Alcohol-related posterior reversible encephalopathy syndrome: a case report of a patient managed with a benzodiazepine-sparing regimen for alcohol withdrawal.

We report a case of posterior reversible encephalopathy syndrome (PRES) during treatment for alcohol withdrawal syndrome with gabapentin and clonidine. The patient developed severe hypertension, confusion and tremor, culminating in bilateral vision loss and a seizure. Imaging revealed posterior cerebral edema. Treatment with benzodiazepines, antihypertensives, and anti-seizure medications led to resolution. One year later, imaging showed resolution of the findings. We review the associated literature and propose the recognition of a PRES sub-entity, Alcohol-Related PRES (ARPRES), which can appear in the setting of alcohol withdrawal syndrome, chronic alcohol use, and acute alcohol intoxication, with or without hypertension.

The spectrum of Epstein-Barr virus infections of the central nervous system after organ transplantation.

BACKGROUND: Epstein-Barr virus (EBV)-related neurologic complications have a diverse presentation in transplant recipients, creating diagnostic and therapeutic challenges for clinicians. In this case series, we report unique manifestations of EBV related neurologic complications following solid organ transplant and highlight pitfalls in management. CASE PRESENTATIONS: A retrospective search of the electronic medical record of all patients from January 2015 to December 2020 who underwent solid organ transplantation and had central nervous system complications as determined by ICD-10 codes were included. Three patients with unique manifestation of EBV-related neurologic complications after liver transplantation were identified. The first was a 52-year-old man with a live-donor liver transplant 11 years prior for Budd-Chiari syndrome presented with several weeks of headache and several lesions on brain MRI; he was diagnosed with primary central nervous system post-transplant lymphoproliferative disorder. The second patient was a 63-year-old man with a deceased-donor liver transplant 16 years prior for alpha-1-antitrypsin deficiency and was found to have a stroke; he was diagnosed with EBV encephalitis. The final patient was a 75-year-old woman with a deceased-donor liver transplant six years prior for primary biliary cirrhosis who presented with four months of gait instability; she was diagnosed with EBV myelitis. A review of the literature was performed to supplement description of the different diseases. CONCLUSIONS: EBV-related central nervous infection in post-transplant patients can manifest in a variety of neurologic syndromes, which can be challenging to diagnose. Careful correlation of clinical, pathologic, and radiologic findings and a high index of suspicion are crucial in identification and appropriate management.

Development of Neurological Emergency Simulations for Assessment: Content Evidence and Response Process.

OBJECTIVE: To document two sources of validity evidence for simulation-based assessment in neurological emergencies. BACKGROUND: A critical aspect of education is development of evaluation techniques that assess learner's performance in settings that reflect actual clinical practice. Simulation-based evaluation affords the opportunity to standardize evaluations but requires validation. METHODS: We identified topics from the Neurocritical Care Society's Emergency Neurological Life Support (ENLS) training, cross-referenced with the American Academy of Neurology's core clerkship curriculum. We used a modified Delphi method to develop simulations for assessment in neurocritical care. We constructed checklists of action items and communication skills, merging ENLS checklists with relevant clinical guidelines. We also utilized global rating scales, rated one (novice) through five (expert) for each case. Participants included neurology sub-interns, neurology residents, neurosurgery interns, non-neurology critical care fellows, neurocritical care fellows, and neurology attending physicians. RESULTS: Ten evaluative simulation cases were developed. To date, 64 participants have taken part in 274 evaluative simulation scenarios. The participants were very satisfied with the cases (Likert scale 1-7, not at all satisfied-very satisfied, median 7, interquartile range (IQR) 7-7), found them to be very realistic (Likert scale 1-7, not at all realistic-very realistic, median 6, IQR 6-7), and appropriately difficult (Likert scale 1-7, much too easy-much too difficult, median 4, IQR 4-5). Interrater reliability was acceptable for both checklist action items (kappa = 0.64) and global rating scales (Pearson correlation r = .70). CONCLUSIONS: We demonstrated two sources of validity in ten simulation cases for assessment in neurological emergencies.

Neurocritical Care Education During Residency: Opinions (NEURON) Study.

BACKGROUND: The American Academy of Neurology (AAN) has established a core curriculum of topics for residency training in neurocritical care. At present there is limited data evaluating neurology residency education within the neurological intensive care unit. This study evaluates learner concerns with the neurological intensive care unit. METHODS: The Communication Committee and Resident & Fellow Taskforce within the Neurocritical Care Society (NCS) developed an online survey that consisted of 20 selection and free-text based questions. The survey was distributed to NCS members and then to neurology residency program directors. Statistical analysis of neurocritical care exposure were completed with t or Fisher exact test with p-value <0.05 considered significant. RESULTS: A total of 95 individuals from 32 different residency programs (36.5 % response rate) responded to the questionnaire. Most individuals train with neurocritical care attendings, fellows and advanced practitioners and have neurocritical care exposure during multiple years of residency training. 54 % of responders cite improvement in education as a means to improve neurocritical care training. Those that raised concern had no difference in time in the neurocritical care unit (9.4 weeks vs 8.8 weeks), exposure to trained neurointensivists, neurocritical care fellows or advanced providers (p value 0.53, 0.19, 0.83, respectively). CONCLUSIONS: There is significant learner concern regarding education within the neurointensive care unit. Although there are educational guidelines and focused neurocritical care educational materials, these alone do not satisfy residents' educational needs. This study demonstrates the need for educational changes, but it does not assess best strategies nor curricular content.

Development and Assessment of a Computer Algorithm for Stroke Vascular Localization Using Components of the National Institutes of Health Stroke Scale.

BACKGROUND: The National Institutes of Health Stroke Scale (NIHSS) was not intended to be used to determine the stroke's vascular distribution. The aim of this study was to develop, assess the reliability, and validate a computer algorithm based on the NIHSS for this purpose. METHODS: Two cohorts of patients with ischemic stroke having similar distributions of Oxfordshire localizations (total anterior, partial anterior, lacunar, and posterior circulation) based on neuroimaging were identified. The first cohort (n = 40) was used to develop a computer algorithm for vascular localization using a modified version of the NIHSS (NIHSS-Localization [NIHSS-Loc]) that included the laterality of selected deficits; the second (n = 20) was used to assess the reliability of algorithm-based localizations compared to those of 2 vascular neurologists. The validity of the algorithm-based localizations was assessed in comparison to neuroimaging. Agreement was assessed using the unweighted kappa (κ) statistic. RESULTS: Agreement between the 2 raters using the standard NIHSS was slight to moderate (κ = .36, 95% confidence interval [CI] .10-.61). Inter-rater agreement significantly improved to the substantial to almost perfect range using the NIHSS-Loc (κ = .88, 95% CI .73-1.00). Agreement was perfect when the 2 raters entered the data into the NIHSS-Loc computer algorithm (κ = 1.00, 95% CI 1.00-1.00). Agreement between the algorithm localization and neuroimaging results was fair to moderate (κ = .59, 95% CI .35-.84) and not significantly different from the localizations of either rater using the NIHSS-Loc. CONCLUSION: A computerized, modified version of the standard NIHSS can be used to reliably and validly assign the vascular distribution of an acute ischemic stroke.

West Nile Virus (WNV) Infection-Associated Acute Flaccid Paralysis With Ophthalmoplegia.

Infection with West Nile virus (WNV) is often characterized by a mild febrile illness, but it can progress to meningitis, encephalitis, flaccid paralysis, and respiratory failure. The neuro-ophthalmological manifestations of this disease are uncommonly discussed. This case describes a 49-year-old undomiciled male who developed WNV flaccid paralysis with ophthalmoplegia. His symptoms began with difficulty in walking and progressed over several days to flaccid paralysis and ophthalmoplegia. Cerebrospinal fluid was positive for WNV immunoglobulin M antibodies and electromyography demonstrated acute denervation in several muscle groups. This is an unusual case of neuro-invasive WNV presenting with flaccid paralysis and ophthalmoplegia.

Gummatous Neurosyphilis With Transient Worsening of Neurological Symptoms After Treatment Initiation.

Cerebral syphilitic gumma is an atypical presentation of neurosyphilis, the clinical manifestations of which depend on the size and location of the lesions. It radiologically presents as enhancing nodular lesion(s) in brain parenchyma. We present a case of a patient with cerebral syphilitic gummas who had worsening neurological symptoms a few hours after initiation of anti-syphilitic antibiotic treatment. We aim to illustrate the clinical and radiological characteristics that might be helpful to clinicians when approaching the challenges they might encounter while treating neurosyphilis.

Single institution experience with death by neurological criteria/brain death guideline adherence.

OBJECTIVE: To determine the effect on adherence to an institutional death by neurological criteria/brain death (DNC/BD) policy of implementation of a standardized DNC/BD checklist in the electronic medical record (EMR). METHODS: The retrospective study cohort included all patients admitted to our institution who were declared dead by neurologic criteria determined by ICD code (G93.82) between June 2015 and October 2019. Two investigators independently reviewed each case for adherence with institutional policy, and agreement was assessed using unweighted kappa statistics. Patient data and adherence to institutional policy before and after implementation of a standardized DNC/BD checklist were compared. RESULTS: There were 66 patients identified by the initial search and 38 were included in the final analysis, with 19 cases in both the pre- and post- checklist periods. There were no significant differences in age, cause of DNC/BD, time to DNC/BD determination, potential toxic, metabolic, physiologic confounders, or use of ancillary testing. The pre-checklist period adherence was 47.4% (n = 9/19) versus 94.6% (n = 18/19; p = 0.001) in the post-checklist EMR DNC/BD period. CONCLUSION: Implementation of a standardized EMR checklist substantially improved DNC/BD policy adherence in our institution. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence on the use of standardized EMR checklist to improve death by neurologic criteria/brain death policy adherence.

Association of Donor Brain Death Due to Stroke With Prognosis After Heart Transplantation.

BACKGROUND: The proximate cause of donor brain death is not considered a conventional risk factor in modern heart transplantation. OBJECTIVES: This study aimed to investigate the effect of the cause of donor brain death on recipients. METHODS: Using the United Network for Organ Sharing registry, long-term mortality and allograft failure were compared in recipients who underwent heart transplantation in the United States from 2005 through 2018 between allograft recipients from donors with stroke as the cause of brain death (n = 3,761) vs nonstroke causes (n = 14,677). Inverse probability weighting was used for risk adjustment. Interactions were investigated between the cause of brain death and other conventional donor risk factors for recipient mortality. RESULTS: There was an interaction between the cause of brain death and donor age (Pinteraction = 0.008). When allografts were procured from donors aged 40 years or younger, stroke as the cause of brain death was associated with an increased risk of mortality (23% vs 19% at 5 years; HR: 1.17; 95% CI: 1.02-1.35) and allograft failure (HR: 1.30; 95% CI: 1.04-1.63). When donors were older than 40 years, the cause of brain death was not associated with outcomes. CONCLUSIONS: As the cause of donor brain death, stroke had a substantially different effect on recipient and allograft survival depending on donor age. In the case of younger donor ages, stroke was associated with higher recipient mortality and allograft failure than other causes of brain death. The strength of this association decreased with increasing donor age such that the increased hazard was no longer present in donors older than approximately 40 years.

Simulation-based assessment of trainee's performance in post-cardiac arrest resuscitation.

Objectives: To assess trainees' performance in managing a patient with post-cardiac arrest complicated by status epilepticus. Methods: In this prospective, observational, single-center simulation-based study, trainees ranging from sub interns to critical care fellows evaluated and managed a post cardiac arrest patient, complicated by status epilepticus. Critical action items were developed by a modified Delphi approach based on American Heart Association guidelines and the Neurocritical Care Society's Emergency Neurological Life Support protocols. The primary outcome measure was the critical action item sum score. We sought validity evidence to support our findings by including attending neurocritical care physicians and comparing performance across four levels of training. Results: Forty-nine participants completed the simulation. The mean sum of critical actions completed by trainees was 10/21 (49%). Eleven (22%) trainees verbalized a differential diagnosis for the arrest. Thirty-two (65%) reviewed the electrocardiogram, recognized it as abnormal, and consulted cardiology. Forty trainees (81%) independently decided to start temperature management, but only 20 (41%) insisted on it when asked to reconsider. There was an effect of level of training on critical action checklist sum scores (novice mean score [standard deviation (SD)] = 4.8(1.8) vs. intermediate mean score (SD) = 10.4(2.1) vs. advanced mean score (D) = 11.6(3.0) vs. expert mean score (SD) = 14.7(2.2)). Conclusions: High-fidelity manikin-based simulation holds promise as an assessment tool in the performance of post-cardiac arrest care.

Acute Traumatic Spinal Cord Injury.

Traumatic spinal cord injury is a common neurologic insult worldwide that can result in severe disability. Early stabilization of the patient's airway, breathing, and circulation as well as cervical and thoracolumbar spinal immobilization is necessary to prevent additional injury and optimize outcomes. Computed tomography (CT) scan and magnetic resonance imaging (MRI) of the spinal column can assist with determining the extent of bony and ligamentous injury, which will guide surgical management. With or without surgical intervention, patients with spinal cord injury require intensive care unit management and close observation to monitor for potential complications.

Reversible Encephalopathy Due to Venous Hypertension From Arteriovenous Hemodialysis Graft.

Venous congestive encephalopathy is a rare complication in patients with arteriovenous hemodialysis grafts. It commonly manifests as encephalopathy of fluctuating severity, often with seizures. Because these patients typically have multiple significant chronic health problems, venous hypertension's contribution to the patient's cognitive decline can easily be overlooked. This nonspecific presentation can make diagnosis challenging, therefore delaying treatment. We describe a case of progressive, fluctuating encephalopathy with seizures due to cerebral venous congestion caused by arterial shunting from an upper limb arteriovenous (AV) fistula to the proximal venous system, that was initially unrecognized, yet ultimately reversed by elimination of the source of venous hypertension.

Movement Disorder Emergencies.

Acute presentation of new movement disorders and acute decompensation of chronic movement disorders are uncommon but potentially life-threatening. Inadvertent or purposeful overdose of many psychiatric medications can result in acute life-threatening movement disorders including serotonin syndrome, neuroleptic malignant syndrome, and malignant catatonia. Early withdrawal of potentiating medications, treatment with benzodiazepines and other diagnosis-specific drugs, and providing appropriate supportive care including airway and breathing management, hemodynamic stabilization, fluid resuscitation, and renal support including possible hemodialysis are the mainstays of acute management. Many of these conditions require admission to the neurologic intensive care unit.

Acute Myelopathy: Vascular and Infectious Diseases.

Vascular and infectious causes are rare but important causes of spinal cord injury. High suspicion for these processes is necessary, as symptoms may progress over hours to days, resulting in delayed presentation and diagnosis and worse outcomes. History and clinical examination findings can assist with localization of the affected vascular territory and spinal level, which will assist with focusing spinal imaging. Open and/or endovascular surgical management depends on the associated vascular abnormality. Infectious myelopathy treatment consists of targeted antimicrobial therapy when possible, infectious source control, and again, close monitoring for systemic complications.

Metabolic values precluding clinical death by neurologic Criteria/Brain death: Survey of neurocritical care society physicians.

BACKGROUND: There are no established ranges for metabolic values prior to death by neurologic criteria/brain death determination (DNC/BD) and the thresholds required by institutional protocols and accepted by neurointensivists is unknown. METHODS: We designed a survey that addressed 1) the metabolic tests required in institutional guidelines prior to brain death determination, 2) the metabolic tests the respondent reviewed prior to brain death determination, and 3) the metabolic test thresholds for laboratory tests that were perceived to preclude or permit clinical DNC/BD determination. The survey was distributed online to physicians in the Neurocritical Care Society from September to December 2019. Respondents were dichotomized based on the number of brain death evaluations they had performed (≤20 vs. > 20) and responses were compared between groups. RESULTS: The survey was completed by 84 physicians. Nearly half (47.6%) of respondents did not believe their institutions required metabolic testing. The metabolic testing for which institutions most commonly provided a defined threshold were arterial pH (34.5%, 29/84), sodium (28.6%, 24/84), and glucose (15.5%, 13/84). Nearly all (97.6%) respondents routinely reviewed metabolic tests prior to brain death evaluation, the most common of which were: sodium (91.7%, 77/84), arterial pH (83.3%, 70/84), and glucose (79.8%, 67/84). Respondents who had performed > 20 evaluations were less likely to check thyroxine and total bilirubin (3.6%, 2/55 vs. 20.7%, 6/29 (p = 0.011) and 12.7%, 7/55 vs. 31%, 9/29 (p = 0.042), respectively), and had a more liberal upper limit of potassium (6.3 mEq/L vs 6.0 mEq/L, p = 0.045). CONCLUSION: Prior to brain death evaluation, neurocritical care providers commonly review similar metabolic tests and have similar thresholds regarding values that would preclude clinical brain death determination. This finding is independent of experience with brain death determination.

Simulation-Based Assessment of Graduate Neurology Trainees' Performance Managing Acute Ischemic Stroke.

BACKGROUND AND OBJECTIVES: Multidisciplinary acute stroke teams improve acute ischemic stroke management but may hinder trainees' education, which in turn may contribute to poorer outcomes in community hospitals on graduation. Our goal was to assess graduate neurology trainee performance independently of a multidisciplinary stroke team in the management of acute ischemic stroke, tissue plasminogen activator (tPA)-related hemorrhage, and cerebral herniation syndrome. METHODS: In this prospective, observational, single-center simulation-based study, participants (subinterns to attending physicians) managed a patient with acute ischemic stroke followed by tPA-related hemorrhagic conversion leading to cerebral herniation. Critical actions were developed by a modified Delphi approach based on relevant American Heart Association guidelines and the Neurocritical Care Society's Emergency Neurologic Life Support protocols. The primary outcome measure was graduate neurology trainees' critical action item sum score. We sought validity evidence to support our findings by comparing performance across 4 levels of training. RESULTS: Fifty-three trainees (including 31 graduate neurology trainees) and 5 attending physicians completed the simulation. The mean sum of critical actions completed by graduate neurology trainees was 15 of 22 (68%). Ninety percent of graduate neurology trainees properly administered tPA; 84% immediately stopped tPA infusion after patient deterioration; but only 55% reversed tPA according to guidelines. There was a moderately strong effect of level of training on critical action sum score (level 1 mean [SD] score 7.2 [2.8] vs level 2 mean [SD] score 12.3 [2.6] vs level 3 mean [SD] score 13.3 [2.2] vs level 4 mean [SD] score 16.3 [2.4], p < 0.001, R 2 = 0.54). DISCUSSION: Graduate neurology trainees reassuringly perform well in initial management of acute ischemic stroke but frequently make errors in the treatment of hemorrhagic transformation after thrombolysis, suggesting the need for more education surrounding this low-frequency, high-acuity event. High-fidelity simulation holds promise as an assessment tool for acute stroke management performance.

Hyponatremia in the Neurologically Ill Patient: A Review.

Hyponatremia is a well-known disorder commonly faced by clinicians managing neurologically ill patients. Neurological disorders are often associated with hyponatremia during their acute presentation and can be associated with specific neurologic etiologies and symptoms. Patients may present with hyponatremia with traumatic brain injury, develop hyponatremia subacutely following aneurysmal subarachnoid hemorrhage, or may manifest with seizures due to hyponatremia itself. Clinicians caring for the neurologically ill patient should be well versed in identifying these early signs, symptoms, and etiologies of hyponatremia. Early diagnosis and treatment can potentially avoid neurologic and systemic complications in these patients and improve outcomes. This review focuses on the causes and findings of hyponatremia in the neurologically ill patient and discusses the pathophysiology, diagnoses, and treatment strategies for commonly encountered etiologies.

Toxin-Induced Subacute Encephalopathy.

Subacute toxic encephalopathies are challenging to identify due to their often insidious tempo of evolution, nonspecific manifestations, relative infrequency as individual entities, and frequent lack of specific diagnostic testing. Yet they are crucial to recognize-in aggregate, subacute toxic encephalopathies are a common problem that can lead to severe, irreversible harm if not diagnosed and treated efficiently. This article reviews the clinically relevant aspects of some of the more important subacute toxic encephalopathy syndromes caused by inorganic toxins, carbon monoxide, antibiotics, antineoplastic agents, and psychiatric medications.