Cortico-muscular coherence and brain networks in familial adult myoclonic epilepsy and progressive myoclonic epilepsy.

OBJECTIVE: Familial Adult Myoclonic Epilepsy (FAME) presents with action-activated myoclonus, often associated with epilepsy, sharing various features with Progressive Myoclonic Epilepsy (PMEs), but with slower course and limited motor disability. We aimed our study to identify measures suitable to explain the different severity of FAME2 compared to EPM1, the most common PME, and to detect the signature of the distinctive brain networks. METHODS: We analyzed the EEG-EMG coherence (CMC) during segmental motor activity and indexes of connectivity in the two patient groups, and in healthy subjects (HS). We also investigated the regional and global properties of the network. RESULTS: In FAME2, differently from EPM1, we found a well-localized distribution of beta-CMC and increased betweenness-centrality (BC) on the sensorimotor region contralateral to the activated hand. In both patient groups, compared to HS, there was a decline in the network connectivity indexes in the beta and gamma band, which was more obvious in FAME2. CONCLUSIONS: In FAME2, better localized CMC and increased BC in comparison with EPM1 patients could counteract the severity and the spreading of the myoclonus. Decreased indexes of cortical integration were more severe in FAME2. SIGNIFICANCE: Our measures correlated with different motor disabilities and identified distinctive brain network impairments.

FAME 2: Reshaping the approach to patients with stable coronary artery disease.

Contrary to its central role in patients with acute coronary syndromes (ACS), percutaneous coronary intervention (PCI) in stable ischemic heart disease (SIHD) remains largely restricted to patients in whom medical treatment fails to control symptoms, or those with a large area of myocardium at risk and/or high risk findings on non-invasive testing.(1,2) These recommendations are based on a number of studies - the largest of which is COURAGE - that failed to show any reduction in mortality or myocardial infarction (MI) with PCI compared to optimal medical therapy (OMT) in this group of patients.(3) A possible limitation in these studies was relying on visual assessment of angiographic stenoses (which is now well-known to be imprecise) to determine lesions responsible for myocardial ischemia. Non-invasive stress testing - including imaging - may also be inaccurate in patients with multivessel coronary artery disease.(4,5) These limitations have inadvertently led to the inclusion of patients with non-ischemic lesions in these studies, which may have diluted any potential benefit with PCI. Given the superiority of fractional flow reserve (FFR) in identifying ischemic lesions compared to angiography, Fractional flow reserve versus Angiography for Multivessel Evaluation 2 (FAME 2) investigators hypothesized that when guided by FFR, PCI plus medical therapy would be superior to medical therapy alone in patients with SIHD.