Investigators at UCL Institute of Neurology, Queen Square, London, UK, used functional magnetic resonance imaging to study the effect of cognitive effort during a working memory task as a trigger of myoclonic jerks in 15 unaffected siblings (10 female; age range 18-65 years, median 40 yrs) of 11 patients with juvenile myoclonic epilepsy (6 female; age range 22-54 yrs, median 35). fMRI activations were compared with 20 age- and gender-matched healthy control subjects.
Unaffected siblings showed abnormal primary motor cortex and supplementary motor area co-activation with increasing cognitive load, as well as increased task-related functional connectivity between motor and prefrontal cognitive networks, with a similar pattern to that in patients with JME (P < 0.001). This finding in unaffected siblings suggests a mechanism for impairment of frontal lobe functions in both patients and siblings, independent of effects of medication or seizure, an endophenotype of JME. 
COMMENTARY. The abnormal frontal lobe function demonstrated by fMRI in adults with JME and their siblings is also demonstrated in children.
Neurodevelopment in new-onset juvenile myoclonic epilepsy. Investigators at Irvine University, CA, studied the maturation of cognitive and brain development in 19 children with new-onset JME in the first 2 years after diagnosis and 57 healthy controls. Abnormal patterns of brain development affecting frontoparietotemporal regions, as assessed by MRI, were evident in children with JME and included attenuation of age-related decline in cortical volume, thickness, and surface area. Children with JME have abnormal structural brain development and impaired cognitive development early in the course of the epilepsy .