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This article characterizes 2 cases of longitudinally extensive transverse myelitis (LETM) that did not respond to immunotherapy and were diagnosed by biopsy as primary central nervous system (CNS) malignancies. Diffuse H3 K27M-mutant glioma is a recently described entity with very few cases of isolated spinal disease described in adults. Primitive neuroectodermal tumor is similarly uncommon in the spinal cord. Malignancies should be considered in patients who fail to improve with immunomodulatory therapy. We believe the experiences of our center will raise awareness about that point, broaden the existing understanding of the diagnostic approach to LETM, and highlight the need for additional studies.

Objective The aim of this study was to describe the natural history of individuals with congenital HIV who develop JC virus (JCV) infection of the central nervous system (CNS). Methods We retrospectively evaluated individuals with congenital HIV who met criteria for progressive multifocal leukoencephalopathy (PML) or JCV granule cell neuronopathy (JCV GCN) at three major healthcare centres in the northeast USA. Data on adherence to combined antiretroviral therapy (cART), neurologic symptoms, serum markers of immunity and HIV infection, cerebrospinal fluid (CSF) analyses, radiographic features, modified Rankin Scale (mRS) scores and survival were collected from the electronic medical record up to a censoring date of 1 August 2020. Results Among 10 adults with congenitally acquired HIV, nine were diagnosed with definitive PML and one was diagnosed with probable JCV GCN. Individuals presented at the time of their PML or JCV GCN diagnosis with a mean mRS of 2.0 (standard deviation 1.0). A premorbid mRS was documented for six patients and was zero in all cases. The most common risk factor was confirmed cART nonadherence in nine individuals. Five individuals with PML and one with JCV GCN died, with a latency from symptom onset to death of approximately 3 months for three individuals, and approximately 2 years for the remaining two. Conclusion Youth-adulthood transition is a high-risk point for dropping off from medical care. The study of this timepoint in people living with HIV could help inform effective care in these individuals.

ObjectiveTo characterize the breadth of neurologic findings associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in a diverse group of inpatients at an urban, safety-net US medical center.MethodsPatients were identified through an electronic medical record review from April 15, 2020, until July 1, 2020, at a large safety-net hospital in Boston, MA, caring primarily for underserved, low-income, and elderly patients. All hospitalized adult patients with positive nasopharyngeal swab or respiratory PCR testing for SARS-CoV-2 during their hospitalization or in the 30 days before admission who received an inpatient neurologic or neurocritical care consultation or admission during the study period were enrolled.ResultsSeventy-four patients were identified (42/57% male, median age 64 years). The majority of patients self-identified as Black or African American (38, 51%). The most common neurologic symptoms at presentation to the hospital included altered mental status (39, 53%), fatigue (18, 24%), and headache (18, 18%). Fifteen patients had ischemic strokes (20%). There were 10 in-hospital mortalities, with moderately severe disability among survivors at discharge (14%, median modified Rankin Scale score of 4).ConclusionsNeurologic findings spanned inflammatory, vascular pathologies, sequelae of critical illness and metabolic derangements, possible direct involvement of the nervous system by SARS-CoV-2, and exacerbation of underlying neurologic conditions, highlighting a broad range of possible etiologies of neurologic complications in patients with coronavirus disease 2019 (COVID-19). Further studies are needed to characterize the infectious and postinfectious neurologic complications of COVID-19 in diverse patient populations.

The COVID-19 pandemic has led to shifts in healthcare utilization for patients both with and without COVID-19. We aimed to determine how trends in neurology service admissions during the pandemic may aid in departmental planning by predicting future clinician staffing and other needs. We examined all admissions to the general neurology, stroke, and neurocritical care services from January 31 to May 16, 2020 at our tertiary-care hospital using an electronic health record query, comparing these to analogous data in 2019. We trended admission rates and projected future censuses using logarithmic regression, tracked changes in length of stay (LOS), and quantified shifts in presentations of specific diagnoses. Daily rates of admissions declined sharply during the week of March 13, 2020 (the week after pandemic status was declared by the World Health Organization). On the censoring date, we projected a return to pre-pandemic censuses in the week of June 21 and used this information to make decisions regarding neurology resident schedules. There was a trend toward increased LOS for general neurology and stroke patients between March 27 and April 9, 2020 compared to in 2019, with subsequent decline coinciding with early hospital initiatives. Since March 13, 2020, there has been a trend toward reduced presentations of ischemic stroke, suggesting a need for community education on stroke awareness. Characterizing early trends in neurology admissions may allow physician administrators to plan local and community-level responses to the pandemic.

Supplemental Digital Content is available in the text., Objectives: To describe the risk factors for and outcomes after myoclonus in a cohort of patients with coronavirus disease 2019. Design: Multicenter case series. Setting: Three tertiary care hospitals in Massachusetts, Georgia, and Virginia. Patients: Eight patients with clinical myoclonus in the setting of coronavirus disease 2019. Interventions & Measurements and Main Results: Outcomes in patients with myoclonus were variable, with one patient who died during the study period and five who were successfully extubated cognitively intact and without focal neurologic deficits. In five cases, the myoclonus completely resolved within 2 days of onset, while in three cases, it persisted for 10 days or longer. Seven patients experienced significant metabolic derangements, hypoxemia, or exposure to sedating medications that may have contributed to the development of myoclonus. One patient presented with encephalopathy and developed prolonged myoclonus in the absence of clear systemic provoking factors. Conclusions: Our findings suggest that myoclonus may be observed in severe acute respiratory syndrome coronavirus 2 infected patients, even in the absence of hypoxia. This association warrants further evaluation in larger cohorts to determine whether the presence of myoclonus may aid in the assessment of disease severity, neurologic involvement, or prognostication.

Introduction Coronavirus disease 2019 (COVID‐19) has rapidly become a global pandemic, but little is known about its potential impact on patients with myasthenia gravis (MG). Methods We studied the clinical course of COVID‐19 in five hospitalized patients with autoimmune MG (four with acetylcholine receptor antibodies, one with muscle‐specific tyrosine kinase antibodies) between April 1, 2020‐April 30‐2020. Results Two patients required intubation for hypoxemic respiratory failure, whereas one required significant supplemental oxygen. One patient with previously stable MG had myasthenic exacerbation. One patient treated with tocilizumab for COVID‐19 was successfully extubated. Two patients were treated for MG with intravenous immunoglobulin without thromboembolic complications. Discussion Our findings suggest that the clinical course and outcomes in patients with MG and COVID‐19 are highly variable. Further large studies are needed to define best practices and determinants of outcomes in this unique population.

PURPOSE OF REVIEW Both broadly immunosuppressive medications and selective immunomodulatory agents that act on particular components of the immune system are increasingly used in the treatment of neurologic and non-neurologic diseases. These therapies predispose patients to particular infections, some of which may affect the nervous system. Therefore, familiarity with the clinical and radiologic features of neurologic infections associated with specific immunomodulatory therapies is of importance for the practicing neurologist. This article reviews these neuroinfectious conditions, as well as other neurologic complications unique to transplant recipients and other patients who are immunocompromised. RECENT FINDINGS Diagnosis of infectious pathogens in patients who are immunocompromised may be particularly challenging because a decreased immune response can lead to atypical imaging or laboratory findings. Next-generation sequencing and other novel diagnostic modalities may improve the rate of early identification of neurologic infections in patients who are immunocompromised and ultimately ameliorate outcomes in this vulnerable population. SUMMARY A broad range of bacterial, viral, fungal, and parasitic infections of the nervous system can complicate solid organ and hematopoietic cell transplantation as well as other forms of immunocompromise. In addition to neurologic infections, such patients are at risk of neurotoxic and neuroinflammatory complications related to immunomodulatory and immunosuppressive therapies. Early recognition of infectious and noninfectious complications of immunocompromise is essential to guide appropriate treatment, which can include antimicrobial therapy and, in some cases, withdrawal of the predisposing medication with a transition to an alternative regimen.

Neurologic complications are common in immunocompromised patients, including those with advanced human immunodeficiency virus, transplant recipients, and patients on immunomodulatory medications. In addition to the standard differential diagnosis, specific pathogens and other conditions unique to the immunocompromised state should be considered in the evaluation of neurologic complaints in this patient population. A thorough understanding of these considerations is critical to the inpatient neurologist in contemporary practice, as increasing numbers of patients are exposed to immunomodulatory therapies. In this review, we provide a chief complaint-based approach to the clinical presentations and diagnosis of both infectious and noninfectious complications particular to immunocompromised patients.

Peripheral nerve disorders may be the presenting manifestation of a systemic infection, and early recognition of these syndromes is essential for prompt diagnosis and treatment. Mechanisms associated with infectious disorders of the peripheral nerve are often complex and multiple, with secondary inflammation, direct infectious invasion of nerves, and toxicities of antimicrobial medications playing a role. Here, we provide a complete review of the most common infectious pathogens that can affect the peripheral nerves.

Neuroinfectious diseases can affect immunocompetent and immunosuppressed individuals and cause a variety of emergencies including meningitis, encephalitis, and abscess. Neurologic infections are frequently complicated by secondary injuries that also present emergently such as cerebrovascular disease, acute obstructive hydrocephalus, and seizure. In most cases, timely recognition and early treatment of infection can improve the morbidity and mortality of infectious neurologic emergencies.

Background/Purpose: Coronavirus disease 2019 (COVID-19) is associated with increased risk of acute ischemic stroke (AIS), however, there is a paucity of data regarding outcomes after administration of intravenous tissue plasminogen activator (IV tPA) for stroke in patients with COVID-19. Methods: We present a multicenter case series from 9 centers in the United States of patients with acute neurological deficits consistent with AIS and COVID-19 who were treated with IV tPA. Results: We identified 13 patients (mean age 62 (±9.8) years, 9 (69.2%) male). All received IV tPA and 3 cases also underwent mechanical thrombectomy. All patients had systemic symptoms consistent with COVID-19 at the time of admission: fever (5 patients), cough (7 patients), and dyspnea (8 patients). The median admission NIH stroke scale (NIHSS) score was 14.5 (range 3–26) and most patients (61.5%) improved at follow up (median NIHSS score 7.5, range 0–25). No systemic or symptomatic intracranial hemorrhages were seen. Stroke mechanisms included cardioembolic (3 patients), large artery atherosclerosis (2 patients), small vessel disease (1 patient), embolic stroke of undetermined source (3 patients), and cryptogenic with incomplete investigation (1 patient). Three patients were determined to have transient ischemic attacks or aborted strokes. Two out of 12 (16.6%) patients had elevated fibrinogen levels on admission (mean 262.2 ± 87.5 mg/dl), and 7 out of 11 (63.6%) patients had an elevated D-dimer level (mean 4284.6 ±3368.9 ng/ml). Conclusions: IV tPA may be safe and efficacious in COVID-19, but larger studies are needed to validate these results.

Traumatic brain injuries are common among refugees and asylum-seekers and can result from a range of etiologies, including intimate partner violence, gang violence, war-related trauma, and torture. Regardless of the cause, these injuries often result in a host of neuropsychiatric and other symptoms that may complicate individuals’ subsequent health outcomes. For asylum-seekers, documenting prior head trauma is essential to the legal process, since traumatic brain injuries and their subsequent effects on memory and cognition may affect the ability to provide thorough testimony. Using three case vignettes, we explore how to approach the forensic evaluation of asylum-seekers with a history of traumatic brain injury, illustrating the range of etiologies and sequelae of traumatic brain injury in this complex population.

Introduction Early studies suggest that acute cerebrovascular events may be common in patients with coronavirus disease 2019 (COVID-19) and may be associated with a high mortality rate. Most cerebrovascular events described have been ischemic strokes, but both intracerebral hemorrhage and rarely cerebral venous sinus thrombosis (CVST) have also been reported. The diagnosis of CVST can be elusive, with wide-ranging and nonspecific presenting symptoms that can include headache or altered sensorium alone. Objective To describe the presentation, barriers to diagnosis, treatment, and outcome of CVST in patients with COVID-19. Methods We abstracted data on all patients diagnosed with CVST and COVID-19 from March 1 to August 9, 2020 at Boston Medical Center. Subsequently, we reviewed the literature and extracted all published cases of CVST in patients with COVID-19 from January 1, 2020 through August 9, 2020 and included all studies with case descriptions. Results We describe the clinical features and management of CVST in 3 women with COVID-19 who developed CVST days to months after initial COVID-19 symptoms. Two patients presented with encephalopathy and without focal neurologic deficits, while one presented with visual symptoms. All patients were treated with intravenous hydration and anticoagulation. None suffered hemorrhagic complications, and all were discharged home. We identified 12 other patients with CVST in the setting of COVID-19 via literature search. There was a female predominance (54.5%), most patients presented with altered sensorium (54.5%), and there was a high mortality rate (36.4%). Conclusions During this pandemic, clinicians should maintain a high index of suspicion for CVST in patients with a recent history of COVID-19 presenting with non-specific neurological symptoms such as headache to provide expedient management and prevent complications. The limited data suggests that CVST in COVID-19 is more prevalent in females and may be associated with high mortality.

To provide an approach to the evaluation and management of primary and secondary inflammatory vasculitides affecting the central nervous system (CNS). Although early descriptions of inflammatory CNS vasculitis report a universally high mortality rate, more recent cohorts demonstrate that favorable outcomes rely on early diagnosis and treatment. New and re-purposed medications have shown promise in the treatment of CNS vasculitides, and novel imaging modalities may aid in diagnosis. A broad array of diseases can affect CNS vasculature. Accurate diagnosis of CNS vasculitides requires a thorough exploration of systemic inflammatory diseases, as well as a clear understanding of the rare entities that cause vasculitides limited to the CNS.

Background Coronavirus Disease 2019 (COVID-19) has rapidly become a global pandemic, with over 1.8 million confirmed cases worldwide to date. Preliminary reports suggest that the disease may present in diverse ways, including with neurological symptoms, but few published reports in the literature describe seizures in patients with COVID-19. Objective The objective of the study was to characterize the risk factors, clinical features, and outcomes of seizures in patients with COVID-19. Methods This is a retrospective case series. Cases were identified through a review of admissions and consultations to the neurology and neurocritical care services between April 1, 2020 and May 15, 2020. Setting The study setting was in a tertiary care, safety-net hospital in Boston, MA. Participants Patients presenting with seizures and COVID-19 during the study period were included in the study. Results Seven patients met inclusion criteria (5 females, 71%). Patients ranged in age from 37 to 88 years (median: 75 years). Three patients had a prior history of well-controlled epilepsy (43%), while 4 patients had new-onset seizures, including 2 patients with prior history of remote stroke. Three patients had no preceding symptoms of COVID-19 prior to presentation (57%), and in all cases, seizures were the symptom that prompted presentation to the emergency department, regardless of prior symptoms of COVID-19. Conclusions Provoking factors for seizures in patients with COVID-19 may include metabolic factors, systemic illness, and possibly direct effects of the virus. In endemic areas with community spread of COVID-19, clinicians should be vigilant for the infection in patients who present with seizures, which may precede respiratory symptoms or prompt presentation to medical care. Early testing, isolation, and contact tracking of these patients can prevent further transmission of the virus.

To provide an approach to the evaluation and management of infectious vasculitides affecting the central nervous system (CNS). A growing number of infectious pathogens are associated with the development of cerebral vasculitis, though the pathophysiology is not well understood. Immunomodulatory drugs are being used as adjunctive therapies in specific conditions. A number of infectious diseases can affect CNS vasculature either as part of intracranial or systemic infection or due to involvement of the cerebrovasculature alone. Imaging is crucial to diagnosis. Serologic and cerebrospinal fluid (CSF) testing are often helpful, but the sensitivities of many tests are poor. Current treatment of infectious vasculitis typically requires targeted antimicrobial therapy, though often with adjunctive use of steroids. Very limited evidence suggests a role for antiplatelets. Here, we characterize the broad spectrum of infectious etiologies of CNS vasculitis and treatment modalities.

The coronavirus disease 2019 (COVID-19) pandemic has led to an acceleration of clinical information dissemination to unprecedented speeds, a phenomenon only partially explained by formal efforts of the scientific community. These have ranged from the establishment of open-source platforms for review of article preprints to the elimination of journal paywalls for COVID-19–related publications. In addition, informal efforts that rely on various modern media platforms that promote, repackage, and synthesize information have played substantial adjunctive roles, many of which did not exist during the severe acute respiratory syndrome pandemic of 2003. Although these latter efforts have greatly bolstered the speed of knowledge dissemination, their unregulated nature subjects them to risk for facilitating the spread of misinformation. In our opinion, the role of modern media in influencing clinical knowledge dissemination was not adequately examined even before the pandemic and therefore remains largely unchecked. In this article, we examine the spread of information in the field of COVID-19 and neurologic disorders, develop a simple model that maps various modern media tools on to the dissemination pipeline, and critically examine its components. Through this exercise, we identify opportunities for the scientific community to regulate and safeguard the clinical knowledge dissemination process, with implications both for the pandemic and beyond.

Background and objectives RCVS (Reversible Cerebral Vasoconstrictive Syndrome) is a condition associated with vasoactive agents that alter endothelial function. There is growing evidence that endothelial inflammation contributes to cerebrovascular disease in patients with coronavirus disease 2019 (COVID-19). In our study, we describe the clinical features, risk factors, and outcomes of RCVS in a multicenter case series of patients with COVID-19. Materials and methods Multicenter retrospective case series. We collected clinical characteristics, imaging, and outcomes of patients with RCVS and COVID-19 identified at each participating site. Results Ten patients were identified, 7 women, ages 21 – 62 years. Risk factors included use of vasoconstrictive agents in 7 and history of migraine in 2. Presenting symptoms included thunderclap headache in 5 patients with recurrent headaches in 4. Eight were hypertensive on arrival to the hospital. Symptoms of COVID-19 included fever in 2, respiratory symptoms in 8, and gastrointestinal symptoms in 1. One patient did not have systemic COVID-19 symptoms. MRI showed subarachnoid hemorrhage in 3 cases, intraparenchymal hemorrhage in 2, acute ischemic stroke in 4, FLAIR hyperintensities in 2, and no abnormalities in 1 case. Neurovascular imaging showed focal segment irregularity and narrowing concerning for vasospasm of the left MCA in 4 cases and diffuse, multifocal narrowing of the intracranial vasculature in 6 cases. Outcomes varied, with 2 deaths, 2 remaining in the ICU, and 6 surviving to discharge with modified Rankin scale (mRS) scores of 0 (n=3), 2 (n=2), and 3 (n=1). Conclusions Our series suggests that patients with COVID-19 may be at risk for RCVS, particularly in the setting of additional risk factors such as exposure to vasoactive agents. There was variability in the symptoms and severity of COVID-19, clinical characteristics, abnormalities on imaging, and mRS scores. However, a larger study is needed to validate a causal relationship between RCVS and COVID-19.

In recent years, literature on neuroinflammatory disorders has dramatically expanded, as have options for treatment. However, few reviews have focused on skull-based manifestations of inflammatory disorders. Here, we review the clinical manifestations, etiologies, diagnostic workup, and treatment of both systemic and localized inflammatory diseases of the skull base with a focus on recent updates to the literature. This review aims to guide the workup and management of this complex set of diseases.

In low-income countries (LICs), there are multiple barriers for children with epilepsy (CWE) to attend school. We examined potentially modifiable associations with poor school performance in CWE in the West African Republic of Guinea.Children with epilepsy of school age were recruited using public announcements and a clinical register of people with epilepsy at the Ignace Deen Hospital in Conakry in 2018. A team of Guinean and U.S. neurologists and neurologists-in-training interviewed each CWE and parent for his/her epilepsy history, household finances, educational attainment level, and perceived stigma using the Stigma Scale of Epilepsy (SSE). Each child was also tested using the Wechsler Nonverbal Scale of Ability (WNV). Low school performance was defined as either not attending school or being held back a grade level at least once. Potential predictors of low school performance were analyzed.Of 128 CWE (mean age: 11.6 years, 48.4% female), 11.7% (n = 15) never attended school, 23.3% (n = 30) dropped out, and 64.8% (n = 83) were currently enrolled. Of CWE attending school, 46.9% (n = 39) were held back a grade level. Overall, 54 children were defined as low performers (LPs) (42%). ;Greater than 100 lifetime seizures (odds ratio (OR) = 8.81; 95% confidence interval (CI) = 2.51, 37.4; p = 0.001) and lower total WNV score (OR = 0.954; 95% CI = 0.926, 0.977; p 0.001) were significantly associated with poor school performance in separate models, when controlling for potential confounders. Given the strong relationship between seizure freedom and school performance, we estimated that 38 additional CWE (33.6%) could become high performers (HPs) if all CWE were adequately treated to achieve the lifetime seizure category of10 seizures and could be cognitively intact again. Models examining SSE and household wealth quintile were not significantly associated with school performance.Higher lifetime seizures and lower WNV score were significantly associated with low school performance in CWE in Guinea. In spite of our conservative definition of high school performance (attending without failing) and risk of referral bias at an academic center where patients were allowed to self-refer, we demonstrate that seizure control in this setting could increase the number of CWE who could attend and stay in school.

Neurocysticercosis (NCC) is a major cause of neurologic disease and epilepsy worldwide. Clinical manifestations of NCC are primarily due to the inflammatory response against degenerating cysts of the Taenia solium tapeworm, which occurs when cysts lose their ability to evade host immune responses. Patients with subarachnoid or severe parenchymal NCC may also develop chronic inflammation requiring long-term immunosuppression.2

Highlights • Posterior reversible encephalopathy syndrome may be associated with coronavirus disease. • Risk factors may include modest blood pressure fluctuations and anakinra. • All reported patients had clinical improvement., Introduction Encephalopathy is a common complication of coronavirus disease 2019. Although the encephalopathy is idiopathic in many cases, there are several published reports of patients with posterior reversible encephalopathy syndrome in the setting of coronavirus disease 2019. Objective To describe the diverse presentations, risk factors, and outcomes of posterior reversible encephalopathy syndrome in patients with coronavirus disease 2019. Methods We assessed patients with coronavirus disease 2019 and a diagnosis of posterior reversible encephalopathy syndrome at our institution from April 1 to June 24, 2020. We performed a literature search to capture all known published cases of posterior reversible encephalopathy syndrome in patients with coronavirus disease 2019. Results There were 2 cases of posterior reversible encephalopathy syndrome in the setting of coronavirus 2019 at our institution during a 3-month period. One patient was treated with anakinra, an interleukin-1 inhibitor that may disrupt endothelial function. The second patient had an underlying human immunodeficiency virus infection. We found 13 total cases in our literature search, which reported modest blood pressure fluctuations and a range of risk factors for posterior reversible encephalopathy syndrome. One patient was treated with tocilizumab, an interleukin-6 inhibitor that may have effects on endothelial function. All patients had an improvement in their neurological symptoms. Interval imaging, when available, showed radiographic improvement of brain lesions. Conclusions Risk factors for posterior reversible encephalopathy syndrome in patients with coronavirus disease 2019 may include underlying infection or immunomodulatory agents with endothelial effects in conjunction with modest blood pressure fluctuations. We found that the neurological prognosis for posterior reversible encephalopathy syndrome in the setting of coronavirus disease 2019 infection is favorable. Recognition of posterior reversible encephalopathy syndrome in this patient population is critical for prognostication and initiation of treatment, which may include cessation of potential offending agents and tight blood pressure control.

Purpose The purpose of this study was to characterize the reasons, extent, and impact of traditional medicine use among people with epilepsy (PWE) in the Republic of Guinea. Methods Guinea is a low-income country in sub-Saharan Africa (SSA) with limited healthcare resources. People with epilepsy and their caregivers were seen at a public referral hospital in Conakry, the capital city, where they completed semi-structured interviews with physicians regarding their beliefs about epilepsy, medical care, and engagement with traditional healers. Results Of 132 participants (49% children, 44% female, 55% with a university-educated head of household), 79% had seen a traditional healer, and 71% saw a traditional healer before seeing a medical provider for their epilepsy. Participants were treated by a traditional healer for a mean of 39 months before seeing a medical provider. By contrast, 58% of participants reported taking antiepileptic drugs (AEDs) regularly; 46% reported having undergone a head computed tomography (CT) scan; 58% reported having had an electroencephalogram, and 4% reported having had a brain magnetic resonance imaging (MRI) scan. Conclusions Traditional healers in Guinea provide frontline care for PWE in Guinea with considerable delays in AED initiation, even among a cohort of PWE actively seeking medical care. Engaging with these healers is critical for both influencing community perceptions and appropriately managing epilepsy throughout the country.

EEG triphasic waves are usually seen in the setting of metabolic encephalopathies, but may also occur secondary to medication toxicity. There are few reports of medications causing continuous triphasic waves, and only rare reports in the literature show an association between these discharges and pregabalin toxicity. Here, we describe triphasic waves in a patient with underlying white matter lesions, as well as pregabalin toxicity, secondary to renal insufficiency. Patients with renal or hepatic failure presenting with triphasic waves and confusion pose a diagnostic challenge in determining whether the etiology of their encephalopathy is purely metabolic or whether it results from impaired medication clearance and subsequent toxicity. In this patient, discontinuing pregabalin improved her EEG despite the ongoing renal insufficiency, confirming pregabalin toxicity as an etiology of triphasic waves and encephalopathy.

Background Clinical manifestations of neurocysticercosis (NCC) are primarily due to the inflammatory response against degenerating cysts of the Taenia solium tapeworm. Inflammation can occur when cysts lose their ability to evade the host immune response during their natural life cycle or result from antihelminthic therapy. A subset can develop chronic perilesional edema requiring immunosuppressive therapy. Although guidelines recommend methotrexate (MTX) as an alternative to long-term steroids, limited information is available regarding when to start a steroid-sparing agent (SSA) and alternative SSAs in case of MTX failure or intolerance. Methods Retrospective chart review. Results Three patients with complicated NCC followed at a single tertiary care center are described in this study: Patient 1 with subarachnoid NCC (age 64, female), patient 2 with subarachnoid and intraventricular NCC (age 48, male) and patient 3 with parenchymal NCC (age 43, male). Patients 1–3 were followed clinically and radiographically for 8, 1.5 and 3 years, respectively. Patient 1 was treated with antihelminthic therapy and steroids for 24 months. She was transitioned to MTX for 1 month, but developed ulcerative stomatitis, leukopenia and transaminitis. She completed a treatment course of steroids, complicated by osteoporosis. Patient 2 was treated with 12 months of antihelminthic therapy and steroids with resolution of a previously positive cysticercal antigen in spinal fluid. Imaging revealed persistent inflammation in spite of adequate antihelminthic therapy. He was started on MTX and has remained on this medication for 5 months. Patient 3 was treated with two courses of antihelminthic therapy. He developed perilesional edema despite treatment with steroids and MTX uptitrated to 20 mg weekly. Adalimumab was added to his regimen and he had a rapid radiographic resolution of edema and clinical improvement in seizures. His seizures returned during an interruption in his adalimumab treatment and again resolved with re-initiation of this medication. Conclusion SSAs are beneficial in the long-term treatment of patients with subarachnoid NCC or chronic perilesional edema. The formulation of guidelines that include multiple options for SSAs will be essential in guiding management of complicated NCC. Disclosures All authors: No reported disclosures.

Studies have suggested that the incidence of multiple sclerosis (MS) in HIV-infected (HIV+) patients is lower than that of the general population. Here, we present a case of MS in an HIV+ patient with a relatively suppressed CD4 cell count who developed clinical and radiographic disease worsening in the setting of antiretroviral therapy (ART) initiation. A 47-year-old HIV+ woman (CD4 cell count 216 cells/µl) presented with decreased vision in her right eye. Magnetic resonance imaging (MRI) revealed optic nerve enhancement and open ring-enhancing lesions in the brain concerning for demyelinating disease. Cerebrospinal fluid was tested extensively for infection and malignancy with no abnormal findings. She received five days of intravenous methylprednisolone. Nine days later, she was restarted on ART. Three weeks later, she was readmitted with left eye vision loss and left hemiplegia (CD4 cell count 342 cells/µl). Repeat imaging showed multiple new enhancing lesions. Several cases have described severe MS relapses and unusually widespread demyelinating lesions on MRI after withdrawal of immunosuppressive drugs. We posit that the clinical and radiographic progression that occurred in our patient after initiation of ART represented an immune reconstitution response to ART. Caution may be warranted when initiating ART in HIV+ patients with suppressed CD4 cell count and active MS.

A 28-year-old woman who had recently had hyperemesis gravidarum presented with confusion and falls. She was found to have nystagmus (shown in videos) and MRI abnormalities consistent with Wernicke’s encephalopathy.

A 52-year-old man with a history of factor V Leiden thrombophilia, persistent headaches and papilloedema presented with worsening vision and confusion. MRI and MR angiography of the brain at the time of this presentation showed findings concerning for transverse sinus thrombosis and an associated dural arteriovenous fistula. Dural venous sinus thrombosis can lead to the formation of a dural arteriovenous fistula, which must be considered in the differential diagnosis for intracranial hypertension in patients with thrombophilia.

A 39-year-old woman presented with a right-hemispheric stroke 1 year after she had suffered a left-hemispheric stroke. Her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test. There was no evidence of carotid dissection or another identifiable cause for her carotid occlusions. These findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries. Young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies.

ObjectiveTo characterize the risk factors, clinical course, and treatment of patients with progressive multifocal leukoencephalopathy (PML) diagnosed and followed over a 25-year epoch at 2 academic hospitals.MethodsPatients with a definite diagnosis of PML were identified by positive CSF PCR for JC virus or histopathology between January 1, 1994, and January 1, 2019. Demographic and PML-specific variables were recorded on symptomatic presentation and at follow-up, including risk factors, clinical outcome, neuroimaging findings, and modified Rankin Scale (mRS) score at last follow-up.ResultsThere were 91 patients with confirmed PML. HIV infection was the most common risk factor, identified in 49% (n = 45). Other frequent risk factors included lymphoma, leukemia, or myelodysplasia, identified in 31% of patients (n = 28); exposure to chemotherapeutic medications (30%, n = 27); and exposure to monoclonal antibody therapies (19%, n = 17). Thirty percent of the cohort was alive at the time of censoring, with a median mRS of 2 points, indicating slight disability at last follow-up. Median survival following PML diagnosis in HIV-infected patients was longer than in HIV-uninfected patients (1,992 vs 101 days, p = 0.024). Forty patients survived more than 1 year after PML symptom onset, of whom 24 were HIV infected (60%). Thirteen patients survived more than 10 years after PML symptom onset, all HIV infected, of the 59 patients diagnosed before June 1, 2009, and eligible for 10-year survivor status (22%).ConclusionsWe add to the limited literature on PML by reporting its epidemiology in a large observational cohort. These parameters may be useful for future clinical trials that measure survival and clinical outcomes.