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Purpose To determine the risk of stroke, transient ischemic attack (TIA), and transient monocular vision loss (TMVL) before and after a central retinal artery occlusion (CRAO). Design Population-based, retrospective case series. Participants Patients diagnosed with a CRAO in Olmsted County, Minnesota, from 1976 to 2016. Methods Patients living in Olmsted County with a diagnosis code of CRAO from 1976 to 2016 were reviewed. New CRAOs were confirmed, and stroke, TIA, and TMVL events in the 15 days before and after CRAO were recorded. Main Outcome Measures Incidence of stroke, TIA, and TMVL events in the 15 days before and after CRAO. Results Eighty-nine patients with a CRAO were identified, providing an annual incidence of 2.58/100 000 (95% confidence interval [CI], 2.04–3.11). Median age at the time of CRAO was 76 years (range, 46–100 years); 56.2% were male, and 89.9% of the cohort was White. In the 15 days before and after CRAO, there were 2 ischemic strokes (2.2%), 1 hemorrhagic stroke (1.1%), 2 TIAs (2.2%), and 9 TMVL events (10.1%). Starting in 1999, 15 of 45 patients underwent magnetic resonance imaging within 2 months of CRAO. One patient (6.7%) had evidence of asymptomatic diffusion restriction, and 9 patients (60%) had a remote infarct. Conclusions This population-based study demonstrated that the risk of symptomatic ischemic stroke is 2.2% in the 15 days before and after a CRAO, which is slightly lower than most studies from tertiary centers. These data should be considered as practice recommendations are developed regarding the urgency of neurovascular workup in patients with acute CRAO.

Introduction There is increased interest in the use of artificial intelligence-based (AI) software packages in the evaluation of neuroimaging studies for acute ischemic stroke. We studied whether, compared to standard image interpretation without AI, Brainomix e-ASPECTS software improved interobserver agreement and accuracy in detecting ASPECTS regions affected in anterior circulation LVO. Methods We included 60 consecutive patients with anterior circulation LVO who had TICI 3 revascularization within 60 minutes of their baseline CT. A total of 16 readers, including senior neuroradiologists, junior neuroradiologists and vascular neurologists participated. Readers interpreted CT scans on independent workstations and assessed final ASPECTS and evaluated whether each individual ASPECTS region was affected. Two months later, readers again evaluated the CT scans, but with assistance of e-ASPECTS software. We assessed interclass correlation coefficient for total ASPECTS and interobserver agreement with Fleiss’ Kappa for each ASPECTS region with and without assistance of the e-ASPECTS. We also assessed accuracy for the readers with and without e-ASPECTS assistance. In our assessment of accuracy, ground truth was the 24 hour CT in this cohort of patients who had prompt and complete revascularization. Results Interclass correlation coefficient for total ASPECTS without e-ASPECTS assistance was 0.395, indicating fair agreement compared, to 0.574 with e-ASPECTS assistance, indicating good agreement (P Conclusions Use of Brainomix e-ASPECTS software resulted in significant improvements in inter-rater agreement and accuracy of ASPECTS score evaluation in a large group of neuroradiologists and neurologists. e-ASPECTS software was more predictive of final infarct/ASPECTS than the overall group interpreting the CT scans with and without e-ASPECTS assistance.

Background Diffuse intracranial susceptibility abnormalities have recently been described among patients with coronavirus disease 2019 (COVID-19), although prior studies have consisted of case reports and/or series. This brief literature review seeks to compile and catalogue the available data to elucidate characteristic features of such findings. Materials and methods Scientific articles and studies on intracranial microhemorrhages in the setting of COVID-19 were searched on PubMed, Google Scholar, and the Cochrane Library. Included studies described intracranial microbleed(s) on magnetic resonance imaging in patients with COVID-19. If multiple patients were described, only patients with intracranial microhemorrhage on magnetic resonance imaging were included for analysis. Patient demographics, severity of illness (e.g. intensive care unit admission and/or intubation), time from diagnosis of COVID-19 to magnetic resonance imaging, and location(s) of any observed microhemorrhages were noted. Results A total of 39 patients with suspected intracranial microhemorrhages have been described in prior studies. The average age of patients was 64.7 years; 21.9% were women. The average time between COVID-19 diagnosis and magnetic resonance imaging was 31.7 days. All patients in the cohort were admitted to critical care and were either intubated or treated with extracorporeal membrane oxygenation during their clinical course. Microhemorrhages were most commonly located in the subcortical/juxtacortical white matter and corpus callosum. Conclusions Intracranial microbleeds are a well-documented finding in patients with severe COVID-19, and are most commonly callosal and subcortical/juxtacortical in location.

An embryological association between moyamoya disease (MMD) and cardiac manifestations has been proposed. Data up to this point remains anecdotal, and the prevalence of cardiac manifestations in a western MMD population is uncertain. The objective of this study was to determine the prevalence of cardiac manifestations including coronary artery disease (CAD) and congenital cardiac defects in a mostly Caucasian population of MMD patients and review prior reports of such cases. Medical records of MMD patients who presented to our institution between 1990 and 2019 were retrospectively reviewed for the presence of various congenital cardiac malformations and concomitant CAD. The prevalence of congenital cardiac defects and CAD was determined. A literature search for prior cases of MMD with concomitant cardiac manifestations was performed. A total of 181 MMD patients were included in our analysis, 139 (76.8%) of whom were Caucasian. Ten patients had cardiac manifestations (5.5%). There were six total MMD patients with congenital cardiac defects (3.3%). All patients with congenital defects were diagnosed in childhood. The prevalence of congenital defects in MMD was slightly higher than the general population as reported previously (0.8–1.2%). Four MMD patients had CAD (2.2%). The mean age of patients with CAD was 41.0 years (SD = 12.3, range = 33–59) in our series and 33.1 years (SD = 15.0) in a review of prior reports. These mean ages of CAD are in contrast to the 7th and 8th decades of lifein the general population as indicated by prior studies. Our findings support an association between MMD and cardiac manifestations. Further investigation is warranted in order to further characterize this potential relationship and shed light on a possible cardio-cephalic neural crest syndrome.

Moyamoya Disease (MMD) and moyamoya Syndrome (MMS) have been reported to be associated with pro-thrombotic states in some patients. To date, however, such reports have been limited to case reports or small case series. We sought to determine the prevalence of pro-thrombotic states among a large cohort of both MMD and MMS patients. We retrospectively reviewed the medical records of all adult patients who were diagnosed with MMD or MMS from our institution. In addition to basic demographic and clinical data, the presence of certain pro-thrombotic conditions was noted. The presence of such conditions was determined based on review of clinical diagnoses and laboratory reports. The length of follow-up and the presence of interval ischemic events were obtained. Out of a total of 180 patients diagnosed with MMD or MMS, 33 were found to have a concomitant pro-thrombotic condition (18.3%). Of 147 patients with MMD, 23 were found to have a pro-thrombotic condition (15.6%). There were 10 out of 33 total patients (30.3%) in the MMS cohort that had a concomitant pro-thrombotic condition. There were no differences in specific pro-thrombotic conditions between MMD and MMS cohorts. There were no differences in future ischemic events between moyamoya patients with pro-thrombotic tendencies compared to those without (13.3% versus 23.7%, respectively, P = 0.32), and this was also true for patients who underwent revascularization procedures (22.6% versus 29.4%, P = 0.76). There was also no difference in prevalence of revascularization graft occlusion between moyamoya patients with pro-thrombotic tendencies and those without at follow-up (1.2% versus 5.9%, P = 0.31). It is likely that both MMD and MMS are associated with or predispose to pro-thrombotic conditions in adult patients, although the clinical and surgical significance is uncertain. Further study is needed in order to further elucidate and characterize this potential association.

Background The MYH11 gene codes for smooth muscle myosin heavy chain, which has a critical function in maintaining vascular wall stability. Patients with this mutation most commonly have aortic and cardiac defects. Documented involvement of intracranial vessels is exceptional. Case Description A 29-year-old woman with a history of patent ductus arteriosus and aortic dissection was found to have incidental bilateral stenosis of the terminal internal carotid arteries as well as the proximal anterior cerebral arteries, middle cerebral arteries, and posterior cerebral arteries on magnetic resonance angiography that was obtained for unrelated symptoms. There was no evidence of basal collateral formation, and a generalized straightening of the vessels was observed. These angiographic findings have been typically observed in patients with ACTA2 mutations. Thus, genetic testing was pursued, which uncovered the presence of an MYH11 mutation. Follow-up imaging at 51 months demonstrated that the intracranial stenosis remained stable without evidence of basal collateral formation. She did not experience any neurologic events during the follow-up interval. Conclusions Intracranial vessel involvement in patients with MYH11 mutations is rare. Vigilant cerebrovascular monitoring should be practiced in this population to guide appropriate management. Reporting of similar cases is important to improve understanding of the development of idiopathic intracranial stenosis in young individuals.

Background Nonarteritic anterior ischemic optic neuropathy (NAION) is a common cause of acute optic neuropathy in adults and is associated with vascular risk factors. Owing to the overlapping risk factor profiles between NAION and cerebral stroke, previous studies have produced conflicting results with regard to NAION as an independent risk factor for stroke. Methods A retrospective chart review was conducted using the Rochester Epidemiology Project database to identify all cases of NAION occurring among Olmsted County, Minnesota residents from January 1, 1990, through December 31, 2016. Stroke events were characterized using clinical and radiologic data. Comparison was made to an age- and sex-matched control group with similar vascular risk factors. Results One-hundred four patients with NAION and 104 control subjects were analyzed. Median age at diagnosis was 65 years (range, 40-90 years). Thirteen patients (13%) with NAION and 10 controls (10%) had symptomatic strokes after the age of 40 years. Among patients with NAION, 6 (46%) suffered a stroke before the diagnosis of NAION, 5 (39%) at least 5 months after the NAION diagnosis, and 2 patients (15%) suffered strokes both before and after the NAION. The cumulative probability of symptomatic strokes for patients with NAION was not significantly different than for controls (hazard ratio = 1.50, 95% confidence interval: 0.66-3.42; P = 0.34). There were no cardioembolic strokes within 1 month of the NAION diagnosis. The mechanism of symptomatic strokes did not differ between the 2 groups. Conclusions NAION does not confer an increased risk of symptomatic stroke beyond the risk posed by age and existing vascular risk factors.

Introduction: Digital subtraction angiography (DSA) is currently the gold standard for evaluation of moyamoya disease (MMD) and is the basis for several grading systems. Non-invasive modalities such as MRA are also used in assessment of MMD. Accurate implementation of these staging systems for use with MRA would imply a less-invasive method of MMD monitoring. Methods: This was a single-center retrospective study that compared MRA to reference DSA studies in determining MMD severity using Berlin and modified Suzuki (mSG) grading systems A single reviewer assigned modified Suzuki and Berlin grades to all included hemispheres on both MRA and DSA. The mean difference between MRA and DSA grades for each DSA reference grade was determined utilizing both grading systems. The distribution of scores was also compared between MRA and DSA using both grading systems. Results: A total of 166 cerebral hemispheres in 90 patients were included: The mean age at MMD diagnosis was 34.8 years (standard deviation [SD] = 14.2), and fifty-three patients (63.9%) were male. Mean MRA grades were significantly lower than DSA reference grades 3 and 4 utilizing the mSG system (p = Table 1 ). The distribution of modified Suzuki grades on MRA was skewed toward relatively lower grades as compared to DSA (p = Table 2 ). Conclusions: DSA is essential in the evaluation of MMD, as MRA is shown to underestimate disease stage, with a greater degree of underestimation in more severe disease. We suggest DSA be performed in order to aid in appropriate treatment planning.

Background Headache in patients with moyamoya disease is an under-addressed topic in the medical literature. Delay in the diagnosis of moyamoya disease or inappropriate treatment of headache could lead to devastating cerebrovascular outcome. With the evolving understanding of moyamoya disease, migraine pathophysiology, and various migraine-specific medications that have become available, it is crucial to provide an updated overview on this topic. Methods We searched PubMed for keywords including moyamoya disease, moyamoya syndrome, headache in moyamoya, surgical revascularization, surgical bypass, migraine and moyamoya, and calcitonin gene-related peptide (CGRP). We summarized the literature and provide a comprehensive review of the headache presentation, possible mechanisms, the impact of various surgical revascularizations on headache in patients with moyamoya disease, and the medical management of headache incorporating novel migraine-specific treatments. Results and conclusion: The most common headache phenotype is migraine; tension-type headache, hemiplegic migraine, and cluster headache have also been reported. Most patients experience improvement of headache after surgical revascularization, though some patients report worsening, or new-onset headache after surgery. Given the complexity of moyamoya disease, careful consideration of different types of medical therapy for headache is necessary to improve the quality of life while not increasing the risk of adverse cerebrovascular events. More prospective studies are warranted to better understand and manage headache in patients with moyamoya disease.

Most ischemic stroke is caused by atherosclerosis (large- and small-vessel disease) and cardioembolic sources (eg, atrial fibrillation). However, it is important to recognize the clinical, laboratory, and radiologic manifestations of rarer causes of stroke since the treatment may differ from the treatment of more typical causes. This chapter reviews the less common causes of stroke in addition to stroke in special situations: stroke in children, stroke in pregnant women, spinal cord infarction, and cerebral venous sinus thrombosis.

An 86-year-old woman with a history of hypertension, hyperlipidemia, coronary artery disease, and hypothyroidism sought care for subacute, progressive cognitive decline. Five months earlier, she was hospitalized for a small, left temporal, lobar, intracerebral hemorrhage with associated receptive aphasia. Over the next several months, she had a precipitous cognitive decline. She was prescribed memantine by her primary physician because of concern for dementia. One month before seeking care, she was found unconscious in her bathroom, which was believed to be an unwitnessed seizure. Brain magnetic resonance imaging 1 month before the current evaluation showed a prior, small, left temporal hemorrhage and diffuse lobar microhemorrhages on gradient echo imaging, focal leptomeningeal gadolinium enhancement in the left temporal lobe, and multifocal T2 hyperintensity with mass effect, maximal in the left temporal lobe. Electroencephalography showed multifocal, independent epileptiform discharges. She underwent open biopsy of the left temporal lobe, which indicated focal granulomatous inflammation causing vascular destruction, with β‎-amyloid plaques within the cortical and leptomeningeal vessels. The findings were consistent with a diagnosis of amyloid-β‎-related angiitis in the setting of severe cerebral amyloid angiopathy. Because of concern for subclinical seizures and epileptiform discharges on electroencephalography, the patient was started on levetiracetam without substantial change in her mental status. After the biopsy findings demonstrated inflammatory changes consistent with amyloid-β‎-related angiitis, she was started on intravenous methylprednisolone, followed by transition to prednisone. After 6 months of treatment, she had significant clinical and radiographic improvement. Follow-up magnetic resonance imaging at that time showed interval improvement in the T2 hyperintensity and mass effect in the left temporal lobe. She was again independent with her activities of daily living, and memantine was discontinued. Cerebral amyloid angiopathy encompasses a heterogeneous group of diseases characterized by amyloid-β‎ peptide deposition. The most common clinical manifestation of cerebral amyloid angiopathy is lobar intracerebral hemorrhage, which can be multifocal and recurrent but can also result in cerebral ischemia and ischemic leukoencephalopathy.

OBJECTIVES Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is inherited microangiopathy characterized by recurrent subcortical infarcts. A majority of those with CADASIL report coexistent migraine with aura. The authors aim to quantitatively describe migraine-related disability within a CADASIL cohort. METHODS A cross-sectional analysis was performed in a cohort of CADASIL. The Migraine Disability Assessment (MIDAS) quantified migraine-related disability. Further metrics obtained include first and last migraine, aura semiology, and therapeutic measures. RESULTS Twenty-four individuals were included [63% (15/24) female individuals; mean age, 56 y; range, 34 to 81 y]. Fifty-four percent (13/24) reported migraine, whereas 46% (11/24) reporting varying degrees of migraine-related disability. MIDAS Questionnaire scores appeared bimodal: 58% (14/24) scored 0 to 5, 7% (1/24) scored 6 to 10, 7% (1/24) scored 11 to 20 and 33% (8/24) scored over 20. Severe disability was associated with the multiplicity of aura semiologies and poor response to pharmacologic prophylaxis. CONCLUSIONS A bimodal distribution of migraine-related disability was observed. A third of individuals had a severe disability and appeared medically refractory to medical migraine prophylactic measures. This study may serve as a reference point for future trials quantitatively gauging response of novel migraine treatment strategies within this unique population.

Mary Broadfoot Walker (1888-1974) was the first to demonstrate the ‘Mary Walker effect’ describing the weakness of other muscle groups following release of the arteriovenous occlusion of an unrelated exercising muscle group in patients with myasthenia gravis, which led to the search for a circulating causative agent for myasthenia gravis. She was the first to clearly demonstrate that strength temporarily improved in patients with myasthenia gravis with physostigmine or Prostigmin (neostigmine). This dramatic treatment response has been erroneously termed the ‘Mary Walker effect'. Further, she noted hypokalaemia during attacks of weakness in familial periodic paralysis, pioneering treatment with potassium chloride. Although Mary Walker practiced in a nonacademic setting and trained at a time when women were not allowed to train alongside men, she was the first to convincingly demonstrate three life-changing treatments in the field of neuromuscular medicine, a feat that few physicians of any era can claim.

Introduction: High-resolution vessel wall imaging (HR-VWI) of the intracranial vasculature may aid in the diagnosis and management of Moyamoya Disease (MMD). The patterns of HR-VWI findings of the cerebral vasculature of MMD patients remains uncertain, however. We sought to describe HR-VWI findings in a single-center cohort of MMD patients. Methods: Consecutive patients who were evaluated at a Midwest American medical center with a HR-VWI exam were included. Fourteen anterior circulation arterial segments in each patient were analyzed: proximal and distal supraclinoid Internal Carotid Artery, proximal M1 segment of the Middle Cerebral Artery, mid-portion of the M1, distal M1, proximal A1 segment of the Anterior Cerebral Artery and distal A1. Imaging variables that were collected from each segment included the presence of wall thickening (concentric vs eccentric), degree and pattern (concentric or eccentric) of contrast enhancement, and presence of positive or negative vessel remodeling. The total number and percentage of vessel segments demonstrating these characteristics were calculated. The number and percentage of patients with at least one segment demonstrating these characteristics were calculated. Results: Thirty-six patients (32 females, 88.9%) with a diagnosis of MMD were included. Overall, 221 of 504 segments (43.8%) demonstrated negative remodeling in 29 (75%) patients. Ninety-eight segments (19.4%) demonstrated wall enhancement (71 concentric, 27 eccentric; 22 high grade, 76 low grade) in 32 (88.9%) patients. Eighty-seven segments (17.3%) demonstrated wall thickening (52 concentric, 35 eccentric) in 32 patients (88.9%). Conclusions: Negative remodeling was the most-commonly observed vessel wall abnormality in all segments analyzed. However, a high percentage of patients had at least one segment demonstrating wall enhancement, wall thickening and negative remodeling. Continued study is necessary to further elucidate the HR-VWI patterns of the cerebral arterial system in MMD patients.

Neurologic complications of diseases of the aorta are common, as the brain and spinal cord function is highly dependent on the aorta and its branches for blood supply. Any disease impacting the aorta may have significant impact on the ability to deliver oxygenated blood to the central nervous system, resulting in ischemia-and if prolonged-cerebral and spinal infarct. The breadth of pathology affecting the aorta is diverse and neurologic complications can vary dramatically based on the location, severity, and underlying etiology. This chapter outlines the major pathology of the aorta while highlighting the associated neurologic complications. This chapter covers the entire spectrum of neurologic complications associated with aortic disease by beginning with a detailed overview of the spinal cord vascular anatomy followed by a discussion of the most common aortic pathologies affecting the nervous system, including aortic aneurysm, aortic dissection, aortic atherosclerosis, inflammatory and infectious aortopathies, congenital abnormalities, and aortic surgery.

Background Intraplaque hemorrhage (IPH) in a carotid artery plaque viewed on vessel wall imaging has been shown to be associated with ischemic stroke. Whether such an association between carotid IPH and retinal artery occlusion (RAO) exists remains unknown. Methods This was a cross-sectional prevalence study. Medical and imaging records of all patients who underwent neck MRA with plaque imaging sequences at our institution from 2015 to 2020 were retrospectively reviewed. Fourteen patients with confirmed RAO and plaque imaging performed within 6 weeks of presentation were included. A group of 211 patients without a prior ischemic event (RAO, stroke, etc.) with plaque imaging were used as controls. A single artery from control patients was randomly selected. The prevalence of IPH and degree of ipsilateral carotid stenosis were compared between RAO and control patients. Multiple regression analysis was performed to determine independent associations between variables and RAO. Results Five patients (35.7%) with RAO had imaging evidence of ipsilateral IPH in contrast to 7 of 211 (3.3%) patients in the control group (odds ratio [OR]: 16.2, 95% confidence interval [95% CI]: 4.3-61.1, P = 0.0002). Of the 5 patients with RAO and ipsilateral IPH, only one (20.0%) was found to ipsilateral carotid stenosis greater than 70%. Carotid IPH was the only variable that was independently associated with RAO (OR: 12.6, 95% CI = 2.2-73.6, P = 0.005). Conclusions Carotid IPH is independently associated with RAO. The use of plaque imaging in the evaluation of patients with acute RAO is therefore supported.

Neurologic complications of diseases of the aorta are common, as the brain and spinal cord function is highly dependent on the aorta and its branches for blood supply. Any disease impacting the aorta may have significant impact on the ability to deliver oxygenated blood to the central nervous system, resulting in ischemia-and if prolonged-cerebral and spinal infarct. The breadth of pathology affecting the aorta is diverse and neurologic complications can vary dramatically based on the location, severity, and underlying etiology. This chapter outlines the major pathology of the aorta while highlighting the associated neurologic complications. This chapter covers the entire spectrum of neurologic complications associated with aortic disease by beginning with a detailed overview of the spinal cord vascular anatomy followed by a discussion of the most common aortic pathologies affecting the nervous system, including aortic aneurysm, aortic dissection, aortic atherosclerosis, inflammatory and infectious aortopathies, congenital abnormalities, and aortic surgery.

Objective To assess whether the creation of a formal structure to measure and promote wellness among neurology residents would facilitate the development of interventions associated with measurable improvements. Methods In 2018 we founded the Resident Wellness Committee for the Department of Neurology at our institution. The Resident Wellness Committee was led by resident and staff neurologist co-chairs and had as its vision to promote well-being through initiatives centered in work-life integration, emotional and physical well-being, and social engagement. Web-based surveys assessing various aspects of well-being were administered at baseline and 1 year after launch, comprising the period June 21, 2018, through June 30, 2019. Results Response rates were high at baseline (21 of 34; 62%) and follow-up (25 of 33; 76%). Interventions pursued in the interim included education on handling unexpected absences, adjustments to holiday schedule policies, infrastructure for nutrition and respite, and a team-based department fitness challenge, among others. Overall, at both timepoints more than 80% (18 of 21; 21 of 25) of respondents endorsed at least mild burnout symptoms, although clinical workload, independence, and education were overwhelmingly viewed positively throughout. Notable improvements at follow-up included greater comfort with adjusting schedules at times of need, increased camaraderie within the program, and a smaller proportion of respondents endorsing substantial burnout symptoms. Conclusion Through deliberate and sustained efforts backed by data, our work demonstrates that concrete changes can be successfully pursued to promote well-being among neurology residents. Given that Neurology has one of the highest burnout rates among medical specialties, our approach may serve as a model for other programs to replicate.

Charles Edward Beevor (1854–1908) was a prominent English neurologist who served in a variety of positions at the National Hospital for the Paralysed and Epileptic, Queen Square, from 1883 until his sudden death due to coronary artery disease in 1908. Staunchly committed to the meticulous study of neuroanatomy and physiology and education of his fellow physicians, Beevor was an accomplished clinician-scientist. He is most well known for describing the Beevor sign (commonly known as “Beevor's sign”), which is the upward movement of the umbilicus with truncal flexion from a supine position, used to indicate a spinal cord lesion between the levels of T10 and T12. His sign has also been reported to be suggestive of facioscapulohumeral muscular dystrophy. While the initial description of the Beevor sign has traditionally been attributed to his 1903 Croonian Lectures, he actually first described his sign in his 1898 textbook Diseases of the Nervous System: A Handbook for Students and Practitioners. In addition to his eponymous sign, Beevor also made significant contributions to the understanding of the representation of motor movements in the cerebral cortex, and, of more importance, utilized a novel method to identify cerebral vascular territory maps that are still utilized by neurologists today.

To describe rare but important cerebrovascular complications of giant cell arteritis (GCA).We report a 59-year-old man who initially presented with vasculitis of the lower extremities. While on steroids, he developed strokes in multiple vascular territories. The conventional angiogram showed stenosis of bilateral carotid and vertebral vessels as they entered the dura. Temporal artery biopsy confirmed GCA. He began cyclophosphamide treatment, which stabilized his clinical course; however, this was switched to tocilizumab by an outside rheumatologist. Two months later, the patient had progression of vessel stenosis and suffered additional strokes. Despite interventions to augment cerebral perfusion, the infarctions continued to expand and the patient passed away.This case highlights several important features of strokes in GCA: the predilection for the dural entry point of cerebral blood vessels, the progression of disease despite steroids, and the need to quickly escalate treatment in these cases. As seen in our patient, however, this disease carries high morbidity and mortality and patients often have poor outcomes despite aggressive immunosuppression.

Medical therapies for the prevention of stroke have advanced considerably in the last several years. There can also be a role for mechanical restoration of the lumen by endarterectomy or stenting in selected patients with high-grade atherosclerotic stenosis of the extracranial carotid artery. Endarterectomy is generally recommended for patients with high-grade symptomatic carotid stenosis. Stenting is considered an option for patients at high risk of complications with endarterectomy. Whether revascularization is better than contemporary medical therapy for asymptomatic extracranial carotid stenosis is a subject of several ongoing randomized clinical trials in the United States and internationally.

Providing the correct level of care for patients with intracerebral hemorrhage (ICH) is crucial, but the level of care needed at initial presentation may not be clear. This study evaluated factors associated with admission to intensive care unit (ICU) level of care. This is an observational study of all adult patients admitted to our institution with non-traumatic supratentorial ICH presenting within 72 h of symptom onset between 2009–2012 (derivation cohort) and 2005–2008 (validation cohort). Factors associated with neuroscience ICU admission were identified via logistic regression analysis, from which a triage model was derived, refined, and retrospectively validated. For the derivation cohort, 229 patients were included, of whom 70 patients (31 %) required ICU care. Predictors of neuroscience ICU admission were: younger age [odds ratio (OR) 0.94, 95 % CI 0.91–0.97; p = 0.0004], lower Full Outline of UnResponsiveness (FOUR) score (0.39, 0.28–0.54; p

Cervical artery dissection is an underrecognized cause of lower cranial neuropathies and diagnosis can remain elusive if not properly investigated. We present a case of an internal carotid artery dissection that was initially missed in a 48-year-old man who presented with subacute-onset of dysarthria, dysphagia, and unilateral tongue weakness. Knowledge of the most common presenting symptoms, relevant neuroanatomy, and neuroimaging techniques is essential to avoid misdiagnosis. Pseudoaneurysm formation from subadventitial carotid artery dissection may result in compressive neuropathies of cranial nerves IX, X, XI, and XII without associated cerebral ischemia. The absence of intraluminal narrowing on CT or MR angiography should not dissuade the clinician; T1-weighted axial cervical MRI with fat-saturation provides the highest sensitivity and specificity to identify these lesions.

The causes of ischemic cerebrovascular disorders and cerebral infarction can be classified according to the site of the source for the arterial blockage within the vascular system, from most proximal to distal: cardiac source, large-vessel disorders, small-vessel occlusive disease, and hematologic disorders.

Objectives Nontraumatic convexal subarachnoid hemorrhages in the elderly can be a manifestation of cerebral amyloid angiopathy associated with a high risk of future intracerebral hemorrhage. The incidence in the elderly population is unknown. Our objectives were to: 1) determine the incidence of convexal subarachnoid hemorrhage in a population-based study, and, 2) to compare apopolipoprotein-E genotype and amyloid positron emission tomographic (PET) imaging for those with versus without hemorrhage. Methods Between 11/29/2004 and 3/11/2017, 4462 individuals without hemorrhage at baseline participated in the population-based Mayo Clinic Study of Aging. We used the Rochester Epidemiology Project medical records-linkage system to identify intracerebral hemorrhages. Records and images were reviewed to identify convexal subarachnoid hemorrhage. Neuroimaging characteristics, demographics, medications, and apopolipoprotein-E genotype were recorded. Results Four cases were identified. The incidence of convexal subarachnoid hemorrhage was 14.1 per 100,000 person years. Three occurred in women, median age, 79 (range: 71-84). One patient had coexisting cerebral microbleeds. Two participants developed a subsequent lobar intracerebral hemorrhage at a median of 4.75 years after convexal subarachnoid hemorrhage. The apopolipoprotein-E -allele combinations of the 4 were: 3/3, 3/3, 2/2, and 2/3. On Pittsburgh Compound B-PET imaging, median standardized uptake value ratio with convexal subarachnoid hemorrhage was 1.86 (range: 1.38-2.34). Conclusions Convexal subarachnoid hemorrhage is rare in the older population, occurring with an incidence of about 14 per 100,000 person years. Yet, when present, it may be associated with a high risk of future intracerebral hemorrhage.

Internal carotid artery pseudoaneurysm is an uncommon complication of retropharyngeal and parapharyngeal abscess in children. Treatment of the pseudoaneurysm has evolved in recent years from surgical ligation to endovascular techniques. Neurologic sequelae most commonly consist of Horner’s syndrome with cerebral ischemia being uncommon. The clinical course of a 2-year-old boy with retropharyngeal abscess complicated by internal carotid artery pseudoaneurysm, is described and the literature is reviewed. A conventional angiogram confirmed the presence of a large pseudoaneurysm with no anterograde flow distal to the pseudoaneurysm and substantial collateral flow across the circle of Willis, with filling of the left anterior and middle cerebral arteries via the anterior and posterior communicating arteries. Endovascular occlusion resulted in nonfilling of the left internal carotid artery, pseudoaneurysm, and left internal jugular vein at the base of the skull. Following the procedure, the patient developed transient mild right hemiparesis associated with frontal lobe ischemia.

A 73-year-old man was admitted for evaluation of acute ischemic strokes. His medical history was notable for pulmonary sarcoidosis treated with oral prednisone 40 mg daily, type 2 diabetes mellitus, hypertension, and hyperlipidemia. Approximately 1 month prior to admission, he underwent elective right total knee arthroplasty at an outside institution and 1 week postoperatively developed acute delirium and fluctuating fevers. No infectious cause was identified. His mental status continued to decline and head MRI was obtained (figure, A), showing small acute infarcts in multiple vascular territories.

Objectives To estimate the risk of fatal and non-fatal myocardial infarction (MI) and stroke in patients with bipolar I disorder compared to people without bipolar I disorder. Method Utilizing a records-linkage system spanning 30 years (1966–1996), a population-based cohort of 334 subjects with bipolar I disorder and 334 age and sex-matched referents from Olmsted County, Minnesota, U.S. was identified. Longitudinal follow-up continued until incident MI or stroke (confirmed by board-certified cardiologist/neurologist), death, or study end date (December 31, 2013). Cox proportional hazards models assessed the hazard ratio (HR) for MI or stroke, adjusting for potential confounders. Results There was an increased risk of fatal or non-fatal MI or stroke (as a composite outcome) in patients with bipolar I disorder [HR 1.54, 95% confidence interval (CI) 1.02, 2.33; p=0.04]. However, after adjusting for baseline cardiovascular risk factors (alcoholism, hypertension, diabetes, and smoking), the risk was no longer significantly increased (HR 1.19, 95% CI 0.76, 1.86; p =0.46). Limitations Small sample size for the study design. Findings were not retained after adjustment for cardiovascular disease risk factors. Psychotropic medication use during the follow-up was not ascertained and was not included in the analyses. Conclusion This study in a geographically defined region in the U.S. demonstrated a significant increased risk of MI or stroke in bipolar I disorder, which was no longer significant after adjustment for cardiovascular risk factors.