The factor structure of the CVLT-C in pediatric epilepsy.

The current study used archival data to evaluate the fit of six latent variable models, originally generated by Donders (1999), for the California Verbal Learning Test-Children's Version (CVLT-C; Delis, Kramer, Kaplan, & Ober, 1994) in a large (N = 289) sample of pediatric epilepsy cases presenting at three tertiary treatment centers. Using confirmatory factor analysis, we found that a model including factors of Attention Span, Learning Efficiency, Free Delayed Recall, Cued Delayed Recall, and Inaccurate Recall demonstrated the best relative fit for our data. These findings are consistent with those reported by Donders (1999) in his reanalysis of the CVLT-C standardization sample data, supporting the validity of this factorial model in pediatric epilepsy populations.

Neuropsychological assessment of patients with late onset GM2 gangliosidosis.

OBJECTIVE: To characterize cognitive status in a sample of individuals with late-onset GM2 gangliosidosis (commonly referred to as late-onset Tay-Sachs disease). METHODS: Seventeen subjects (13 men, 4 women) diagnosed with GM2 gangliosidosis were evaluated. Subjects ranged in age from 18 to 56 years and were in various stages of disease progression. Subjects underwent comprehensive neuropsychological assessment. Impairment was defined as performance more than 1.6 SD below the normative mean. RESULTS: Group mean performance was within the denoted normal range on all measures except on a task assessing visual sequencing and set shifting. Approximately one-half of the sample scored in the impaired range on measures of processing speed, visual sequencing, and set shifting. One-third of the sample also scored in the impaired range on measures of delayed verbal recall. Impairment tended to be restricted to a subset of the sample, as 5 of the 14 subjects able to undergo formal testing accounted for 70% of the total number of impaired scores. If the three subjects unable to participate in formal testing are also considered impaired, 47% of the current sample exhibited significant cognitive impairment in at least one cognitive domain. CONCLUSION: In late-onset GM2 gangliosidosis, there is a risk of impairment in executive functioning and memory as well as cerebellar dysfunction. Dementia was not present in any subjects in the current sample.