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Aims: To conduct a review of the literature on the use of botulinum toxin for the treatment of pediatric chronic migraine. Methods: A review of the literature was performed using EMBASE, PubMed, and Cochrane/Ovid. Using our inclusion and exclusion criteria, we targeted any study, published before April 2020, evaluating the efficacy of botulinum toxin in migraineurs younger than 18 years. Results: Seven studies were included: 2 retrospective analyses, 3 case series, a case report, and a randomized control trial. Studies included 1 to 125 patients, with the number of botulinum toxin treatments ranging from 1 to 11 treatments. The results of the randomized controlled trial showed nonsuperiority between placebo and botulinum toxin. Results of the other studies were generally favorable but were difficult to compare because of lack of standardization of botulinum toxin dosing, injection paradigm, frequency and duration of treatment, usage of accompanying prophylaxis, and variation in outcome measures across studies. There was low-quality evidence that botulinum toxin improved headache frequency and intensity, though some studies demonstrated efficacy in treatment with botulinum toxin. Conclusion: This review is the first of its kind, updating the literature on the efficacy of botulinum toxin in pediatric patients. Given evidence of its utility in treating pediatric migraines, off-label use should be considered in certain cases. Further study is warranted to better characterize injection paradigms and patient selection because of the limited and inconsistent data available.

Aims To conduct a systematic review of the literature on the use of botulinum toxin for the treatment of cluster headache. Methods A systematic review and data quality analysis were performed using PRISMA and GRADE guidelines, respectively. Inclusion and exclusion criteria were outlined prior to the search and aimed to select prospective studies that examined the use of botulinum toxin for the treatment of cluster headache. Results Three studies resulted from the search that each included 10 to 17 subjects. All three demonstrated significant improvement in the frequency of headaches that occurred as quickly as 1 week following treatment. There was low-quality evidence that botulinum toxin was effective in reducing headache frequency and severity by at least 50%. Injections into the sphenopalatine ganglion may have a higher incidence of adverse events. Conclusion This review summarizes the only prospectively collected efficacy and safety data regarding the use of botulinum toxin in cluster headache. Off-label use should be considered in certain cases. Further study is warranted to better characterize injection paradigms and patient selection, given the encouraging but limited data available.

Background and ObjectiveMedication management in pregnant women with epilepsy (PWWE) poses challenges, and understanding the effects of metabolic changes on antiseizure medications (ASMs) is important in planning care for PWWE. The possible teratogenic effects and risks of poorly controlled seizures have to be weighed. There are data in the literature on clinical management of ASMs including the effects of drug levels on seizures and factors that predict seizure frequency, but timing and frequency of monitoring and dose adjustment paradigms have not been well studied.MethodsThis retrospective study was approved by the Institutional Review Board at Johns Hopkins University. We retrospectively identified adult PWWE evaluated during pregnancy at the Johns Hopkins Bayview Medical Center epilepsy clinic, between January 1, 2007, and January 1, 2021. We reviewed charts for information regarding demographics, medical and epilepsy history, medications, serum drug levels, and dosing paradigms. We assessed risk factors for breakthrough seizures with a focus on frequency and timing of laboratory testing. We calculated the dose-normalized concentration (DNC) for analysis with levetiracetam and lamotrigine, assessing changes in DNC over time by half trimesters, and analyzed DNC and effects on seizures in pregnancy. We also compared preemptive vs clinically based lamotrigine dose adjustments in managing epilepsy in pregnancy.ResultsA total of 45 pregnancies in 39 patients were included in this study, 8 generalized, 28 focal epilepsy, and 3 unclassified. 31 PWWE (36 pregnancies) were on lamotrigine and/or levetiracetam, and 14 of these pregnancies experienced breakthrough seizures, 77% in the first trimester. Seizures led to the diagnosis of pregnancy in 5 patients. The DNC for levetiracetam decreased significantly compared with prepregnancy levels by the second half of the first trimester and demonstrated variable but frequently significant or near significant reduction throughout pregnancy. DNC for lamotrigine decreased significantly in the first half of the first trimester and remained significant throughout pregnancy. Age of mother at conception, week of first ASM serum level and number of levels obtained during pregnancy, and epilepsy type were not associated with breakthrough/increase in seizures. The history of drug resistance (p= 0.038) was associated with a higher odds of seizures. In those on lamotrigine, preemptive dose adjustments demonstrated similar results regarding seizure control when compared with clinical-based or laboratory-based dose management (p= 0.531).DiscussionThis study demonstrates that frequency and timing of ASM level monitoring may not affect overall seizure outcomes during pregnancy in those on lamotrigine or levetiracetam. Furthermore, one can consider preemptive dose adjustments or a laboratory-based/clinical-based approach in managing lamotrigine as both seem safe and feasible. However, in those with drug-resistant epilepsy before pregnancy, earlier and closer monitoring is warranted given the risk of seizures early during pregnancy. Larger prospective studies are needed to confirm these results.

SUMMARY Triphasic waves are EEG phenomena typically seen in patients with acute encephalopathy and have importance in diagnosis and prognosis in these cases. The underlying metabolic disturbances associated with their incidence have been described previously, but neuroimaging characteristics are not well delineated. There are a few small studies that define neuroimaging results in patients with triphasic waves. This review highlights the most common neuroimaging findings in these patients, including subcortical white matter disease, which itself may be a risk factor for triphasic waves.

The authors describe a case of a 70-year-old man presenting with increased right arm flexion, right leg extension, and limb shaking ischemic events because of an atypical anterior cerebral artery (ACA) stroke in concurrence with normal vital signs.1 We agree with the concluded localization of the stroke. However, serial focal motor seizures, focal-impaired awareness seizures, or status epilepticus with acute stroke may be more likely explanations of the event than limb shaking ischemic events and an akinetic mutism resulting in seizure mimicry.

Highlights • Smartphone videos are useful cost-effective means to evaluate and characterized epileptic and nonepileptic seizures. • Convulsive and motor semiologies are particularly well-suited for accurate interpretation using smartphone videos but require ictal-post-ictal separation. • Patients and caregivers should be educated on optimal smartphone video recording focused on capturing the onset of the event. • Long-term video-EEG monitoring remains the gold standard when discordant video information is presented or diagnostic questions remain after clinical evaluation., Expert review of seizure semiology looking at video recordings independent of EEG has been found to be useful for diagnosing functional seizures. Videos recorded outside the hospital containing “spells” have similar sensitivity to EEG when quality recordings are evaluated. Recently, smartphone videos were shown to serve as an adjunct to standard history and physical examination with similar diagnostic yields when compared to diagnostic video-EEG monitoring and reviewed by experts. However, caution must be exercised when interpreting videos of paroxysmal neurological events recorded by caregivers to ensure proper video quality is maintained and recorded event is representative. In this report, we present a case of initial identification of and event falsely suggesting functional seizures in a patient with epilepsy. The smartphone video of a “seizure” was recorded by his wife using her smartphone. Despite a quality recording and a history consistent with epilepsy, the smartphone video reviewed during evaluation in the clinic suggested a functional behavior in contrast to the history that suggested epilepsy manifest as convulsions. Instead of bilateral tonic-clonic motor movements, bizarre, intermittent non-clonic wild flinging movements and vocalization were identified on the smartphone video. The discordance between the clnical history and ideo prompted inpatient video-EEG monitoring. The same nonepileptic semiology was subsequently clarified to represent a physiological nonepileptic event. The event on the smartphone was typical of his agitated post-ictal state following an electroclinical tonic-clonic seizure. With treatment the seizures became controlled with antiseizure medication in long-term follow-up. We highlight the pitfalls using patient-recorded smartphone videos in patients diagnosed with epilepsy. Understanding the utility of smartphones as an adjunct to the clinical history will help in differentiating epileptic from functional seizures.

Purpose The purpose of this analysis is to assess the effect of antiepileptics (AEDs) on seizure prevention and short and long term functional outcomes in patients with acute intracerebral hemorrhage. Method The meta-analysis was conducted using the PRISMA guidelines. A literature search was performed of the PubMed, the Cochrane Library, and EMBASE databases. Search terms included “Anticonvulsants”, “Intracerebral Hemorrhage”, and related subject headings. Articles were screened and included if they were full-text and in English. Articles that did not perform multivariate regression were not included. Overall effect size was evaluated with forest plots and publication bias was assessed with the Begg’s and Egger’s tests. Results A total of 3912 articles were identified during the initial review. After screening, 54 articles remained for full review and 6 articles were included in the final analysis. No significant association between the use of AEDs after ICH and functional outcome (OR 1.53 [95%CI: 0.81–2.88] P = 0.18, I 2 = 81.7%). Only one study evaluated the effect AEDs had in preventing post-ICH seizures. Conclusions The use of prophylactic AEDs was not associated with improved short and long outcomes after acute ICH. This analysis supports the 2015 AHA/ASA recommendation against prophylactic AEDs (class III; level of evidence b).

Anti-N-methyl-d-aspartate receptor encephalitis (anti-NMDARE) is common among autoimmune encephalitides (AE) and can present with protean features and nonspecific laboratory and neuroimaging results. Antibody testing is specific, but results are often delayed, and prompt diagnosis and treatment is vital given associated morbidity and mortality. Electroencephalography (EEG) may be a useful tool given ease of performance and short turn-around time for results. As such, we performed a systematic review of the literature on anti-NMDARE and EEG characteristics. We found that EEG is often pathologic in anti-NMDAR encephalitis, though at times nonspecific. A stronger focus on common EEG features in patient with this type of encephalitis and an effort to identify when these features are present in the course of the disease may allow for improved diagnosis, description and differentiation of anti-NMDARE.

BACKGROUND Botulinum toxin is approved to treat chronic migraine and has been shown to confer significant benefit in refractory cases. Due to its effect on pain by sensory modulation, there may also be efficacy in its use in chronic tension-type headache (CTTH). METHODS A systematic review of the literature was performed using our predefined inclusion and exclusion criteria. We targeted prospective trials, randomized or nonrandomized, studying botulinum toxin in tension-type headaches (TTHs) in adults. RESULTS Twenty-two studies were included, including 9 nonrandomized, uncontrolled studies, 8 randomized, placebo-controlled and double-blinded trials (RCTs), 3 RCTs with a crossover, open-label period, 1 comparative, randomized, single-blinded evaluation, and 1 retrospective study with prospective evaluation of headache response to cosmetic botulinum toxin. Studies included 11 to 300 subjects, with duration typically less than 6 months and with only 1 treatment period. Results were mixed, likely due to variable study design, including toxin dosing, injection paradigms, duration/frequency of treatment, and outcome measures. There was moderate-quality evidence that botulinum toxin improved VAS scoring, and some studies demonstrated efficacy based on improved frequency/severity. CONCLUSION This systematic review demonstrates variable study designs contributing to the low quality of evidence available regarding botulinum toxin in TTH, but some data suggest efficacy. There does not appear to be irrefutable evidence of a lack of efficacy of botulinum toxin in TTH. Using the paradigm for botulinum toxin in chronic migraine may prove fruitful in treating CTTH. Further studies are warranted to evaluate the utility of botulinum toxin in this common debilitating condition.

Triphasic waves are EEG phenomena typically seen in patients with acute encephalopathy and have importance in diagnosis and prognosis in these cases. The underlying metabolic disturbances associated with their incidence have been described previously, but neuroimaging characteristics are not well delineated. There are a few small studies that define neuroimaging results in patients with triphasic waves. This review highlights the most common neuroimaging findings in these patients, including subcortical white matter disease, which itself may be a risk factor for triphasic waves.

This study examines the safety and effectiveness of brain-responsive neurostimulation (RNS System) therapy in patients with refractory autoimmune-associated epilepsy (AAE).We retrospectively reviewed 85 medical records of patients who were treated with the RNS System at the three Mayo Clinic sites to identify patients with AAE. We collected clinical data including demographics, epilepsy history, prior evaluations and treatment, RNS implantation and lead information, long term ambulatory electrocorticography (ECoG) data, and patient-reported seizure details. Inclusion criteria included: (1) confirmed neural antibodies, or absent/negative autoimmune panel with Antibody Prevalence in Epilepsy (APE2) score ≥ 4 and (2) at least 6 months of follow up after RNS implantation. The primary outcomes measured were patient-reported seizure frequencies at last follow-up as compared to baseline, ECoG long-episode frequencies, and adverse events following RNS implantation.Of the 85 patients reviewed, nine (11 %) met the inclusion criteria. Three patients had GAD65 antibodies (mean serum titer = 816 ug/dl), one had Rasmussen's encephalitis (biopsy proven), and five had absent/negative neuronal antibody panel but APE scores were ≥ 4. Six out of nine patients (67 %) reported improvement in clinical seizure frequency, all reported improvements in seizure duration and intensity. Four of 9 patients (44 %) showed trends of decreasing frequency of prolonged periods of epileptiform activity over time. One patient (11 %) developed a superficial wound infection at the implant site.The findings from our study suggest that adjunctive treatment with the RNS System may be a safe option for patients with refractory AAE.

Distinguishing treatable causes for rapidly progressive dementia from those that are incurable is vital. Creutzfeldt-Jakob disease (CJD) and voltage-gated potassium channel complex–associated autoimmune encephalitis (VGKC AE) are 2 such conditions with disparate outcomes and response to treatment. To determine the differences in electroencephalography between CJD and VGKC AE, we performed a retrospective review of medical records and examined clinical data, neuroimaging, and electroencephalographs performed in patients admitted for evaluation for rapidly progressive dementia diagnosed with CJD and VGKC AE at the Johns Hopkins Hospital and Bayview Medical Center between January 1, 2007 and December 31, 2015. More patients in the VGKC AE group had seizures (12/17) than those with CJD (3/14; P = .008). Serum sodium levels were lower in those with VGKC AE ( P = .001). Cerebrospinal fluid (CSF) white blood cell count was higher in VGKC AE ( P = .008). CSF protein 14-3-3 ( P = .018) was more commonly detected in CJD, and tau levels were higher in those with CJD ( P < .006). On neuroimaging, diffusion restriction in the cortex ( P = .001), caudate ( P < .001), and putamen ( P = .001) was more frequent in CJD. Periodic sharp wave complexes ( P = .001) and generalized suppressed activity ( P = .008) were more common on initial EEG in CJD. On serial EEGs, generalized periodic discharges ( P = .004), generalized suppressed activity (P=0.008), and periodic sharp wave complexes ( P < .001) were detected more in CJD. This study shows that there are a number of differentiating features between CJD and VGKC AE, and electroencephalography can aid in their diagnoses. Performing serial EEGs better delineates these conditions.

PURPOSE Periodic discharges (PDs) are EEG patterns denoting brain dysfunction and ictal tendency. Their exact meaning regarding etiology and outcomes is not well known. In particular, bilateral independent PDs (BIPDs) are poorly described. METHODS We performed a retrospective, multicenter study evaluating neuroimaging, epileptic, clinical, and EEG correlates of BIPDs. RESULTS Twenty-five patients studied with a mean Glasgow Coma Scale 6.5 and modified Rankin scale 3.9 who underwent EEG monitoring, mean duration 287 hours (range 0.75-3,216). Most common causes of BIPDs were cardiac arrest, Central Nervous System infections, and acute/chronic ischemic/hemorrhagic stroke. Most had subcortical and cortical injuries on neuroimaging. Most of the PDs ranged from 0.5 to 2 Hz in frequency, were of multiple phase types, and localized to the frontal head regions. Eighteen of 25 patients had clinical or electrographic seizures. There was a trend toward seizures in those with BIPDs with a history of epilepsy (P = 0.08) and acute metabolic dysfunction (P = 0.08), particularly with coincident acute structural lesions (P = 0.05). Seizures were predicted by bilaterally symmetric frequencies (P = 0.02) and trended toward higher likelihood with PD frequency

Background: Therapeutic hypothermia and targeted temperature management are considered standard of care in the management of patients following out-of-hospital cardiac arrests due to shockable rhythms to improve neurological outcomes. In those presenting out-of-hospital cardiac arrests associated with non-shockable rhythms, the benefit of hypothermia is less clear. In this review we try to clarify the utility of implementing a hypothermia protocol after cardiac arrests due to non-shockable rhythms. Methods: PUBMED, Ovid, MEDLINE, EMBASE, and clinicaltrials.gov websites were searched through during October, 2016 using the terms “non shockable”, “hypothermia,” and “cardiac arrest.” Studies were excluded if they solely evaluated in-hospital cardiac arrests, shockable rhythms, and/or pediatric patients. Data was extracted by two authors. Results: Forty studies were included in this review, most of which were not randomized or controlled, nor were they powered to make significant conclusions about the efficacy of hypothermia in this population. Some did evaluate specific factors that may portend to a better outcome in patients presenting with out-of-hospital cardiac arrest due to non-shockable rhythms undergoing hypothermia. Shortcomings included incorporating in-hospital cardiac arrest patients in analyses, comparing results of hypothermia in shockable versus non-shockable rhythm patients as an outcome measure, lacking standardization in cooling protocols, and short-term measures of outcomes. Conclusions: It was concluded that further study is needed to characterize patients presenting nonshockable rhythms who would benefit from hypothermia to better guide its use in this population given the costs and implications of treatment and long-term care in those who survive with poor outcomes.

Pediatric_Botox_Paper_Supplemental_Table_2 for The Efficacy of Botulinum Toxin in Pediatric Chronic Migraine: A Literature Review by Raymundo Marcelo and Brin Freund in Journal of Child Neurology

Objective To investigate the magnitude of postural sway induced by different balance tasks in adolescents with concussion and to examine the associations of postural sway with concussion symptoms. Design Cross-sectional study. Patients or Other Participants Fifty-six adolescents (20 girls, 36 boys) between 13 and 17 years of age who sustained a concussion within the past 44 days and were still symptomatic. Main Outcome Measure(s) Anterior-posterior postural sway was measured using an accelerometer attached to the participant's lower back while he or she performed 6 static-balance tasks that varied the visual input, type of surface, and foot stance. Participants self-reported symptoms that occurred at the time of the concussion (eg, dizziness, confusion, amnesia) as well as at the time of balance testing (eg, eye and head movement–induced dizziness). Results The normalized path length of postural sway during the different balance tasks was greater with the eyes closed (mean = 19.3 mG/s) compared with the eyes open (mean = 12.4 mG/s; P < .001). Furthermore, sway while standing with the feet together on a foam surface (mean = 17.9 mG/s) or while tandem standing on a firm surface (mean = 19.4 mG/s) was greater than sway while standing with the feet together on a firm surface (mean = 10.3 mG/s; P < .001). Greater sway was associated with dizziness and confusion reported at the time of injury (P < .05). Dizziness and headache symptoms at rest were positively correlated with sway (P < .05). Conclusions Using accelerometers to measure postural sway during different challenging balance conditions in adolescents with concussion may provide an objective means of quantifying balance impairments in clinical environments. Furthermore, the association of these measurements with symptoms suggests a need to account for symptom severity at the time of testing.

Periodic discharges (PDs) are EEG patterns that may have important clinical and prognostic implications. There are different subtypes of PDs that are delineated by their location, and each type may have different meaning regarding prognosis and clinical associations. Bilateral independent PDs are a subtype that have not been analyzed recently and remain poorly understood. In this article, we systematically review the literature to better describe bilateral independent PDs regarding underlying neuropathology, neuroimaging, and neuroexamination correlates, seizure incidence, EEG characteristics, their comparison with other PD subtypes, and prognostic meaning.

Introduction: Intracranial hemorrhage (ICH) is a devastating form of stroke that leads to development of seizures in up to 2%-40% of patients. Seizure prophylaxis in ICH remains controversial and it is unclear if the benefits of antiepileptic drugs (AEDs) outweigh the risk of potential adverse drug effects. The objective of this analysis is to assess the effect of AED on seizure prevention and long term functional outcomes in patients with acute ICH. Methods: Our group conducted a meta-analysis using the PRISMA guidelines. A literature search was performed of the PubMed, the Cochran Library, and EMBASE databases using the keywords “Anticonvulsants”, “Intracerebral Hemorrhage”, and related subject headings. Articles were screened and included if they assessed the impact of AEDs on long-term functional outcomes in ICH population using multivariate logistic regression analysis. Overall effect sizes were evaluated with forest plots and publication bias was assessed with the Begg’s and Egger’s tests. Long term functional outcomes were assessed at ≥ 3 months post-ICH with either the National Institute of Health Stroke Scale (NIHSS) or the modified Rankin Score (mRS). Results: A total of 3,912 articles were identified during the intimal review. After screening, 54 articles remained for full review and 6 articles were included in the final analysis. The quality assessment criteria ranged from moderate to high for evidence synthesis. A total of 3,193 patients were included in the analysis. There were no significant association between the use of AEDs after ICH and poor outcome defined as mRS ≥3 or NIHSS ≥15 (OR 1.53 [95% CI: 0.81-2.88] p=0.18, I 2 =81.7%). Only one trial evaluated the effect AEDs had in prevent post-ICH seizures. Publication bias was not observed in the analysis (Egger’s bias=2.23, P=0.23). Conclusion: In conclusion, the use of prophylactic AEDs was not associated with improved or worse functional outcomes after acute ICH.

Prognostication after cardiac arrest often depends primarily on neurological function, and characterizing the extent of neurological injury hinges on neurophysiological testing and clinical neurological examination. The presence of early posthypoxic myoclonus (PHM) following cardiac arrest had been invariably associated with poor outcome, but more recent studies have shown that those with early PHM may survive with good neurological function. Electroencephalographic patterns suggestive of severe brain injury may be more valuable than the presence of PHM itself in portending poor functional status, and phenotyping PHM may also be useful in delineating benign and malignant forms. Patients with early PHM should be evaluated similarly to others who suffer cardiac arrest by using a multimodal approach in determining prognosis until further studies are performed that better characterize early PHM subtypes and their outcomes.

OBJECTIVEDelay in diagnosis and subsequent treatment of nonconvulsive seizures can lead to worsened outcomes. The gold standard in detecting nonconvulsive seizures is continuous video-electroencephalography (cEEG). Compared to routine, 30-minute EEG, the use of cEEG increases the likelihood of capturing intermittent nonconvulsive seizures. Studies of critically ill patients in intensive care units demonstrate a particularly high rate of nonconvulsive seizures. Some of these studies included postneurosurgical patients, but often subanalyses of specific populations were not done. In particular, few studies have specifically evaluated postneurosurgical patients by using cEEG in the acute postoperative setting. Therefore, the incidence and predictors of acute postneurosurgical seizures are unclear.METHODSIn this study, the authors focused on patients who were admitted to the neurological critical care unit following neurosurgery and who underwent cEEG monitoring within 72 hours of surgery.RESULTSA total of 105 cEEG studies were performed in 102 patients. Twenty-nine patients demonstrated electrographic (subclinical) seizures, of whom 10 had clinical seizures clearly documented either before or during cEEG monitoring. Twenty-two patients had subclinical seizures only detected on cEEG, 19 of whom did not have clinical seizure activity at any point during hospitalization. Those with seizures were more likely to have had a history of epilepsy (p = 0.006). The EEG studies of patients with seizures were more likely to show lateralized periodic discharges (p = 0.012) and lateralized rhythmic delta activity (p = 0.012). The underlying neuropathological disorders most associated with seizure risk were lobar tumor on presentation (p = 0.048), subdural hematoma (SDH) requiring craniotomy for evacuation (p = 0.002), subarachnoid hemorrhage (SAH) (p = 0.026), and perioperative SAH (p = 0.019). In those undergoing craniotomy, the presence of SDH (p = 0.032), particularly if requiring evacuation (p = 0.003), increased the risk of seizures. In those without preoperative intracranial bleeding, perioperative SAH after craniotomy was associated with a higher incidence of seizures (p = 0.014). There was an additive effect on seizure incidence when perioperative SAH as well as concomitant intraparenchymal hemorrhage and/or stroke were present. The clinical examination of the patient, including the presence or absence of altered mental status and the presence or absence of repetitive movements, was not predictive of subclinical seizures.CONCLUSIONSIn postneurosurgical patients referred for cEEG monitoring, there is a high rate of both clinical and subclinical seizures in the early postoperative period. Seizures are particularly common in patients with SDH or lobar tumor and perioperative SAH. There was an additive effect on seizure incidence when more extensive brain injury was present. As expected, those with a history of epilepsy also demonstrated higher seizure rates. Further studies are needed to evaluate the time period of maximum seizure incidence after surgery, and the effects acute postneurosurgical seizures have on long-term outcomes.

Morvan syndrome is a rare autoimmune/paraneoplastic disorder involving antibodies to the voltage-gated potassium channel complex. It is defined by subacute encephalopathy, neuromuscular hyperexcitability, dysautonomia, and sleep disturbance. It may present a diagnostic dilemma when trying to differentiate from amyotrophic lateral sclerosis with frontotemporal dementia.A 76-year-old man with a history of untreated prostate adenocarcinoma was evaluated for subacute cognitive decline, diffuse muscle cramps, and hyponatremia.MRI demonstrated atrophy most prominent in the frontal and temporal regions. Electromyography (EMG) demonstrated diffuse myokymia/neuromyotonia. Polysomnography lacked REM and N3 sleep. Paraneoplastic panel detected antibodies to voltage-gated potassium channel complex (CASPR2 subtype).It is difficult to differentiate between Morvan syndrome and amyotrophic lateral sclerosis with frontotemporal dementia with examination and neuroimaging alone. There may be a link between Morvan syndrome and prostate adenocarcinoma which could help with screening/diagnosis. The authors found that laboratory and neurophysiological tests are indispensable in diagnosing and treating Morvan syndrome.

Acute posthypoxic myoclonus portends a poor prognosis. Another form of posthypoxic myoclonus, Lance-Adams syndrome, is associated with a better outcome. Differentiating these two entities is important in prognostication and guiding further medical intervention. This can be difficult in the acute setting after hypoxic brain injury but the use of neurophysiologic studies may be helpful. In this article, we present a case of a patient who presented after pulseless electrical activity arrest, underwent targeted temperature management and subsequently developed Lance-Adams syndrome. The neurologic and electroencephalographic findings in Lance-Adams syndrome are discussed with an updated review.

Rationale: The cytosolic protease calpain has been recently implicated in the vascular remodeling of angiotensin II (Ang II) type 1 receptor (AT 1 R) signaling. The role of Ang II/AT 1 R/calpain signaling on endothelial function, an important and early determinant of vascular pathology, remains though totally unknown. Accordingly, we investigated the role of calpain in the endothelial dysfunction of Ang II. Objective: To demonstrate a mechanistic role for calpain in the endothelial dysfunction induced by Ang II/AT 1 R signaling. To establish endothelial-expressed calpains as an important target of AT 1 R signaling. Methods and Results: Subchronic administration of nonpressor doses of Ang II to rats and mice significantly increased vascular calpain activity via AT 1 R signaling. Intravital microscopy studies revealed that activation of vascular expressed calpains causes endothelial dysfunction with increased leukocyte–endothelium interactions and albumin permeability in the microcirculation. Western blot and immunohistochemistry studies confirmed that Ang II/AT 1 R signaling preferentially activates the constitutively expressed μ-calpain isoform and demonstrated a calpain-dependent degradation of IκBα, along with upregulation of nuclear factor κB–regulated endothelial cell adhesion molecules. These physiological and biochemical parameters were nearly normalized following inhibition of AT 1 R or calpain in vivo. RNA silencing studies in microvascular endothelial cells, along with knockout and transgenic mouse studies, further confirmed the role of μ-calpain in the endothelial adhesiveness induced by Ang II. Conclusions: This study uncovers a novel role for calpain in the endothelial dysfunction of Ang II/AT 1 R signaling and establishes the calpain system as a novel molecular target of the vascular protective action of renin–angiotensin system inhibition. Our results may have significant clinical implications in vascular disease.

The report by Beekman et al.1 highlighted the difficulties surrounding prognostication after cardiac arrest, particularly regarding posthypoxic myoclonus (PHM). We reviewed the literature on PHM and Lance-Adams syndrome (LAS) and their utility in prognostication after cardiac arrest.2–4 Despite the authors' statement that LAS is distinguished by “the early onset of myoclonic status epilepticus (MSE),”1 historical definitions actually described LAS (“good” PHM) occurring after 24 hours postarrest, and MSE (“bad” PHM) more acutely. We found many cases of acute PHM associated with good outcomes, and that LAS may occur as early as 5 hours postarrest.4 This demonstrates the possible confusion defining PHM using time of onset.

Background: Although previous research has demonstrated that patients with posttraumatic migraine (PTM) after concussion report more symptoms and cognitive deficits after injury than do those without PTM, it is not known whether these effects persist beyond the first week of injury or whether PTM predicts recovery time. Purpose: To determine whether PTM during the first week after injury predicts (1) cognitive impairment and symptoms in the second week after injury and (2) overall recovery time. Study Design: Cohort study; Level of evidence, 2. Methods: Participants were 138 male high school football players with a mean age of 15.96 years (SD, 1.18 years; range, 13-19 years). They were classified into 3 groups: PTM (headache, nausea, and photosensitivity or phonosensitivity), headache (headache without the other PTM symptoms), or no headache (no headache or PTM symptoms). The Immediate Post-concussion Assessment and Cognitive Test (ImPACT) was used to assess cognitive performance and symptoms at baseline, postinjury days 1-7, and postinjury days 8-14. Recovery time data were collected from medical records. Results: The PTM group performed worse on verbal memory than did the headache group at 8-14 days after injury. The PTM group performed worse on visual memory, reaction time, and symptoms than did the other groups at 1-7 days and 8-14 days after injury. The PTM group was 7.3 times (95% confidence interval, 1.80-29.91) more likely to have protracted recovery (>20 days) than the no headache group and 2.6 times (95% confidence interval, 1.10-6.54) more likely than the headache group. Conclusion: Results suggest that PTM is associated with cognitive impairments and protracted recovery and that headache alone is not a good predictor of recovery.