testing, family members demonstrated saccadic hypometria, reduced steady-state pursuit gain, and a higher rate of voluntary response inhibition errors relative to controls. They also showed lateralized deficits in procedural learning and open-loop pursuit gain (initial 100 milliseconds of pursuit) and increased variability in the accuracy of large-amplitude saccades that were confined to rightward movements. In neuropsychological studies, only executive functions were impaired relative to those of controls. Family members reported more communication abnormalities and obsessive-compulsive behaviors than controls. Deficits across oculomotor, neuropsychological, and psychological domains were relatively independent from one another. CONCLUSIONS: Family members of individuals with autism demonstrate oculomotor abnormalities implicating pontocerebellar and frontostriatal circuits and left-lateralized alterations of frontotemporal circuitry and striatum. The left-lateralized alterations have not been identified in other neuropsychiatric disorders and are of interest given atypical brain lateralization and language development associated with the disorder. Similar oculomotor deficits have been reported in individuals with autism, suggesting that they may be familial and useful for studies of neurophysiological and genetic mechanisms in autism.

Developmental outcome after epilepsy surgery in infancy.

OBJECTIVES: Our goals were to determine the effect of epilepsy surgery in infants (<3 years of age) on development and describe factors associated with postoperative developmental outcome. METHODS: We identified 50 infants among 251 consecutive pediatric patients (<18 years old) undergoing epilepsy surgery. Charts were reviewed for clinical data and neurodevelopmental testing with the Bayley Scales of Infant Development. A developmental quotient was calculated to compare scores of children at different ages. RESULTS: Complete data were available on 24 of 50 infants. Surgeries included 14 hemispherectomies and 10 focal resections. Seventeen patients became seizure free; 5 patients had >90% seizure reduction, 1 had >50% seizure reduction, and 1 had no change. The developmental quotient indicated modest postoperative improvement of mental age. The preoperative and postoperative development quotients correlated well. Younger infants had a higher increase in developmental quotient after surgery. Patients with epileptic spasms were younger and had a lower developmental quotient at presentation, but increase in developmental quotient was higher in this subgroup. CONCLUSIONS: After surgery, seizure frequency and developmental quotient improved. Developmental status before surgery predicted developmental function after surgery. Patients who were operated on at younger age and with epileptic spasms showed the largest increase in developmental quotient after surgery.