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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.

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This report summarises service user feedback from a Crisis Resolution and Home Treatment team (CRHT). We reflect on the limited research in this area and the importance of incorporating service user feedback in service delivery.

It is increasingly becoming apparent that patients with Juvenile Myoclonic Epilepsy (JME) experience difficulties with various aspects of cognition. Studies that have attempted to find specific seizure-related factors contributing to and predicting cognitive dysfunction have provided conflicting and inconclusive results. In this brief paper we aimed to discuss the nature and context of sub-clinical EEG activity on cognition in JME using two individual cases. It seems that the potential 'mechanism' for cognitive impairments in JME is unlikely to be solely attributable to the effects of paroxysmal discharges and further research is required to clarify the longer term , cumulative effects of a range of factors that may help to explain such deficits in cognitive functioning.