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We compared sudden unexpected death in epilepsy (SUDEP) diagnosis rates between North American SUDEP Registry (NASR) epileptologists and original death investigators, to determine degree and causes of discordance. In 220 SUDEP cases with post‐mortem examination, we recorded the epileptologist adjudications and medical examiner‐ and coroner‐ (ME/C) listed causes of death (CODs). COD diagnosis concordance decreased with NASR's uncertainty in the SUDEP diagnosis: highest for Definite SUDEP (84%, n = 158), lower in Definite Plus (50%, n = 36), and lowest in Possible (0%, n = 18). Rates of psychiatric comorbidity, substance abuse, and toxicology findings for drugs of abuse were all higher in discordant cases than concordant cases. Possible SUDEP cases, an understudied group, were significantly older, and had higher rates of cardiac, drug, or toxicology findings than more certain SUDEP cases. With a potentially contributing or competing COD, ME/Cs favored non–epilepsy‐related diagnoses, suggesting a bias toward listing CODs with structural or toxicological findings; SUDEP has no pathognomonic features. A history of epilepsy should always be listed on death certificates and autopsy reports. Even without an alternate COD, ME/Cs infrequently classified COD as "SUDEP." Improved collaboration and communication between epilepsy and ME/C communities improve diagnostic accuracy, as well as bereavement and research opportunities. [ABSTRACT FROM AUTHOR]

Objective We studied our collective open-label, compassionate use experience in using cannabidiol (CBD) to treat epilepsy in patients with CDKL5 deficiency disorder and Aicardi, Doose, and Dup15q syndromes. Methods We included patients aged 1–30 years with severe childhood-onset epilepsy who received CBD for ≥10 weeks as part of multiple investigator-initiated expanded access or state access programs for a compassionate prospective interventional study: CDKL5 deficiency disorder (n = 20), Aicardi syndrome (n = 19), Dup15q syndrome (n = 8), and Doose syndrome (n = 8). These patients were treated at 11 institutions from January 2014 to December 2016. Results The percent change in median convulsive seizure frequency for all patients taking CBD in the efficacy group decreased from baseline [n = 46] to week 12 (51.4% [n = 35], interquartile range (IQR): 9–85%) and week 48 (59.1% [n = 27], IQR: 14–86%). There was a significant difference between the percent changes in monthly convulsive seizure frequency during baseline and week 12, χ 2 (2) = 22.9, p = 0.00001, with no difference in seizure percent change between weeks 12 and 48. Of the 55 patients in the safety group, 15 (27%) withdrew from extended observation by week 144: 4 due to adverse effects, 9 due to lack of efficacy, 1 withdrew consent, and 1 was lost to follow-up. Significance This open-label drug trial provides class III evidence for the long-term safety and efficacy of CBD administration in patients with treatment-resistant epilepsy (TRE) associated with CDKL5 deficiency disorder and Aicardi, Dup15q, and Doose syndromes. Adjuvant therapy with CBD showed similar safety and efficacy for these four syndromes as reported in a diverse population of TRE etiologies. This study extended analysis of the prior report from 12 weeks to 48 weeks of efficacy data and suggested that placebo-controlled randomized trials should be conducted to formally assess the safety and efficacy of CBD in these epileptic encephalopathies. [ABSTRACT FROM AUTHOR]

Although cases of trigeminal neuralgia (TN) induced by brainstem infarct have been reported, the neurosurgical literature lacks a comprehensive review for this subpopulation of patients. We present the first systematic review of the literature to discuss pathology, surgical management, and future directions for therapeutic innovation in this population. Our systematic review was conducted according to PRISMA guidelines. Resulting articles were screened for those that presented cases of TN associated with brainstem infarct. A review of the literature identified 18 case reports of 21 patients with TN induced by brainstem infarct: 14 pontine infarcts and 7 medullary infarcts. Although many cases of ischemic brainstem lesions are caused by acute stroke, cerebral small vessel disease also plays a role in certain cases, and the relationship between these chronic lesions and TN is more likely to be overlooked. Furthermore, we found that reports of self-resolving TN pain after brainstem infarct is disproportionately biased, as most case reports published their data within the first few months after initial presentation. Reports with follow-up periods >13 months reported eventual pain recurrence that necessitated surgical intervention. Microvascular decompression was not sufficient to treat TN pain associated with concurrent neurovascular compression and brainstem infarct. Brainstem infarcts affecting the trigeminal pathway represent an understudied pathologic cause of TN. Although the neurosurgical literature lacks a clear picture of the most efficacious interventions in this population, we are optimistic that this review will encourage further investigation into the best treatment for these patients. [ABSTRACT FROM AUTHOR]