Improving Outcome in Severe Myasthenia Gravis and Guillain-Barré Syndrome.

When progressive and severe, myasthenia gravis and Guillain-Barré syndrome may have the potential for fatal and unfavorable clinical outcomes. Regardless of important differences in their clinical course, the development of weakness of oropharyngeal muscles and respiratory failure with requirement of mechanical ventilation is the main driver of poor prognosis in both conditions. The need for prolonged mechanical ventilation is particularly relevant because it immobilizes the patient and care becomes extraordinarily complex due to daily risks of systemic complications. Additionally, patients with myasthenia gravis often require long-term immunosuppressive treatments with associated toxicity and infectious risks. Unlike myasthenia gravis, the recovery period is prolonged in Guillain-Barré syndrome, but often favorable, even in the more severely affected patients. Outcome, for a large part, is determined by expert neurocritical care.

Aneurysmal Subdural Hematoma: A Systematic Review.

BACKGROUND: Aneurysmal subdural hematoma (aSDH) is a rare complication of aneurysm rupture, affecting between 0.5 and 7.9% of patients with aneurysmal subarachnoid hemorrhage (aSAH). The clinical presentation, course, and outcomes of these patients are largely unknown. OBJECTIVE: This study aims to systematically review the literature to evaluate the demographics, clinical presentation, aneurysm location, treatment options, and outcomes of patients with aSDH with and without aSAH. METHODS: In accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, we conducted a systematic review of three databases (PubMed, EMBASE, and Google Scholar). From identified reports, we extracted data on patients' demographics, clinical presentation, imaging findings, surgical interventions, and clinical outcomes. We compared clinical outcomes, need for surgical treatment, and aneurysm location between patients with aSDH with and without concurrent aSAH using χ2 and Fisher's exact tests. We used simple and multivariable logistic regression models to further examine the association between the presence of aSAH and surgical treatment with clinical outcomes. RESULTS: We identified 112 articles with a total of 270 patients (70% women, mean age 52.8 [± 15.5] years). The most common aneurysm locations were the middle cerebral artery, followed by the posterior communicating artery, and the internal carotid artery. Patients with isolated aSDH fully recovered more frequently than those with concomitant aSAH (38% vs. 6%). The presence of aSAH increased the odds of unfavorable outcome (odds ratio [OR] 2.68, 95% confidence interval [CI] 1.34-5.37). Surgical treatment was inversely associated with unfavorable outcome in the univariable (OR 0.48, 95% CI 0.28-0.84) but not in the multivariable analysis (OR 0.76, 95% CI 0.35-1.66). CONCLUSION: aSDH occurs infrequently. Simultaneous presence of both aSDH and aSAH from an aneurysmal source is associated with poor outcomes. Surgical treatment is associated with lower rates of unfavorable outcomes including death and severe disability.

Perfusion Deficits in Patients with Posterior Reversible Encephalopathy Syndrome: a Retrospective, Two-Center Study.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is manifested by acute neurological symptoms in patients with varied predisposing factors and characteristic findings on brain imaging. Cerebrovascular autoregulation is thought to be altered in PRES. However, it remains unclear whether cerebral hypoperfusion or hyperperfusion is the initiating event. We aimed to describe the brain perfusion status in untreated patients with PRES. METHODS: Patients with PRES who underwent cerebral perfusion studies on presentation were retrospectively identified from (1) a prospective database of patients with PRES admitted to Saint Mary's Hospital, Mayo Clinic, Rochester from January 2005 to December 2021 and (2) University of Nebraska Medical Center electronic database from January 2010 to December 2021. Demographics, past medical history, presenting symptoms, cause of PRES, and clinical outcomes were recorded. Brain imaging studies were reviewed. We recorded the location of brain lesions, the time from symptoms onset to perfusion study, blood pressure at the time of the perfusion study, and blood pressure lowering treatments. RESULTS: Five patients (four women, median age 66 years) were included. Causes of PRES were acute hypertension (n = 3), perioperative blood pressure fluctuations, and treatment with pazopanib. Four patients had chronic hypertension. Presenting symptoms were encephalopathy (n = 5), focal neurological symptoms (n = 4), and seizures (n = 2). All patients underwent computed tomography (CT) perfusion performed within 12 h of symptoms onset. All but one patient was hypertensive at the time of CT perfusion. Scans showed diffuse cerebral hypoperfusion, more pronounced in the corona radiata and areas with brain edema. No patient had critical cerebral ischemia or arterial vasoconstriction on CT angiogram. CONCLUSIONS: Patients with PRES can have cerebral hypoperfusion despite severe hypertension. A perfusion study in the acute setting may be helpful to better understand the perfusion status and guide blood pressure treatment.

The Origin of the Burst-Suppression Paradigm in Treatment of Status Epilepticus.

After electroencephalography (EEG) was introduced in hospitals, early literature recognized burst-suppression pattern (BSP) as a distinctive EEG pattern characterized by intermittent high-power oscillations alternating with isoelectric periods in coma and epileptic encephalopathies of childhood or the pattern could be induced by general anesthesia and hypothermia. The term was introduced by Swank and Watson in 1949 but was initially described by Derbyshire et al. in 1936 in their study about the anesthetic effects of tribromoethanol. Once the EEG/BSP pattern emerged in the literature as therapeutic goal in refractory status epilepticus, researchers began exploring whether the depth of EEG suppression correlated with improved seizure control and clinical outcomes. We can conclude that, from a historical perspective, the evidence to suppress the brain to a BSP when treating status epilepticus is inconclusive.

Stroke Knowledge in the EstEPA Project, a Population-Based Study.

OBJECTIVES: Stroke knowledge is poor in the general population worldwide. Yet, data from Spanish-Speaking populations, particularly in Latin America, are scant. We aim to evaluate stroke awareness using personal interviews in a population-based study. MATERIALS AND METHODS: A questionnaire of stroke awareness was administered to a randomly selected sample of households. "Good stroke knowledge for action" was defined as recognition of impaired strength, sensation and language plus intention to seek urgent medical attention in a hypothetical stroke situation. Demographics, the term to name stroke, recognition of warning signs and attitude towards seeking medical attention were compared between individuals with and without "good stroke knowledge for action". RESULTS: From 1986 respondents (87%, median age 59 years [IQR 23], 50.7% female), most recognized stroke as ACV (cerebrovascular accident, [63%]). Weakness/decreased sensation were recognized as stroke warning signs by 83.5% of respondents, followed by aphasia (77.9%), incoordination (71.6%) and headache (70.5%). Chest pain was misclassified as stroke warning sign by 25% of subjects. In a hypothetical stroke situation, most respondents would go to the hospital (52.3%), or activate the EMS (39%). Individuals with a good stroke knowledge for action (63.5%) recognized visual symptoms (60.4% vs 43.8, p<0.0001), incoordination (78.8% vs 34.4%, p<0.0001) and headache (70.5% vs. 57.8%, p<0.0001) more frequently, and were less likely to misrecognize chest pain as stroke warning sign (23.8% vs. 28.9%, p=0.015). Neither, age (OR 1 CI 0.99-1.00, p=0.94), gender (OR 0.95, CI 0.79-1.16, p=0.61) or race (OR 1.17, CI 0.97-1.42, p=0.097) predicted good stroke knowledge for action. CONCLUSIONS: Most people recognize stroke as ACV. The recognition of stroke warning signs and the attitude towards seeking emergent medical attention appears acceptable. Yet, most respondents would go directly to the hospital avoiding the EMS.

Causes of Death in Status Epilepticus.

OBJECTIVES: To determine the causes of death in patients with status epilepticus. To analyze the relative contributions of seizure etiology, seizure refractoriness, use of mechanical ventilation, anesthetic drugs for seizure control, and medical complications to in-hospital and 90-day mortality, hospital length of stay, and discharge disposition. DESIGN: Retrospective cohort. SETTING: Single-center neuroscience ICU. PARTICIPANTS: Patients with status epilepticus were identified by retrospective search of electronic database from January 1, 2011, to December 31, 2016. INTERVENTIONS: Review of electronic medical records. MEASUREMENTS AND MAIN RESULTS: Demographics, clinical characteristics, treatments, and outcomes were collected. Univariable and multivariable logistic regression analysis were used to determine whether the use of anesthetic drugs, mechanical ventilation, Status Epilepticus Severity Score, refractoriness of seizures, etiology of seizures, or medical complications were associated with in-hospital, 90-day mortality or discharge disposition. Among 244 patients with status epilepticus (mean age was 64 yr [interquartile range, 42-76], 55% male, median Status Epilepticus Severity Score 3 [interquartile range, 2-4]), 24 received anesthetic drug infusions for seizure control. In-hospital and 90-day mortality rates were 9.2% and 19.2%, respectively. Death was preceded by withdrawal of life-sustaining treatment in 19 patients (86.3%) and cardiac arrest in three (13.7%). Only Status Epilepticus Severity Score was associated with in-hospital and 90-day mortality, whereas the use of anesthetic drugs for seizure control, mechanical ventilation, medical complications, etiology, and refractoriness of seizures were not. Hospital length of stay was longer in patients with medical complications (p = 0.0091), refractory seizures (p = 0.0077), and in those who required anesthetic drugs for seizure control (p = 0.0035). Patients who had refractory seizures were less likely to be discharged home (odds ratio, 0.295; CI, 0.143-0.608; p = 0.0009). CONCLUSIONS: In this cohort, death primarily resulted from the underlying neurologic disease and withdrawal of life-sustaining treatment and not from our treatment choices. Use of anesthetic drugs, medical complications, and mechanical ventilation were not associated with in-hospital and 90-day mortality.

A Population-Based Survey of Stroke Knowledge in Argentina: The SIFHON Study.

BACKGROUND AND OBJECTIVES: Information about stroke awareness in Latin America is scant. We conducted a large population survey in Argentina to assess stroke knowledge. METHODS: We distributed 110,000 multiple-choice anonymous questionnaires using the house distribution system of a bottled water dispensing company. The survey assessed demographic characteristics and stroke knowledge. RESULTS: A total of 12,710 surveys were returned (12%). Even though 95% of the respondents reported some prior information about stroke, only 37% had adequate knowledge based on prespecified criteria. The Spanish acronym for accidente cerebrovascular, was the most frequently identified name for stroke. Sixty nine percent of respondents were able to identify stroke main risk factors and only 29% knew about transient ischemic attacks. If a hypothetical scenario of stroke was presented, 63% knew the existence of a time-dependent treatment, 25% would call an ambulance, and 50% would go to an emergency room by own means. A lower degree of knowledge was present in young, single, and nonuniversity men. CONCLUSIONS: This study represents the largest stroke awareness survey in a Spanish-speaking population. There was good recognition of some basic facts of stroke. However, the population had poor knowledge of prevalence and severity of the disease, transient ischemic attacks, and treatment availability.

Neurological Prognostication After Cardiac Arrest in the Era of Target Temperature Management.

PURPOSE OF REVIEW: The purpose of this study is to provide an updated review on neurological prognostication in comatose patients after cardiac arrest in light of current targeted temperature management (TTM) strategies. RECENT FINDINGS: With improved pre-hospital and hospital care, death due to cardiac arrest is decreasing. Yet, most survivors have poor neurological outcomes. While TTM has demonstrated to improve neurological outcomes, it may cloud our prognostic accuracy. A multimodal approach is currently used to diminish prognostic uncertainty. The neurological examination remains the mainstay for prognosis after cardiac arrest. The combination electroencephalogram, somatosensory evoked potentials, and neuron-specific enolase improve prognostic accuracy, mostly in patients who underwent TTM. Quantitative analysis of pupillary reaction and EEG background variability, neuroimaging (CT perfusion and DWI-MRI), and middle/long-latency evoked potentials are promising methods that may further improve the precision of outcome prognostication.

Stroke Epidemiology in Argentina. Design of a Population-Based Study in General Villegas (EstEPA).

BACKGROUND: Epidemiological data on stroke is scarce in Latin America. Estudio Epidemiológico Poblacional sobre Accidente Cerebrovascular (EstEPA) is a population-based program planned to assess prevalence, incidence, mortality, and burden of disease for stroke in the Department of General Villegas, province of Buenos Aires, Argentina. METHODS AND DESIGN: Prevalence study will consist of a two-phase survey approach in the urban area of General Villegas. First, trained social workers with a structured questionnaire will collect data in 2000 randomly selected housing units. Those subjects screened positive for possible strokes will be interviewed and examined by stroke neurologists to confirm diagnosis. The incidence study will be performed according to the methodology of WHO STEPS stroke surveillance manual and will detect all new strokes in the department during a 5-year period. General and disease-specific mortality rates will be assessed monthly during a 5-year period, using different sources of information. To assess the overall burden of cerebrovascular disease, disability adjusted life years will be calculated. DISCUSSION: EstEPA will assess for the first time all aspects of stroke epidemiology in Argentina. Its results will help to implement population-based interventions and to properly allocate public health resources.

Renal Function and Risk Stratification of Patients With Embolic Stroke of Undetermined Source.

Background and Purpose- We aimed to assess if renal function can aid in risk stratification for ischemic stroke or transient ischemic attack (TIA) recurrence and death in patients with embolic stroke of undetermined source (ESUS). Methods- We pooled 12 ESUS datasets from Europe and America. Renal function was evaluated using the estimated glomerular filtration rate (eGFR) and analyzed in continuous, binary, and categorical way. Cox-regression analyses assessed if renal function was independently associated with the risk for ischemic stroke/TIA recurrence and death. The Kaplan-Meier product limit method estimated the cumulative probability of ischemic stroke/TIA recurrence and death. Results- In 1530 patients with ESUS followed for 3260 patient-years, there were 237 recurrences (15.9%) and 201 deaths (13.4%), corresponding to 7.3 ischemic stroke/TIA recurrences and 5.6 deaths per 100 patient-years, respectively. Renal function was not associated with the risk for ischemic stroke/TIA recurrence when forced into the final multivariate model, regardless if it was analyzed as continuous (hazard ratio, 1.00; 95% CI, 0.99-1.00 for every 1 mL/min), binary (hazard ratio, 1.27; 95% CI, 0.87-1.73) or categorical covariate (likelihood-ratio test 2.59, P=0.63 for stroke recurrence). The probability of ischemic stroke/TIA recurrence across stages of renal function was 11.9% for eGFR ≥90, 16.6% for eGFR 60-89, 21.7% for eGFR 45-59, 19.2% for eGFR 30-44, and 24.9% for eGFR <30 (likelihood-ratio test 2.59, P=0.63). The results were similar for the outcome of death. Conclusions- The present study is the largest pooled individual patient-level ESUS dataset, and does not provide evidence that renal function can be used to stratify the risk of ischemic stroke/TIA recurrence or death in patients with ESUS.

Outcomes in Patients With Severe West Nile Neuroinvasive Disease.

OBJECTIVE: To assess the long-term outcomes of patients hospitalized with severe West Nile neuroinvasive disease. DESIGN: Retrospective cohort. SETTING: Patients admitted to a referral center (Saint Mary's Hospital, Mayo Clinic). PARTICIPANTS: Twenty-six patients with West Nile neuroinvasive disease were identified by retrospective search of electronic database of Saint Mary's Hospital from January 1999 to November 2016. INTERVENTIONS: Retrospective electronic medical records review and prospective telephone follow-up. MEASUREMENTS AND MAIN RESULTS: Functional disability and cognitive outcomes were evaluated with the modified Rankin Scale and the Telephone Interview for Cognitive Status scores. Data on the time that the patient returned home after the hospitalization for West Nile neuroinvasive disease and the time of return to work were also collected. We identified 26 patients (81% males), 59 ± 17 years old. After a median hospital stay of 14.5 days (3-126), four patients died and 90% of survivors had a modified Rankin Scale of 3-5. Two additional patients died, and 80% of survivors had a modified Rankin Scale of 0-2 after a median follow-up of 73 months (1-144). Seven patients had cognitive impairment, which was severe in two of them. The combination of encephalitis and acute flaccid paralysis at presentation was associated with lower likelihood of returning home within 1 month after discharge (p < 0.01). Patients who required mechanical ventilation were more likely to have a modified Rankin Scale of 3-5 at last follow-up (p = 0.03), less likely to return home within 1 month of discharge (p < 0.01), less likely to return to their jobs (p < 0.01), and showed a trend toward having cognitive impairment (p = 0.05). CONCLUSIONS: Despite having poor outcomes at discharge, most West Nile neuroinvasive disease survivors with severe early disability can recover functional independence in the long term, justifying aggressive support during the acute phase and extensive rehabilitation efforts.

Systemic Complications Following Status Epilepticus.

PURPOSE OF REVIEW: Status epilepticus (SE) is a multisystem disorder. Initially, complications of a massive catecholamine release followed by the side effects of medical therapies, impact patients' outcomes. The aim of this article is to provide an updated summary of the systemic complications following SE. RECENT FINDINGS: In recent years, the importance of the multifaceted nature of SE and its relationship with clinical outcomes has been increasingly recognized. The cumulative systemic effects of prolonged seizures and their treatment contribute to morbidity and mortality in this condition. Most systemic complications after SE are predictable. Anticipating their occurrence and respecting a number of simple guidelines may improve the prognosis of these patients.

Critical West Nile Neuroinvasive Disease.

BACKGROUND: Data to guide neurointensivists seeing patients with West Nile Neuroinvasive disease (WNND) are lacking. We present a comparatively large series of patients with WNND admitted to the intensive care unit (ICU) and provide data on their early diagnosis, triage to the ICU and predictors of short-term outcomes. METHODS: We retrospectively identified patients aged ≥ 18 years old with WNND from January 1999 to November 2016. Demographic and clinical data, the modified Rankin Scale at discharge and disposition were collected. Univariate analysis was performed to find predictors of ICU admission and to assess the impact of ICU admission on the short-term outcomes. P values < 0.05 were considered significant. RESULTS: Among 26 patients, 16 were admitted to the ICU. Age < 60 years and the presentation with encephalitis and acute flaccid paralysis predicted ICU admission (P = 0.044 and 0.0007). Among patients requiring ICU admission, four died and no one was discharged home. ICU admission predicted longer hospital stay (P = 0.021), inhospital death (P = 0.034), survival with inability to walk independently (P = 0.0094), and discharge disposition other than home (P = 0.007). In the ICU group, older age was associated with longer hospital stay (P = 0.0001) and inhospital death (P = 0.035). CONCLUSION: WNND requiring ICU care has a high morbidity and mortality, especially among older patients. Survivors are highly disabled at discharge, but many improve over time. Therefore, more data on the long-term prognosis of survivors are needed to guide the goals of care in the acute setting.

Age may contribute to the increased frequency of axonal Guillain-Barré syndrome.

INTRODUCTION: The frequency of axonal Guillain-Barré syndrome (GBS) varies among countries. Previous studies supporting the high frequency of axonal GBS in South America have been carried out with pediatric populations. We seek to determine the frequency of axonal GBS in both children and adults in South America. METHODS: This is a retrospective cohort analysis of patients who were diagnosed with GBS between January 2006 and December 2013 in a neurological center in Buenos Aires, Argentina. Adults and children with a diagnosis of GBS were included and classified by applying Ho and colleagues' criteria1 for axonal GBS. RESULTS: The study included 105 patients with GBS. Among 58 adults, only 5 individuals were classified as axonal GBS compared with 16 of 47 children. The frequency of axonal GBS was significantly higher in children than in adults (34% vs. 8.6%, P = 0.0001). DISCUSSION: As shown in a cohort of South American patients, age may impact the frequency of axonal GBS. Muscle Nerve 56: 1311-1313, 2017.

Headache and Treatment of Unruptured Intracranial Aneurysms.

BACKGROUND AND PURPOSE: The relationship between unruptured intracranial aneurysms (UIAs) and chronic headache and the impact of aneurysm treatment on headache outcome are controversial. The aim of this study was to determine clinical features of a supposedly primary headache in patients with UIA. We also assessed changes in headache characteristics after UIA treatment. METHODS: We examined clinical and imaging data of patients in whom a UIA was diagnosed during diagnostic workup of a suspected primary headache. Medical records were reviewed and personal telephone follow-ups were performed after UIA treatment to assess changes in the frequency and intensity of the headache. RESULTS: Forty-two patients (76%) reported a substantial improvement in headache frequency and intensity after UIA treatment. Forty-five patients (81%) reported a decrease in headache frequency from a median of 8 days/month before treatment to 1 day/month after treatment (95% confidence interval [CI] 81-83, P < .001). The average intensity in an analog pain scale was 7.7 ± 1.6 before treatment and 5.6 ± 2.4 after treatment (P < .001). Higher headache frequency was associated with a greater odd of improvement after treatment (odds ratio 1.12, 95% CI 1.0-1.26, P = .03). No associations were found between the type of headache, type of treatment (endovascular versus surgical), number, size, or localization of the aneurysms and the response to treatment. CONCLUSIONS: The treatment of UIA had a robust beneficial effect on previous headache. Although a "placebo" effect of aneurysm treatment cannot be ruled out, these results suggest a potential association between UIA and certain chronic headaches usually considered to be primary.

Risk Stratification for Recurrence and Mortality in Embolic Stroke of Undetermined Source.

BACKGROUND AND PURPOSE: The risk of stroke recurrence in patients with Embolic Stroke of Undetermined Source (ESUS) is high, and the optimal antithrombotic strategy for secondary prevention is unclear. We investigated whether congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, and stroke or transient ischemic attack (TIA; CHADS2) and CHA2DS2-VASc scores can stratify the long-term risk of ischemic stroke/TIA recurrence and death in ESUS. METHODS: We pooled data sets of 11 stroke registries from Europe and America. ESUS was defined according to the Cryptogenic Stroke/ESUS International Working Group. Cox regression analyses were performed to investigate if prestroke CHADS2 and congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, stroke or TIA, vascular disease, age 65-74 years, sex category (CHA2DS2-VASc) scores were independently associated with the risk of ischemic stroke/TIA recurrence or death. The Kaplan-Meier product limit method was used to estimate the cumulative probability of ischemic stroke/TIA recurrence and death in different strata of the CHADS2 and CHA2DS2-VASc scores. RESULTS: One hundred fifty-nine (5.6% per year) ischemic stroke/TIA recurrences and 148 (5.2% per year) deaths occurred in 1095 patients (median age, 68 years) followed-up for a median of 31 months. Compared with CHADS2 score 0, patients with CHADS2 score 1 and CHADS2 score >1 had higher risk of ischemic stroke/TIA recurrence (hazard ratio [HR], 2.38; 95% confidence interval [CI], 1.41-4.00 and HR, 2.72; 95% CI, 1.68-4.40, respectively) and death (HR, 3.58; 95% CI, 1.80-7.12, and HR, 5.45; 95% CI, 2.86-10.40, respectively). Compared with low-risk CHA2DS2-VASc score, patients with high-risk CHA2DS2-VASc score had higher risk of ischemic stroke/TIA recurrence (HR, 3.35; 95% CI, 1.94-5.80) and death (HR, 13.0; 95% CI, 4.7-35.4). CONCLUSIONS: The risk of recurrent ischemic stroke/TIA and death in ESUS is reliably stratified by CHADS2 and CHA2DS2-VASc scores. Compared with the low-risk group, patients in the high-risk CHA2DS2-VASc group have much higher risk of ischemic stroke recurrence/TIA and death, approximately 3-fold and 13-fold, respectively.

Stroke knowledge in Spanish-speaking populations.

BACKGROUND: Spanish is the second most-spoken language in the world. Spanish-speaking populations (SSP) have heterogeneous cultural backgrounds, racial and ethnical origins, economic status, and access to health care systems. There are no published reviews about stroke knowledge in SSP. We reviewed the existing literature addressing stroke knowledge among SSP and propose here some future directions for research. SUMMARY: We identified 18 suitable studies by searching PubMed, Lilacs, Scopus, Embase, Cochrane and Scielo databases, and by looking at reference lists of eligible articles. We also included 2 conference abstracts. Data related to stroke knowledge from studies of Spanish-speakers were analyzed. Key Messages: Little is known about stroke knowledge in SSP, especially in Latin America. Information is lacking even among subjects at risk, stroke patients, stroke survivors, and health care providers. 'Ictus', the word used for stroke in Spanish, is largely unrecognized among subjects at risk. Furthermore, access to medical care and the availability of neurologists are suboptimal in many regions. There are several potential issues to solve regarding stroke knowledge and stroke care in SSP. Programs to educate the general population and non-neurologists medical providers in stroke and telemedicine may be better ways of improving the present situation.