Faculty Bibliography

Tuberous sclerosis complex is a multisystem disorder in which neurologic problems cause the greatest disability. High rates of mental retardation and autism spectrum disorders are associated with the diagnosis. Early-onset seizures and increased tuber burden are risk factors for cognitive impairment. Early-onset seizures, particularly infantile spasms, are risk factors for autism. Tubers within the temporal lobe and cerebellum are often mentioned as risk factors for autism, although the findings are inconsistent. Seizure control is important for developmental outcome and quality of life. Early behavioral assessment and therapeutic intervention, as well as seizure control, are the most effective means of promoting neurodevelopmental outcome

The goals of this work were to: (1) determine the prevalence of clinically significant obsessive-compulsive (OC) symptoms in patients with temporal lobe epilepsy (TLE), (2) characterize the differences in self-reported OC symptoms in patients with TLE and a normative control group, and (3) compare the severity of OC symptoms in right and left hemisphere TLE patients. Patients with TLE (n=30) were administered the Obsessive-Compulsive Inventory (OCI). As a group, patients with TLE had a higher prevalence of OC symptoms than the nonpatient normative sample. In addition, TLE patients exhibited elevated scores on all but 3 of the 16 OCI scales and subscales. There were no reliable differences in OC symptoms in patients with right versus left hemisphere seizure foci, although the right hemisphere patients tended to score higher on both scales of the OCI

Findings from previous research have argued for the dissociation of two visual-perceptual tasks traditionally thought to be mediated by the nondominant hemisphere (i.e. perceptual closure and facial discrimination). This, primarily methodological, study examined the extent to which the facial closure measure (Mooney closure faces test) involves 'closure' and/or facial discrimination. A factor analysis of six visual perceptual measures, carried out separately for left brain damaged (LBDs, n=33) and right brain damaged (RBDs, n=30) patients, resulted in two relatively independent factors (i.e. a closure factor and a facial discrimination factor), with the Mooney closure faces test loading on both. The mixed factorial structure did not aid the facial closure measure's sensitivity to right-sided brain disease. Moreover, age and education intercorrelated differently with the two factors. Results argue for the use of more discrete visual-perceptual measures when examining perceptual functioning and/or right hemisphere integrity, and imply the existence of at least two discrete cortical level visual-perceptual neural systems

Animal word list generation (ANWLG) was administered to 47 first-episode schizophrenia (FES) participants and 31 controls. Fifty-nine left temporal lobe epilepsy (LTLE) participants were included as a comparison group with known temporal lobe damage and expected semantic deficits. Semantic knowledge was assessed with the Association Index (AI), a measure of the semantic relatedness of all consecutive ANWLG responses. Neuropsychological tests of language and executive functioning were also administered. Results showed that both FES and LTLE groups generated fewer ANWLG responses than controls, but only the LTLE participants obtained a lower AI relative to controls. FES participants did not differ from controls on the AI. FES and LTLE groups produced fewer semantic subcategories (clusters), however, only the LTLE group produced fewer words per subcategory compared to controls (cluster size). FES participants produced a higher rate of perseverative responses compared to the other groups. Finally, correlation analyses showed that for FES participants both executive and language tests significantly correlated with ANWLG total responses, while the correlation between ANWLG and only 1 language test was significant for LTLE participants. Taken together, the results suggest that reduced ANWLG output in FES participants may be best conceptualized as a deficit in the executive component of word list generation (i.e., semantic search/access, response monitoring) or global cognitive impairment.

OBJECTIVE: To prospectively examine recovery of cognitive function within one month following subconcussive sports related head trauma. DESIGN: A prospective study of New York State licensed professional boxers who underwent testing of cognitive functioning before and after (within days, one week, and one month) a professional bout. SETTING: Male professional athletes recruited from the New York State Athletic Commission and local boxing gyms. PARTICIPANTS: Twenty-six licensed professional boxers were enrolled in the protocol. Data is presented on the 18 participants who completed testing on at least three of the four time points. INTERVENTIONS: Serial neuropsychological assessment before and after the athletes engaged in competition. MAIN OUTCOME MEASURES: Neuropsychological measures of cognitive functioning, including new learning and memory, information processing speed, and mental flexibility. RESULTS: A series of repeated measures MANOVAS revealed significant within subject differences across testing on complex information processing and verbal fluency. Post hoc analyses indicated significant differences between time 1 (baseline) and time 4 (one month post), with scores one month following the bout indicating significantly improved performance. Memory scores did not change significantly across testing; however, prior boxing exposure measured by total number of professional bouts was associated with poorer memory performance. CONCLUSIONS: Cognitive testing one month following participation in a professional boxing bout yielded scores suggestive of recovery to a level above the baseline. We conclude that baseline assessment taken during periods of intense training are likely confounded by other pre-bout conditions (i.e., sparring, rapid weight loss, pre-bout anxiety) and do not represent true baseline abilities. Instability of performance associated with mild head injury may complicate the interpretation of post-injury assessments. Practice effects may also confound the interpretation of serial assessments, leading to underestimation of the effects of sports related head trauma. Poorer cognitive performance was evident during the presumed recovery period in boxers with greater exposure to the sport (>12 professional bouts). This finding is consistent with reports of a cumulative effect of repetitive head trauma and the subsequent development of chronic traumatic brain injury. These data have implications for assessing recovery of function following head injury in players of other contact sports as well as determination of return-to-play following an injury.