Abstracts

Rationale: Acute post hypoxic myoclonus (Lance-Adams syndrome) is often super refractory despite the use of several recommended drugs including many anti epileptic drugs. Perampanel (PER) is a recent anti epileptic drug, it is the first orally active non competitive antagonist of the AMPA glutamate receptor.PER is approved for the treatment of focal epilepsy and more recently for primary generalized tonic clonic (PGTC) seizures. It has been also reported to have good efficacy on myoclonus associated with PGTC, myoclonus in Lafora disease and in Unvericht Lundborg disease and in a few cases of hypoxic epileptic and one nonepileptic myoclonus of Lance-Adams syndrome (LAS). Methods: We used PER in two patients with LAS after they attempted suicide. Both arrived in a deep coma at the hospital.  When initial sedative drug used in intensive care unit were tapered down  the two patients presented with a typical myoclonus of LAS type. Both patients had super refractory very disabling non epileptic myoclonus. They were not able to walk, neither to eat to drink without help.Both patients were hospitalized in rehabilitation a few weeks after allowing careful supervision. Most recommended drugs for myoclonus were tried for 6 to 8 month. Results: After most recommended drugs failed, the add-on administration of 2 then 4mg of PER significantly improved the myoclonus and subsequently its major functional burden. In these two patients the long term (after more than 6 month) PER clinical benefit and tolerance were excellent. Walking with help, eating, drinking alone became possible again. Conclusions: PER may be a very useful drug in super refractory myoclonus of the LAS after the failure of the usually recommended drugs. PER may decrease myoclonus and improve autonomy in some LAS patients, with a good long term tolerance. Funding: No funding