Therapeutic approach to difficult-to-treat typical absences and related epilepsy syndromes.

Introduction: typical absences (TAs), are brief, generalized epileptic seizures of abrupt onset and termination clinically manifesting with impairment of awareness and associated with 3 Hz spike-wave discharges on EEG. TAs may occur in different idiopathic generalized epilepsies (IGE). Despite treatment with adequate anti-seizure medications (ASMs), TAs may persist in ~25% of subjects. This narrative review focuses on the therapeutic approach to difficult-to-treat TAs occurring in the setting of IGE.Areas covered: a literature search was conducted on the topic of treatment of TAs.Expert opinion: ethosuximide (ESX), valproic acid (VPA) and lamotrigine (LTG), alone or in combination, are considered the first-choice drugs. In women of childbearing potential, VPA should be avoided. Alternative therapies (benzodiazepines, levetiracetam, topiramate, or zonisamide) should be considered in subjects unresponsive to monotherapy after the exclusion of pseudo-drug resistance. Newer ASMs such as brivaracetam and perampanel seem to be promising options. Well-conducted clinical trials aimed to evaluate the efficacy of alternative monotherapy (beyond ESX, VPA or LTG) or combination of ASMs on difficult-to-treat TAs, are warranted.

Serial MRI findings in brain anoxia leading to Lance-Adams syndrome: a case report.

Although in literature almost 150 patients with Lance-Adams Syndrome (LAS) have been reported, neuroradiological evaluations were often performed in late stages and there is no serial study evaluating LAS from early stages. We herein report a serial neuroimaging study demonstrating early and transient involvement of cerebellum and thalami in a LAS patient. We may hypothesize that a transient cerebral hypoxia provoked a permanent synaptic rearrangements of the neuronal networks involved in the pathogenesis of post-hypoxic myoclonus in our patient.