Sporadic Creutzfeldt-Jakob Disease Initially Presenting With Posterior Reversible Encephalopathy Syndrome: A Case Report.

INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal neurodegenerative condition caused by prion proteins. Cortical and subcortical diffusion-weighted imaging restriction on magnetic resonance imaging (MRI) is associated with sCJD. Posterior reversible encephalopathy syndrome (PRES) results from impaired vessel autoregulation due to an identifiable trigger, which is associated with subcortical fluid-attenuated inversion recovery changes on MRI. We report a case of sCJD initially presenting with PRES. CASE REPORT: A 70-year-old woman presented to an outside hospital with progressive confusion and difficulty in managing activities of daily living. Initial examination revealed stuporous mental state and stimulus-induced myoclonus. MRI revealed bilateral subcortical occipital lobe T2-fluid-attenuated inversion recovery hyperintensities without contrast enhancement suggestive of PRES. Electroencephalogram (EEG) revealed frequent generalized periodic discharges meeting criteria for nonconvulsive status epilepticus. Clinical examination and EEG did not improve despite escalating antiseizure medications. Initial lumbar puncture was unremarkable. She was transferred to our hospital with a presumptive diagnosis of PRES, although there was no clear trigger. Continuous EEG revealed ongoing generalized periodic discharges with myoclonic activity meeting criteria for myoclonic seizures that were refractory to multiple antiseizure medications. Repeat MRI showed resolution of PRES but revealed subtle diffuse cortical diffusion-weighted imaging restriction. Repeat lumbar puncture was performed and 14-3-3 and real-time quaking-induced conversion returned positive, confirming sCJD. CONCLUSIONS: This case reports highlights that sCJD can present with neuroimaging consistent with PRES. The diagnosis of sCJD should be considered in patients with PRES who continue to show neurological decline despite optimal management and radiographic improvement of PRES on MRI. Further research is needed to identify a pathophysiological relationship between these clinical phenotypes.

Acute and subacute neurovascular impact of cryptogenic air emboli.

Background: Cerebral air embolism is a rare cause of acute ischemic stroke that is becoming increasingly well-described in the literature. However, the mechanism and severity of this type of injury can vary, with ischemia typically emerging early in the course of care. To the best of our knowledge, delayed ischemia in this setting has not yet been described. Case Description: A stroke code was called for an unresponsive, hospitalized, 75-year-old man. A computerized tomography (CT) scan of the head revealed air within the right greater than left hemispheric cortical veins with loss of sulcation, concerning for developing ischemia, and CT angiography revealed absent opacification of the distal cortical vessels in the right anterior cerebral artery and middle cerebral artery territories. Magnetic resonance imaging (MRI) of the brain was obtained 5.75 h after the patient's last known well-showed small areas of subtle cortical diffusion restriction. Follow-up CT head within 24 h showed near-complete resolution of the air emboli after treatment with 100% fraction of inspired oxygen on mechanical ventilation. Subsequent MRI, performed 4 days after the initial event, showed extensive cortical diffusion restriction and cerebral edema crossing vascular territories. Conclusion: This case highlights that cerebral air emboli can cause delayed ischemia that may not be appreciated on initial imaging. As such, affected patients may require intensive neurocritical care management, close neurologic monitoring, and repeat imaging irrespective of initial radiographic findings.

Cerebral microhemorrhages in severe COVID-19 pneumonia.

Background: Cerebral microhemorrhages are a complication in patients with severe COVID-19 pneumonia. Case Description: A 50-year-old woman presented to the hospital with shortness of breath due COVID-19 (SARS-CoV-2, alpha variant) pneumonia. Her hospital course was complicated by hypoxic respiratory failure requiring intubation and severe acute respiratory distress syndrome. She received a tracheostomy for ongoing ventilator support after prolonged intubation. Her mental status was notable for disorientation and severe agitation for which a magnetic resonance imaging (MRI) of the brain was obtained. MRI revealed numerous microhemorrhages predominantly at the gray-white matter junctions. Conclusion: Cerebral microhemorrhages in severe COVID-19 pneumonia are associated with critical illness, increased mortality, and worse functional outcome. The pathophysiology of cerebral microhemorrhages in COVID-19 is unknown.

Cerebral toxoplasmosis in a patient with multiple myeloma.

Background: Cerebral toxoplasmosis is a rare complication of relapsed/refractory multiple myeloma (MM) after autologous stem cell transplant (SCT). Imaging characteristics can be helpful in leading to rapid diagnosis and treatment. Case Description: A 76-year-old man with relapsed/refractory IgA kappa MM status post autologous SCT who presented to the hospital with altered mental status. His hospital course was complicated by rapid decompensation to obtundation requiring intubation. Magnetic resonance imaging (MRI) of the brain revealed numerous ring-enhancing lesions with eccentric target signs, which were concerning for cerebral toxoplasmosis. Diagnosis was confirmed with positive toxoplasma cerebrospinal fluid polymerase chain reaction test. Conclusion: Cerebral toxoplasmosis should be considered in the differential diagnosis for MM patients who present with altered mental status and neurologic findings. The ring-enhancing lesion with eccentric target sign on MRI can be helpful in the diagnosis of cerebral toxoplasmosis.

Long-Term Risk of Ischemic Stroke among Elderly Survivors of Non-Traumatic Subarachnoid Hemorrhage.

INTRODUCTION: Non-traumatic subarachnoid hemorrhage (SAH) is associated with poor long-term functional outcomes, but the risk of ischemic stroke among SAH survivors is poorly understood. OBJECTIVES: The aim of this study was to evaluate the risk of ischemic stroke among survivors of SAH. METHODS: We performed a retrospective cohort study using claims data from Medicare beneficiaries from 2008 to 2015. The exposure was a diagnosis of SAH, while the outcome was an acute ischemic stroke, both identified using previously validated ICD-9-CM diagnosis codes. We used Cox regression analysis adjusting for demographics and stroke risk factors to evaluate the association between SAH and long-term risk of ischemic stroke. RESULTS: Among 1.7 million Medicare beneficiaries, 912 were hospitalized with non-traumatic SAH. During a median follow-up of 5.2 years (IQR, 2.7-6.7), the cumulative incidence of ischemic stroke was 22 per 1,000 patients per year among patients with SAH, and 7 per 1,000 patients per year in those without SAH. In adjusted Cox models, SAH was associated with an increased risk of ischemic stroke (HR, 2.0; 95% confidence interval, 1.4-2.8) as compared to beneficiaries without SAH. Similar results were obtained in sensitivity analyses, when treating death as a competing risk (sub HR, 3.0; 95% CI, 2.8-3.3) and after excluding ischemic stroke within 30 days of SAH discharge (HR, 1.5; 95% CI, 1.1-2.3). CONCLUSIONS: In a large, heterogeneous national cohort of elderly patients, survivors of SAH had double the long-term risk of ischemic stroke. SAH survivors should be closely monitored and risk stratified for ischemic stroke.

Risk of Mortality After an Arterial Ischemic Event Among Intracerebral Hemorrhage Survivors.

BACKGROUND AND PURPOSE: The impact of arterial ischemic events after intracerebral hemorrhage (ICH) on outcomes is unclear. This study aimed to evaluate the risk of death among ICH survivors with and without an incident arterial ischemic event. METHODS: We performed a retrospective cohort study using claims data from Medicare beneficiaries with a non-traumatic ICH from January 2008 to October 2015. Our exposure was an arterial ischemic event, a composite of acute ischemic stroke or myocardial infarction (MI), identified using validated ICD-9-CM diagnosis codes. The outcome was mortality. We used marginal structural models to analyze the risk of death among ICH patients with and without an arterial ischemic event, after adjusting for confounders as time-varying covariates. RESULTS: Among 8,804 Medicare beneficiaries with ICH, 2,371 (26.9%) had an arterial ischemic event. During a median follow-up time of 1.9 years (interquartile range, 0.7-3.9), ICH patients with an arterial ischemic event had a mortality rate of 21.7 (95% confidence interval [CI], 20.4-23.0) per 100 person-years compared to a rate of 15.0 (95% CI, 14.4-15.6) per 100 person-years in those without. In the marginal structural model, an arterial ischemic event was associated with an increased risk of death (hazard ratio [HR], 1.8; 95% confidence interval [CI], 1.6-1.9). In secondary analyses, the mortality risk was elevated after an ischemic stroke (HR, 1.7; 95% CI, 1.5-1.8), and MI (HR, 3.0; 95% CI, 2.4-3.8). CONCLUSIONS: We found that elderly patients who survived an ICH had an increased risk of death after a subsequent ischemic stroke or MI.

Smoking Cessation in Stroke Survivors in the United States: A Nationwide Analysis.

BACKGROUND: Continued smoking after stroke is associated with a high risk of stroke recurrence and other cardiovascular disease. We sought to comprehensively understand the epidemiology of smoking cessation in stroke survivors in the United States. Furthermore, we compared smoking cessation in stroke and cancer survivors because cancer is another smoking-related condition in which smoking cessation is prioritized. METHODS: We performed a cross-sectional analysis of data from the Centers for Disease Control and Prevention Behavioral Risk Factor Surveillance System, an annual, nationally representative health survey. Using pooled data from 2013 to 2019, we identified stroke and cancer survivors with a history of smoking. We used survey procedures to estimate frequencies and summarize quit ratios with attention to demographic and geographic (state-wise and rural-urban) factors for stroke survivors. The quit ratio is conventionally defined as the proportion of ever smokers who have quit. Then, we used multivariable logistic regression to compare quit ratios in stroke and cancer survivors while adjusting for demographics and smoking-related comorbidities. RESULTS: Among 4 434 604 Americans with a history of stroke and smoking, the median age was 68 years (interquartile range, 59-76), and 45.4% were women. The overall quit ratio was 60.8% (95% CI, 60.1%-61.6%). Quit ratios varied by age group, sex, race and ethnicity, and several geographic factors. There was marked geographic variation in quit ratios, ranging from 48.3% in Kentucky to 71.5% in California. Furthermore, compared with cancer survivors, stroke survivors were less likely to have quit smoking (odds ratio, 0.72 [95% CI, 0.67-0.79]) after accounting for differences in demographics and smoking-related comorbidities. CONCLUSIONS: There were considerable demographic and geographic disparities in smoking quit ratios in stroke survivors, who were less likely to have quit smoking than cancer survivors. A targeted initiative is needed to improve smoking cessation for stroke survivors.

HaNDL Syndrome Presenting with Thunderclap Headache.

INTRODUCTION: Transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) is defined as a secondary, nonvascular headache disorder characterized by the findings described in its name. Patients with HaNDL syndrome typically present with gradual onset migrainous headaches of moderate to severe intensity with transient neurological symptoms. Case Report. We discuss a patient who presented with thunderclap headache, recent transient neurologic deficits, and was ultimately diagnosed with HaNDL after an extensive neurologic evaluation. CONCLUSION: Thunderclap headache has very rarely been described in patients with HaNDL. After excluding emergent and secondary causes, HaNDL should be considered in patients with thunderclap-quality headaches, particularly when there is a history of transient neurological symptoms.

Non-Traumatic Subdural Hemorrhage and Risk of Arterial Ischemic Events.

Background and Purpose- The risk of arterial ischemic events after subdural hemorrhage (SDH) is poorly understood. This study aimed to evaluate the risk of acute ischemic stroke and myocardial infarction among patients with and without nontraumatic SDH. Methods- We performed a retrospective cohort study using claims data from 2008 through 2014 from a nationally representative sample of Medicare beneficiaries. The exposure was nontraumatic SDH. Our primary outcome was an arterial ischemic event, a composite of acute ischemic stroke and acute myocardial infarction. Secondary outcomes were ischemic stroke alone and myocardial infarction alone. We used validated International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes to identify our predictor and outcomes. Using Cox regression and corresponding survival probabilities, adjusted for demographics and vascular comorbidities, we computed the hazard ratio in 4-week intervals after SDH discharge. We performed secondary analyses stratified by strong indications for antithrombotic therapy (composite of atrial fibrillation, peripheral vascular disease, valvular heart disease, and venous thromboembolism). Results- Among 1.7 million Medicare beneficiaries, 2939 were diagnosed with SDH. In the 4 weeks after SDH, patients' risk of an arterial ischemic event was substantially increased (hazard ratio, 3.6 [95% CI, 1.9-5.5]). There was no association between SDH diagnosis and arterial ischemic events beyond 4 weeks. In secondary analysis, during the 4 weeks after SDH, patients' risk of ischemic stroke was increased (hazard ratio, 4.2 [95% CI, 2.1-7.3]) but their risk of myocardial infarction was not (hazard ratio, 0.8 [95% CI, 0.2-1.7]). Patients with strong indications for antithrombotic therapy had increased risks for arterial ischemic events similar to patients in the primary analysis, but those without such indications did not demonstrate an increased risk for arterial ischemic events. Conclusions- Among Medicare beneficiaries, we found a heightened risk of arterial ischemic events driven by an increased risk of ischemic stroke, in the 4 weeks after nontraumatic SDH. This increased risk may be due to interruption of antithrombotic therapy after SDH diagnosis.

Mobile Stroke Units: Bringing Treatment to the Patient.

PURPOSE OF REVIEW: Mobile stroke units (MSUs) have revolutionized emergency stroke care by delivering pre-hospital thrombolysis faster than conventional ambulance transport and in-hospital treatment. This review discusses the history of MSUs technological development, current operations and research, cost-effectiveness, and future directions. RECENT FINDINGS: Multiple prospective and retrospective studies have shown that MSUs deliver acute ischemic stroke treatment with intravenous recombinant tissue plasminogen activator (IV r-tPA) approximately 30 min faster than conventional care. The 90-day modified Rankin Scores for patients who received IV r-tPA on the MSU compared to conventional care were not statistically different in the PHANTOM-S study. Two German studies suggest that the MSU model is cost-effective by reducing disability and improving adjusted quality-life years post-stroke. The ongoing BEST-MSU trial will be the first multicenter, randomized controlled study that will shed light on MSUs' impact on long-term neurologic outcomes and cost-effectiveness. MSUs are effective in reducing treatment times in acute ischemic stroke without increasing adverse events. MSUs could potentially improve treatment times in large vessel occlusion and intracranial hemorrhage. Further studies are needed to assess functional outcomes and cost-effectiveness. Clinical trials are ongoing internationally.

Alzheimer Disease and Cancer: A National Inpatient Sample Analysis.

INTRODUCTION: Studies have demonstrated an inverse relationship between Alzheimer dementia (AD) and cancer. This inverse relationship was further explored. In addition, Pin1 expression has been implicated in the cell cycle regulation of both disease processes. The relationship of Pin1 expression in 10 cancer types and secondary diagnosis of AD was examined. MATERIALS AND METHODS: A cross-sectional analysis was performed using discharge data from the National Inpatient Sample from 1999 to 2008. Cancer was defined as the primary discharge diagnosis and AD was defined as the secondary discharge diagnosis. Cancer types were grouped according to their Pin1 expression to examine its relationship with AD. Analysis was performed by logistic regression. RESULTS: Of ∼3 million cancer discharge diagnoses, 1.0% had a secondary diagnosis of AD. Discharge data of all 10 cancer types revealed a lower likelihood of secondary AD diagnosis. Prostate [crude odds ratios (OR): 0.26 (0.24 to 0.29), multivariate OR: 0.39 (0.35 to 0.43)], ovarian [crude OR: 0.38 (0.32 to 0.44), multivariate OR: 0.35 (0.30 to 0.41)], and lung cancer [crude OR: 0.39 (0.36 to 0.41), multivariate OR: 0.41 (0.39 to 0.44)] demonstrated the lowest odds of secondary AD diagnosis. When cancer types were grouped per Pin1 expression, cancer types with Pin1 underexpression were more likely to be associated with secondary diagnosis of AD than cancer types with Pin1 overexpression [crude OR: 1.4 (1.3 to 1.4), multivariate OR: 1.08 (1.02 to 1.14)]. DISCUSSION: This secondary data analysis further demonstrated an inverse relationship between AD and 10 cancer types, with prostate, ovarian, and lung cancers displaying the greatest inverse relationship. Pin1 underexpressing cancer types had a significantly higher likelihood of secondary diagnosis of AD than Pin1 overexpressing cancer types.

Sleep Disorders and Stroke: Does Treatment of Obstructive Sleep Apnea Decrease Risk of Ischemic Stroke?

PURPOSE OF REVIEW: This review aims to support obstructive sleep apnea (OSA) as a risk factor for ischemic stroke, review treatment strategies for OSA, provide a comprehensive review of clinical data on OSA treatment and ischemic stroke risk, and to critically assess if treatment of OSA decreases the risk of ischemic stroke and if treatment improves outcomes and subsequent ischemic stroke risk in post-stroke patients. RECENT FINDINGS: Several observational studies, randomized controlled trials (RCTs), and meta-analyses have examined the risk of ischemic stroke and cardiovascular events in patients with OSA and have also examined continuous positive airway pressure (CPAP) treatment in these patients. Observational studies have shown an increased risk of ischemic stroke in patients with untreated OSA when compared with patients treated with CPAP; however, results are not statistically significant. RCTs and meta-analyses have shown no significant ischemic stroke risk reduction in CPAP treated patients with OSA. Several studies have shown improved outcomes in post-stroke patients with OSA treated with CPAP; however, few data is available for subsequent ischemic stroke risk reduction. Further research is needed for surgical treatment of OSA and assessment of ischemic stroke risk. OSA is associated with increased risk of ischemic stroke, and OSA should be treated with the appropriate therapy. While the current data is promising, more studies are necessary to state whether treatment of OSA reduces ischemic stroke risk and subsequent ischemic stroke risk. A practical approach to the sleep disorder evaluation and treatment of patients with cerebrovascular disease is outlined.

A Case of Lyme Carditis Presenting with Atrial Fibrillation.

We report a case of a 20-year-old man who presented to our institution with a new arrhythmia on a routine EKG. Serial EKG tracings revealed various abnormal rhythms such as episodes of atrial fibrillation, profound first degree AV block, and type I second degree AV block. He was found to have positive serologies for Borrelia burgdorferi. After initiation of antibiotic therapy, the atrial arrhythmias and AV block resolved. Here, we present a case of Lyme carditis presenting with atrial fibrillation, a highly unusual presentation of Lyme carditis.

Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis.

Central pontine myelinolysis (CPM) is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Common clinical manifestations of CPM include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees; however, coma, "locked-in" syndrome, or death can occur in most severe cases. Rarely, CPM presents with neuropsychiatric manifestations, such as personality changes, acute psychosis, paranoia, hallucinations, or catatonia, typically associated with additional injury to the brain, described as extrapontine myelinolysis (EPM). We present a patient with primarily neuropsychiatric manifestations of CPM, in the absence of focal neurologic deficits or radiographic extrapontine involvement. A 51-year-old female without significant medical history presented with dizziness, frequent falls, diarrhea, generalized weakness, and weight loss. Physical examination showed no focal neurological deficits. Laboratory data showed severe hyponatremia, which was corrected rather rapidly. Subsequently, the patient developed symptoms of an acute psychotic illness. Initial brain magnetic resonance imaging (MRI) was unremarkable, although a repeat MRI two weeks later revealed changes compatible with CPM. This case demonstrates that acute psychosis might represent the main manifestation of CPM, especially in early stages of the disease, which should be taken into consideration when assessing patients with acute abnormalities of sodium metabolism.