The presence and degree of specialization between the anterior temporal lobes (ATLs) is a key issue in debates about the neural architecture of semantic memory. Here, we comprehensively assessed multiple aspects of semantic cognition in a large group of postsurgical temporal lobe epilepsy (TLE) patients with left versus right anterior temporal lobectomy (n = 40). Both subgroups showed deficits in expressive and receptive verbal semantic tasks, word and object recognition, naming and recognition of famous faces and perception of faces and emotions. Graded differences in performance between the left and right groups were secondary to the overall mild semantic impairment; primarily, left resected TLE patients showed weaker performance on tasks that required naming or accessing semantic information from a written word. Right resected TLE patients were relatively more impaired at recognizing famous faces as familiar, although this effect was observed less consistently. These findings unify previous partial, inconsistent results and also align directly with fMRI and transcranial magnetic stimulation results in neurologically intact participants. Taken together, these data support a model in which the 2 ATLs act as a coupled bilateral system for the representation of semantic knowledge, and in which graded hemispheric specializations emerge as a consequence of differential connectivity to lateralized speech production and face perception regions.

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SUMMARYObjective: To examine executive function, intelligence, visuospatial skills, language, memory, attention, reaction time, anxiety, depression, and emotional and behavioral traits most frequently associated with executive dysfunction in patients with juvenile myoclonic epilepsy (JME) compared with a sibling and a normal control group under video-electroencephalography (video-EEG) conditions. Methods: Twenty-two sibling pairs, one with JME, were compared with 44 controls matched for age, gender, and educational level. All participants were administered a comprehensive set of neuropsychological and questionnaire measures during and without video-EEG recording. Results: The JME group differed significantly from controls in measures of phonemic and semantic verbal fluency. They scored significantly higher on the dysexecutive self-rating questionnaire, being more likely to report traits associated with executive dysfunction than both siblings and controls. Patients with JME reported significantly low mood than both controls and their siblings. Unaffected siblings differed significantly from controls on psychomotor speed, phonemic verbal fluency and were considered to exhibit traits associated with executive dysfunction by others. Qualitative inspection of data suggested a convincing trend for patients with JME and their siblings to perform worse than controls on most measures. Significance: This study supports the existence of a distinct neuropsychological profile among patients with JME and their siblings, which is likely to be genetically determined. The similarity of neuropsychological profiles between JME patients and their siblings is independent of antiepileptic drug effects or subclinical EEG activity. The significant differences between the sibling and controls suggests that there is a neurocognitive endophenotype for JME.

Educational initiatives and therapeutic interventions that aim to address stigma in people with epilepsy are recommended; however, these need to be culturally informed to ensure that they are valid and effective.

One critical feature of any well-engineered system is its resilience to perturbation and minor damage. The purpose of the current study was to investigate how resilience is achieved in higher cognitive systems, which we explored through the domain of semantic cognition. Convergent evidence implicates the bilateral anterior temporal lobes (ATLs) as a conceptual knowledge hub. While bilateral damage to this region produces profound semantic impairment, unilateral atrophy/resection or transient perturbation has a limited effect. Two neural mechanisms might underpin this resilience to unilateral ATL damage: 1) the undamaged ATL upregulates its activation in order to compensate; and/or 2) prefrontal regions involved in control of semantic retrieval upregulate to compensate for the impoverished semantic representations that follow from ATL damage. To test these possibilities, 34 postsurgical temporal lobe epilepsy patients and 20 age-matched controls were scanned whilst completing semantic tasks. Pictorial tasks, which produced bilateral frontal and temporal activation, showed few activation differences between patients and control participants. Written word tasks, however, produced a left-lateralized activation pattern and greater differences between the groups. Patients with right ATL resection increased activation in left inferior frontal gyrus (IFG). Patients with left ATL resection upregulated both the right ATL and right IFG. Consistent with recent computational models, these results indicate that 1) written word semantic processing in patients with ATL resection is supported by upregulation of semantic knowledge and control regions, principally in the undamaged hemisphere, and 2) pictorial semantic processing is less affected, presumably because it draws on a more bilateral network.