Hocker, Sara E.

Purpose: Neuroinvasive West Nile virus (WNV) is rare, occurring in less than 1% of those infected, and may manifest as meningitis, encephalitis, and/or acute flaccid paralysis. Patients may present initially with nonspecific symptoms including fevers. Although rare, neuroinvasive WNV is associated with significant morbidity and mortality. The mainstay of treatment is supportive care. Electroencephalography (EEG) allows for identification of nonconvulsive status epilepticus and other epileptiform and nonepileptiform patterns suggestive of underlying cognitive dysfunction. Our aim was to describe specific EEG patterns observed in WNV neuroinvasive disease. Methods: A retrospective chart review was conducted. West Nile virus was confirmed with serum and/or cerebrospinal fluid markers. Patients with a history of abnormal EEG were excluded. Electroencephalography reports were classified into categories based on the presence of epileptiform activity, focal slowing, generalized periodic discharges with triphasic morphology, and frontally predominant generalized rhythmic delta activity. Results: In our cohort of 34 patients, 60% of focal EEG abnormalities were anterior-predominant, seen as epileptiform discharges, focal slowing, or frontally predominant generalized rhythmic delta activity. Nonepileptiform EEG patterns included nonspecific slowing and generalized periodic discharges with triphasic morphology. Two patients had electrographic seizures, one arising from the frontocentral head region. Conclusions: EEGs are important in the evaluation of WNV infection to rule out seizures or alternative causes of encephalopathy, and because of the risk of nonconvulsive seizures or status epilepticus in encephalitis. Although an anterior predominance of EEG abnormalities was seen in our cohort, this most likely is more correlative to encephalopathy than WNV itself. Although a specific correlative EEG pattern may not accompany all cases of WNV neuroinvasive disease, WNV should be considered as a possible etiology in patients presenting with an encephalitic or meningitic syndrome in the presence of abnormal EEG findings including encephalopathic patterns, particularly those with anterior predominant EEG changes.

SummaryWorsening pharmacoresistance to antiseizure drugs is common with ongoing excitotoxic neuronal and systemic injury. Early initiation of anesthetic drugs in refractory status epilepticus (RSE) may halt these processes while allowing time for treatment targeting the cause of the seizures. Current guidelines support the use of anesthetic drugs as the third line pharmacologic therapy in generalized convulsive status epilepticus but do not clearly define the indications for these drugs in other types of status epilepticus. There is wide practice variation in choice of third line therapy for RSE, but there is overall consensus that anesthetics should be initiated earlier in generalized convulsive status epilepticus than in nonconvulsive forms. More recently, doubt has been cast on the appropriateness of anesthetic treatment of RSE following a series of studies associating their use with higher mortality and morbidity. This suggests that efforts should focus on determination of who benefits most, optimal use, and prevention of refractoriness. The risk‐benefit ratio of anesthetic use is discussed, with specific indications proposed. In addition, anesthetic dosing, supportive neurocritical care, electroencephalogram suppression target, and weaning of anesthesia are reviewed.

SummaryDespite phenobarbital (PB) being a key component in status epilepticus (SE) treatment algorithms for decades, it has fallen out of favor compared to newer nonsedating medications due to potential for respiratory suppression and prolonged sedation. We retrospectively analyzed all nonintubated patients with refractory SE treated with parenteral PB. Forty patients were identified as having received PB in the neurologic intensive care unit at Mayo Clinic over a 7‐year period through our pharmacy dispensing database. Patients who received PB for maintenance therapy, those replenishing subtherapeutic levels, those who were already intubated, and those receiving PB for a non‐SE indication were excluded. Clinical data, prior treatments, therapeutic response, and outcome were reviewed. Eight patients were identified. Ages ranged from 24 to 77 years (median = 64 years); all had focal SE, and none was comatose. Seizure activity improved acutely following PB administration in seven and stopped in six. Dosages ranged from 5 to 19.8 mg/kg (median = 10.1 mg/kg); none required intubation, and one received supplemental oxygen. Patients received a median of four antiepileptic drugs prior to PB. Median interval between first drug and PB was 23.5 hours. Glasgow Coma Scale score did not change following PB administration. One patient required intervention for hypotension. Moderate‐dose parenteral PB was effective in attaining seizure control in a significant proportion of noncomatose refractory SE patients. None required ventilatory support. PB dosages below those in recent guidelines may be sufficient to stop SE without clinically significant cardiopulmonary complications.

Summary To describe the demographics, etiologies, types of status epilepticus ( SE ), and outcomes in people with refractory and super‐refractory SE from around the world, we prospectively collected cases of refractory SE ( RSE ) treated with continuous intravenous anesthetic drugs in an intensive care unit setting through online questionnaires using “active surveillance.” We collected information about 776 cases of RSE in 50 countries over 4 years. Control of SE was achieved in 74% of the cases. Neurologic outcomes were poor in 41% of patients, and 24% died. Good outcome was associated with younger age and a history of epilepsy. Etiology strongly influenced the outcome. Patients from Asia were younger, more frequently presented with convulsive SE , and were more frequently affected by infectious etiologies when compared with patients from Europe and the Americas. Despite these differences, outcomes were similar in all countries. Demographics of patients with RSE in a global audit are similar to those in prior single center series, providing evidence of generalizability of those studies. Important differences exist among patients with RSE from different regions of the world, but these do not seem to significantly influence patient outcomes.

AbstractAcute neurologic (and potentially disability prone) conditions are rarely taught and, if so, at the bedside and usually after the fact. Simulation training offers a realistic environment to teach rapid-fire decision making, how to take charge, and how best to approach a deteriorating, acutely ill neurologic patient.Simulating acute neurology is a newly emerging educational program. Proven principles of simulation, which may include teaching technical skills, can be applied to acute neurology (and neurointensive care). Communication skills pertaining to catastrophic neurologic injury with all its ethical ramifications can be taught and practiced. There is a growing need for well-designed, tested teaching scenarios and a need to validate this approach. This review provides an overview of the potential opportunities available in medical institutions with a simulation center.

SummaryWe convened an international group of experts to standardize definitions of New‐Onset Refractory Status Epilepticus (NORSE), Febrile Infection‐Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in‐person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care. The proposed consensus definitions are as follows: NORSE isa clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause. FIRES is a subcategory of NORSE, applicable for all ages, that requires a prior febrile infection starting between 2 weeks and 24 hours prior to onset of refractory status epilepticus, with or without fever at onset of status epilepticus. Proposed consensus definitions are also provided for Infantile Hemiconvulsion‐Hemiplegia and Epilepsy syndrome (IHHE) and for prolonged, refractory and super‐refractory status epilepticus. This document has been endorsed by the Critical Care EEG Monitoring Research Consortium. We hope these consensus definitions will promote improved communication, permit multicenter research, and ultimately improve understanding and treatment of these conditions.