Epilepsy Emergencies: Status Epilepticus, Acute Repetitive Seizures, and Autoimmune Encephalitis

Stephen VanHaerents, MD; Elizabeth E. Gerard, MD p. 454-476 April 2019, Vol.25, No.2 doi: 10.1212/CON.0000000000000716
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PURPOSE OF REVIEW: This article reviews epilepsy emergencies, including status epilepticus, acute repetitive seizures, autoimmune encephalitis, and the current perspective on their diagnosis and treatment.

RECENT FINDINGS: Recent guidelines on the treatment of status epilepticus from the Neurocritical Care Society in 2012 and the American Epilepsy Society in 2016 highlight areas of consensus in the treatment of status epilepticus as well as areas of uncertainty. The TRENdS (Treatment of Recurrent Electrographic Nonconvulsive Seizures) trial is the first prospective randomized clinical trial to evaluate the efficacy of IV antiseizure medications in controlling nonconvulsive seizures on continuous EEG. It demonstrated that IV lacosamide is noninferior to fosphenytoin in this setting. Autoimmune encephalitis is an increasingly recognized cause of new-onset seizures or status epilepticus. Recently described scoring systems, the Antibody Prevalence in Epilepsy score and the Response to Immunotherapy in Epilepsy score, can help in the assessment of autoimmune encephalitis.

SUMMARY: Status epilepticus, acute repetitive seizures, and autoimmune encephalitis are neurologic emergencies. For all these conditions, rapid and appropriate treatment may influence patient prognosis and mitigate neuronal injury. For convulsive status epilepticus, there is reasonable consensus on the initial steps that need to be taken. There is less agreement about the management of acute repetitive seizures and nonconvulsive status epilepticus. An increasingly recognized etiology of status epilepticus is autoimmune encephalitis, which may not be as rare as previously thought.

Address correspondence to Dr Stephen VanHaerents, 675 N St Clair, Ste 7-112, Chicago, IL 60611, svanhaer@gmail.com.

RELATIONSHIP DISCLOSURE: Dr VanHaerents has received research/grant support from the Citizens United for Research in Epilepsy, National Institute of Mental Health, National Institute on Aging, National Institutes of Health, and SAGE Therapeutics. Dr VanHaerents has received travel honoraria from NeuroPace and SAGE Therapeutics. Dr Gerard has received personal compensation as a lecturer for the Society for Maternal-Fetal Medicine, Society of OB/GYN Hospitalists, and UCB China and has received research/grant support from the Eleanor Wood-Prince Grant from the Woman’s Board of Northwestern Memorial Hospital, the National Institute of Neurological Disorders and Stroke/National Institutes of Health, SAGE Therapeutics, and Sunovion Pharmaceutical Inc.

UNLABELED USE OF PRODUCTS USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs VanHaerents and Gerard discuss the unlabeled/investigational use of immunosuppressant medications for the treatment of autoimmune encephalitis (cyclophosphamide, IV immunoglobulin, IV methylprednisolone/corticosteroids, plasma exchange, and rituximab) and the pharmacologic and nonpharmacologic therapies for the treatment of seizures and status epilepticus (convulsive and nonconvulsive), which include diazepam, fosphenytoin/phenytoin, levetiracetam, lorazepam, midazolam, phenobarbital, and valproate sodium/valproic acid. Drs VanHaerents and Gerard discuss the unlabeled/investigational use of several agents for the treatment of refractory and super-refractory status epilepticus, which include corticosteroids/methylprednisolone, electroconvulsive therapy, hypothermia, isoflurane, IV immunoglobulin, ketamine, ketogenic diet, midazolam, pentobarbital, propofol, thiopental, transcranial magnetic stimulation, and vagal nerve stimulation.

© 2019 American Academy of Neurology.