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.

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.

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.

Blood Pressure Variability After Cerebrovascular Events: A Possible New Therapeutic Target: A Narrative Review.

Blood pressure variability, the variation of blood pressure during a certain period, results from the interaction of hemodynamic, neuronal, humoral, behavioral, and environmental factors. Cerebral autoregulation is impaired in acute cerebrovascular disease. Hence, increased blood pressure variability (BPV) may provoke or exacerbate secondary brain injury. In fact, available data showed that increased blood pressure variability is associated with worse outcomes after acute ischemic stroke, intracerebral hemorrhage, and aneurysmal subarachnoid hemorrhage. Consequently, BPV may represent a usual modifiable therapeutic target. This concept is particularly attractive because reduction of BPV can be feasible in regions with lower resources and can be applicable to patients with various forms of acute stroke. Prospective studies are needed to further clarify the relationship between BPV and secondary brain damage and the determinants of BPV in different clinical populations. Ultimately, cerebrovascular disease-specific randomized controlled trials aimed at reducing BPV, irrespective of the absolute blood pressure values, are needed to determine whether reduction of BPV can improve outcomes in patients with acute cerebrovascular disease.

Changes in the Care of Neurological Diseases During the First Wave of the COVID-19 Pandemic: A Single Private Center Study in Argentina.

Introduction: Healthcare systems are struggling to cope with the rapid evolution of the COVID-19 pandemic. In Argentina, the pandemic is advancing despite prolonged lockdown measures. We aim to analyze the impact of the easing of lockdown measures in the number of visits to the emergency department (ED), and outpatient consultations (OC) to a tertiary neurological center. Methods: We compared the number of ED visits with the social mobility overtime. We also compared the number of OC, and the geographic distribution of patients' addresses between 2019 and 2020. Results: ED visits decreased 48.33% (n = 14,697 in 2019 vs. n = 7,595 in 2020). At the beginning of the lockdown, the social mobility decreased in pharmacies/groceries, and workplaces, along with a reduction in the number of ED visits. With the easing of lockdown restrictions, the social mobility decreased in residential places, slightly increased in workplaces and almost return to normal in pharmacies/groceries. Variations in ED visits correlate better with social mobility in workplaces (coef. =0.75, p < 0.001) than in groceries/pharmacies (coef. =0.68, p < 0.001). OC decreased 43%. Fourteen percent of OC were tele consults. This was associated with an increase of the geographical area of influence of our center (standard distance of 109 km in 2019 and 127 km in 2020). Conclusions: Despite an increase in social mobility, the number of ED visits and OC to an Argentinian tertiary neurological center remain worrisomely low. The pandemic catalyzed the introduction of telemedicine in our country. This has also allowed patients from distant zones to gain access to specialized neurological care.

Acute Hypertensive Response in Patients With Acute Intracerebral Hemorrhage: A Narrative Review.

OBJECTIVE: To review the role of the acute hypertensive response in patients with intracerebral hemorrhage, current treatment options, and areas for further research. METHODS: Review of the literature to assess 1) frequency of acute hypertensive response in intracerebral hemorrhage; 2) consequences of acute hypertensive response in clinical outcomes; 3) acute hypertensive response and secondary brain injury: hematoma expansion and perihematomal edema; 4) vascular autoregulation, safety data side effects of acute antihypertensive treatment; and 5) randomized clinical trials and meta-analyses. RESULTS: An acute hypertensive response is frequent in patients with acute intracerebral hemorrhage and is associated with poor clinical outcomes. However, it is not clear whether high blood pressure is a cause of poor clinical outcome or solely represents a marker of severity. Although current guidelines recommend intensive blood pressure treatment (<140 mm Hg) in patients with intracerebral hemorrhage, 2 randomized clinical trials have failed to demonstrate a consistent clinical benefit from this approach, and new data suggest that intensive blood pressure treatment could be beneficial for some patients but detrimental for others. CONCLUSIONS: Intracerebral hemorrhage is a heterogenous disease, thus, a one-fit-all approach for blood pressure treatment may be suboptimal. Further research should concentrate on finding subgroups of patients more likely to benefit from aggressive blood pressure lowering, considering intracerebral hemorrhage etiology, ultra-early randomization, and risk markers of hematoma expansion on brain imaging.

Neurologic complications of rheumatic fever.

Sydenham chorea, also known as St. Vitus dance, is a major clinical criterion for the diagnosis of acute rheumatic fever. Clinically, it results in a combination of movement disorders and complex neuropsychiatric symptoms. Cardiac damage due to rheumatic fever may also predispose to neurologic complications later in life. Rheumatic heart disease (RHD) is associated with heart remodeling, cardiac arrhythmias, and ischemic stroke. Furthermore, chronically damaged heart valves are predisposed to infection. Septic brain embolism, a known complication of infective endocarditis, may result in brain ischemia, hemorrhage, and spread of the infection to the brain.

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.

A tool to identify patients with embolic stroke of undetermined source at high recurrence risk.

OBJECTIVE: A tool to stratify the risk of stroke recurrence in patients with embolic stroke of undetermined source (ESUS) could be useful in research and clinical practice. We aimed to determine whether a score can be developed and externally validated for the identification of patients with ESUS at high risk for stroke recurrence. METHODS: We pooled the data of all consecutive patients with ESUS from 11 prospective stroke registries. We performed multivariable Cox regression analysis to identify predictors of stroke recurrence. Based on the coefficient of each covariate of the fitted multivariable model, we generated an integer-based point scoring system. We validated the score externally assessing its discrimination and calibration. RESULTS: In 3 registries (884 patients) that were used as the derivation cohort, age, leukoaraiosis, and multiterritorial infarct were identified as independent predictors of stroke recurrence and were included in the final score, which assigns 1 point per every decade after 35 years of age, 2 points for leukoaraiosis, and 3 points for multiterritorial infarcts (acute or old nonlacunar). The rate of stroke recurrence was 2.1 per 100 patient-years (95% confidence interval [CI] 1.44-3.06) in patients with a score of 0-4 (low risk), 3.74 (95% CI 2.77-5.04) in patients with a score of 5-6 (intermediate risk), and 8.23 (95% CI 5.99-11.3) in patients with a score of 7-12 (high risk). Compared to low-risk patients, the risk of stroke recurrence was significantly higher in intermediate-risk (hazard ratio [HR] 1.78, 95% CI 1.1-2.88) and high-risk patients (HR 4.67, 95% CI 2.83-7.7). The score was well-calibrated in both derivation and external validation cohorts (8 registries, 820 patients) (Hosmer-Lemeshow test χ2: 12.1 [p = 0.357] and χ2: 21.7 [p = 0.753], respectively). The area under the curve of the score was 0.63 (95% CI 0.58-0.68) and 0.60 (95% CI 0.54-0.66), respectively. CONCLUSIONS: The proposed score can assist in the identification of patients with ESUS at high risk for stroke recurrence.

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.

Can we stop the stuttering in stroke? Interventions in 40 patients with acute lacunes.

BACKGROUND: Whether any treatment can stop fluctuations of stuttering lacunar syndromes (SLS) is unclear. Case reports have variably suggested effectiveness of intravenous thrombolysis, dual antiplatelet treatment, blood pressure augmentation and anticoagulation. We aim to describe our experience with different treatments used in in patients presenting with SLS and their effect on clinical fluctuations and functional outcome. METHODS: We collected demographic and clinical data of consecutive adult patients with SLS. Descriptive summaries were reported as median and inter-quartile range (IQR) for continuous variables and as frequencies and percentages for categorical variables. RESULTS: Forty patients (72 ±â€¯10 years, 36% female) were included. Pure motor syndrome (57%) was the most frequent clinical presentation. Clinical fluctuations stopped and the improvement was temporally related to aspirin-clopidogrel in 11/17 cases, intravenous thrombolysis in 4/6 cases, blood pressure augmentation in 1/3 cases and aspirin in 1/7 cases. Two patients continued fluctuating after IVT and later responded to blood pressure augmentation (n = 1) or aspirin-clopidogrel (n = 1). CONCLUSIONS: Aspirin plus clopidogrel may be followed by clinical improvement when intravenous thrombolysis is not an option. Blood pressure augmentation may beneficial as ad-on treatment in patients with labile blood pressure.

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.