Continuous EEG Findings in Autoimmune Encephalitis.

PURPOSE: Autoimmune encephalitis (AE) is a cause of new-onset seizures, including new-onset refractory status epilepticus, yet there have been few studies assessing the EEG signature of AE. METHODS: Multicenter retrospective review of patients diagnosed with AE who underwent continuous EEG monitoring. RESULTS: We identified 64 patients (male, 39%; white, 49%; median age, 44 years); of whom, 43 (67%) were antibody-proven AE patients. Of the patients with confirmed antibody AE, the following were identified: N-methyl-D-aspartate receptor (n = 17, 27%), voltage-gated potassium channel (n = 16, 25%), glutamic acid decarboxylase (n = 6, 9%), and other (n = 4, 6%). The remaining patients were classified as probable antibody-negative AE (n = 11, 17%), definite limbic encephalitis (antibody-negative) (n = 2, 3%), and Hashimoto encephalopathy (n = 8, 13%). Fifty-three percent exhibited electrographic seizures. New-onset refractory status epilepticus was identified in 19% of patients. Sixty-three percent had periodic or rhythmic patterns; of which, 38% had plus modifiers. Generalized rhythmic delta activity was identified in 33% of patients. Generalized rhythmic delta activity and generalized rhythmic delta activity plus fast activity were more common in anti-N-methyl-D-aspartate AE (P = 0.0001 and 0.0003, respectively). No other periodic or rhythmic patterns exhibited AE subtype association. Forty-two percent had good outcome on discharge. Periodic or rhythmic patterns, seizures, and new-onset refractory status epilepticus conferred an increased risk of poor outcome (OR, 6.4; P = 0.0012; OR, 3; P = 0.0372; OR, 12.3; P = 0.02, respectively). CONCLUSION: Our study confirms a signature EEG pattern in anti-N-methyl-D-aspartate AE, termed extreme delta brush, identified as generalized rhythmic delta activity plus fast activity in our study. We found no other pattern association with other AE subtypes. We also found a high incidence of seizures among patients with AE. Finally, periodic or rhythmic patterns, seizures, and new-onset refractory status epilepticus conferred an increased risk of poor outcome regardless of AE subtype.

Survey of the diagnostic and therapeutic approach to new-onset refractory status epilepticus.

PURPOSE: We conducted a survey of providers to assess for practice patterns in diagnosing and treating new-onset refractory status epilepticus (NORSE). NORSE is the occurrence of prolonged seizures that are not responsive to initial therapies in otherwise healthy individuals without obvious cause on initial presentation. This entity is thought to have multiple etiologies, including autoimmune. METHOD: A 29-question electronic survey was sent to providers included in the Neurocritical Care Society emailing list. Questions regarded responders' demographics, existing institutional practice protocols, diagnostic work-up, therapeutic management and expected outcomes in NORSE. Responses were collected from October 23, 2014, to November 25, 2014. RESULTS: There were 107 respondents out of 1334 (8%). CT head, continuous EEG, lumbar puncture and microbe serologies were suggested within 24h of presentation as part of a diagnostic work-up. MRI brain, autoimmune work-up (systemic and anti-neuronal antibodies) and cytology/flow cytometry were favored later in the course. About 25% of providers would never perform an autoimmune work-up in this setting. Initial treatment included up to 3 anticonvulsants (including one anesthetic), followed by additional anticonvulsants/anesthetics along with antimicrobials, followed by steroids, plasma exchange, hypothermia and ketogenic diet. Many respondents would never use IV immunoglobulin or steroid-sparing immunosuppressants (29% and 42%, respectively) for NORSE. CONCLUSIONS: This survey could serve as the foundation for development of a standardized approach for the diagnosis and treatment of NORSE.

Vestibular rehabilitation: rationale and indications.

Treatment options of the patient with dizziness include medication, rehabilitation with physical therapy, surgery, counseling, and reassurance. Here the authors discuss vestibular rehabilitation for patients with benign paroxysmal positional vertigo (BPPV), unilateral vestibular loss or hypofunction, and bilateral vestibular loss/hypofunction. They describe the different mechanisms for recovery with vestibular rehabilitation, the exercises that are used, and which ones are best. An exhaustive literature review on clinical outcomes with the best research publications for BPPV, unilateral vestibular loss/hypofunction, and bilateral vestibular loss/hypofunction is presented. For BPPV, the authors also summarize the evidence-based review practice parameters published in Neurology by Fife et al. (2008) and review all relevant articles published since then.