Parent ratings on the MEMRY questionnaire predict children's academic performance.

Given the high rates of learning challenges in children with medical conditions, efficient and reliable screening methods are crucial. This study examined whether parent report of daily learning and memory predicts academic achievement in youth. Parents of 213 youth (aged 6-18) with varied medical diagnoses completed the Multidimensional Everyday Memory Ratings for Youth (MEMRY), and youth completed subtests from the Wechsler Individual Achievement Test-Third Edition (WIAT-III) as part of a comprehensive assessment. All scales of the MEMRY (Learning, Daily Memory, Executive/Working Memory) correlated significantly with WIAT-III Spelling, Word Reading, and Numerical Operations, while only the MEMRY Learning subscale correlated significantly with WIAT-III Pseudoword Decoding. Regression analyses indicated that MEMRY Learning significantly predicted WIAT-III Word Reading and Spelling, while both the MEMRY Learning and MEMRY Daily Memory scales significantly predicted WIAT-III Numerical Operations. When Full Scale IQ was entered into the models first, the MEMRY Learning subscale accounted for an additional 4% of variance in WIAT-III Word Reading and 7% of variance in WIAT-III Spelling, but did not account for additional variance in WIAT-III Numerical Operations or Pseudoword Decoding. Analyses in a subset of children with broadly normal intellectual functioning demonstrated very similar results, with even higher variance in academic testing accounted for by the MEMRY. In sum, the MEMRY questionnaire may serve as an efficient screen to identify children at risk for reading, spelling, and math deficits.

Neuropsychological profiles of two patients with differing SCN8A-pathogenic variants.

The impact of gene-related early infancy onset epilepsies in cognitive development can be potentially devastating. Here we report two cases of SCN8A-related epilepsy that highlight the neuropsychological heterogeneity seen with differing de-novo pathogenic variants. Case 1 is a 6-year-old right-handed girl who presented with SCN8A-developmental and epileptic encephalopathy (SCN8A-DEE) and a missense pathogenic variant (c.802A > C), not previously documented in the literature. Her history includes speech and motor delay, with focal motor seizures starting at 4-months. Early EEG showed bilateral centroparietal epileptiform discharges. She shows motor and language delays and prominent motor tics. Testing documented Intellectual Disability (ID) (Mild) with widespread neuropsychological deficits (i.e., academics, attention/executive functions, memory, visual-spatial skills, fine motor, language). Case 2 is an 8-year-old right-handed girl who presented with SCN8A-related epilepsy with c.5630A > G pathogenic variant with seizure onset at 5-months. Her initial EEG showed right occipital spikes. She shows low average intellect and average academics, but evaluation documented attention deficits, fine motor delays, and behavioral issues in addition to tics; she was diagnosed with Attention-Deficit/Hyperactivity Disorder, Oppositional Defiant Disorder, Obsessive Compulsive Disorder, and Tourette's. These cases expand limited knowledge regarding neuropsychological functioning of children with SCN8A-related epilepsy with unique de-novo pathogenic variants. While SCN8A-DEE is clearly associated with ID, other pathogenic variants may show better preserved intellect, despite other neuropsychological and behavioral concerns.