Abstracts

Rationale: Report a case of Lance Adams Syndrome (LAS) with disabling myoclonus responding to Brivaracetam refractory to other suggested medications. Chronic post-hypoxic myoclonus, also known as LAS is a neurological complication characterized by uncontrolled myoclonic jerks following anoxic brain injury. There’s currently no consensus on pharmacological therapy but there’s some suggestion that levetiracetam, valproic acid, Zonisamide, clonazepam, and perampanel are helpful. Despite treatment with available anti-myoclonic agents, some patients may recover cognitively but remain completely disabled by severe myoclonus. Methods: We report a 31 year old man with history of LAS after anoxic brain injury in 2003, when he choked on food and was anoxic for approximately 30 minutes. During his hospital course, he developed LAS characterized by stimulus induced myoclonus without cognitive decline. He was started on clonazepam early during his disease with partial control of his myoclonus. Higher doses of clonazepam didn’t significantly improve his symptoms and caused significant fatigue and drowsiness. Other medications were unsuccessfully tried as add-ons including Keppra, Depakote, Zonisamide, Tryptophan, and even vagal nerve stimulator. He was finally tried on Brivaracetam at a dose of 50 mg BID in addition to clonazepam. Results: The patient reported dramatic improvement in his myoclonus reporting minimal symptoms while walking and performing fine motor tasks which he initially had difficulty performing. He reported increased somnolence which resolved after the two-week titration, but otherwise no adverse effects were reported.  Conclusions: Brivaracetam, approved for treatment of Focal onset seizures by the US FDA in February 2016, is believed to act by binding to the ubiquitous synaptic vescicle glycoprotein 2A, like levetiracetam but with 20-fold greater affinity, though the exact mechanism is unclear. With the incidence of LAS increasing due to improved survival post cardiac arrest, Brivaracetam can prove a valuable add on therapy for action myoclonus in LAS with relatively fewer side effects. In our literature search, we did not find any reported cases of successful treatment of myoclonus in LAS with Brivaracetam Funding: None