Behavioral phenotypes of pediatric temporal lobe epilepsy.

OBJECTIVE: A broad spectrum of emotional-behavioral problems have been reported in pediatric temporal lobe epilepsy (TLE), but with considerable variability in their presence and nature of expression, which hampers precise identification and treatment. The present study aimed to empirically identify latent patterns or behavioral phenotypes and their correlates. METHODS: Data included parental ratings of emotional-behavioral status on the Behavior Assessment System for Children, 2nd Edition (BASC-2) of 81 children (mean age = 11.79, standard deviation [SD] = 3.93) with TLE. The nine clinical subscales were subjected to unsupervised machine learning to identify behavioral subgroups. To explore concurrent validity and the underlying composition of the identified clusters, we examined demographic factors, seizure characteristics, psychosocial factors, neuropsychological performance, psychiatric status, and health-related quality of life (HRQoL). RESULTS: Three behavioral phenotypes were identified, which included no behavioral concerns (Cluster 1, 43% of sample), externalizing problems (Cluster 2, 41% of sample), and internalizing problems (Cluster 3, 16% of sample). Behavioral phenotypes were characterized by important differences across clinical seizure variables, psychosocial/familial factors, everyday executive functioning, and HRQoL. Cluster 2 was associated with younger child age, lower maternal education, and higher rate of single-parent households. Cluster 3 was associated with older age at epilepsy onset and higher rates of hippocampal sclerosis and parental psychiatric history. Both Cluster 2 and 3 demonstrated elevated family stress. Concurrent validity was demonstrated through the association of psychiatric (i.e., rate of Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) disorders and psychotropic medication) and parent-rated HRQoL variables. SIGNIFICANCE: Youth with TLE present with three distinct behavioral phenotypes that correspond with important clinical and sociodemographic markers. The current findings demonstrate the variability of behavioral presentations in youth with TLE and provide a preliminary framework for screening and targeting intervention to enhance support for youth with TLE and their families.

Neuropsychological Phenotypes in Pediatric Temporal Lobe Epilepsy.

OBJECTIVE: Adults with temporal lobe epilepsy (TLE) have been found to have a fairly characteristic pattern of neuropsychological performance, but there is considerably less research and more variability in findings with children. Because the cognitive domains included in most studies with children have been limited, the current study attempted to better characterize the cognitive phenotype of children with TLE using a broader neuropsychological battery. METHODS: The study included 59 children with TLE (59% male) age 7 to 16 (M = 12.67; SD = 3.12) who underwent comprehensive neuropsychological evaluation. Patient results were grouped into cognitive domains (reasoning, language, visuoperceptual, verbal memory, executive function, and motor function) based upon their test performance. These factor scores were subjected to Ward's hierarchical clustering method with squared Euclidean distance. RESULTS: Cluster analysis revealed three distinct cognitive profiles: (1) normal functioning (20% of sample); (2) delayed verbal memory and motor weaknesses (61% of the sample); and (3) global impairment (19% of the sample). Cluster 3 had longer epilepsy duration and a higher incidence of hippocampal sclerosis (HS) compared to Cluster 1 (p < .05). There were no significant differences among the three cluster groups on demographic characteristics or remaining clinical characteristics. CONCLUSIONS: Children with TLE present with distinct cognitive phenotypes ranging from average performance to global impairment. Results partially support previous hypotheses highlighting the cumulative neurobiological burden on the developing brain in the context of chronic epilepsy and provide a preliminary framework for the cognitive domains most vulnerable to the TLE disease process.

The future is now: pediatric neuropsychological presurgical epilepsy evaluation in the age of COVID-19.

The objective of this brief report is to review an assessment paradigm for conducting virtual neuropsychological pre-surgical evaluations in the context of the COVID-19 pandemic. A multidisciplinary epilepsy team at a Level 4 epilepsy center within a large children's academic medical center convened to discuss the challenges and possible solutions for Phase II evaluations for pediatric patients with pharmacoresistant epilepsy during the COVID-19 pandemic. The neuropsychologists explored evidence-based methods of virtual evaluation and developed a systematic decision-making process for youth requiring a Phase II evaluation. We propose models of assessment which prioritize teleneuropsychology when possible to reduce the risk of infection: (1) evaluation with directly administered tests through a completely virtual format; (2) virtual/in-person hybrid evaluation; and (3) clinical observation/interview in a virtual format supplemented by survey data. These models are illustrated by three cases. Using virtual assessment models, the team was able to meet the urgent patient care needs and collect useful data while minimizing the risk of virus spread. The paradigms presented may be useful examples for other multidisciplinary surgical teams interested in incorporating teleneuropsychology into their practices.