Clinical presentation, natural history, and outcomes for infantile intracranial cavernous malformations: case series and systematic review of the literature.

INTRODUCTION: Intracranial cavernous malformations (CMs) are rare vascular malformations of the central nervous system in children. Infantile patients, being a developmentally vulnerable age group, pose a special challenge for management of these lesions. We pooled data from infantile patients diagnosed at our institution and individual cases published in the literature to provide input towards therapeutic decision-making. METHODS: A systematic search of PubMed, MEDLINE, Embase, and Scopus was performed in accordance with PRISMA guidelines to identify all reported cases of intracranial CMs in the literature for infantile patients aged ≤ 2 years. In addition, cases from our institution diagnosed between 2010 and 2020 were also included. Individual cases were pooled and analyzed for clinical presentation, natural history, and outcomes from conservative and surgical management. RESULTS: A total of 36 cases were included, of which 32 were identified from the literature. Median age at presentation was 14 months (range: 2 days to 24 months) months; 53% (n = 19) were females. Most cavernomas (64%, 23/36) were supratentorial, while 30% (n = 11) were located in brainstem and 5.5% (n = 2) in the cerebellum. With the exception of one patient, all cases were reported to be symptomatic; seizures (n = 15/31, 48.3%) and motor deficits (n = 13/31, 42%) were the most common symptom modalities. A total of 13 patients were managed conservatively upon initial presentation. No symptomatic hemorrhages were observed during 26 total person-years of follow-up. A total of 77% (28/36) underwent surgery; either upfront (23/28, 82%) at initial presentation or following conservative management. Among 12 patients who had preoperative seizures, 11/12 (91.6%) achieved seizure freedom post-resection. Among 7 patients who presented with hemiparesis preoperatively, 5 (71%) demonstrated some improvement, while 1 remained unchanged, and another patient with a brainstem cavernous malformation had worsening of motor function postoperatively. Postoperative recurrence was noted in 3 cases (3/27, 11%). CONCLUSION: Annual risk of repeat hemorrhage may be low for infantile patients with intracranial cavernous malformations; however, better follow-up rates and higher number of cases are needed to make a definitive assertion. Surgical resection may be associated with high rates of epilepsy cure and provide improvement in neurological function in a select number of cases.

Intracranial Aneurysms in Patients with Marfan Syndrome: A Multicenter Propensity-Matched Analysis.

OBJECTIVE: We sought to identify risk factors for intracranial aneurysms (IAs) in Marfan syndrome (MFS) patients and to describe their characteristics. METHODS: Patients with confirmed MFS and vessel-dedicated brain imaging from January 1, 1980-December 1, 2020 were categorized according to the presence (IA) or absence (NIA) of IAs. Unmatched logistic regression analysis and propensity score matching were used for comparison. RESULTS: We included 159 patients, of whom 18 (11.3%) patients had radiographic diagnosis of IAs. Three patients (16.7%) had multiple lesions for a total of 24 IAs. One patient (5.5%) had de novo IA formation. Four patients (22.2%) underwent treatment: Two (11.1%) had open surgical clipping for ruptured aneurysms, and two (11.1%) patients had endovascular embolization for growth. In the unmatched analysis, current tobacco smoker status (odds ratio [OR]: 4.20; confidence interval [CI]: 1.11-15.6; P = 0.027) and history of coronary artery disease (CAD) (OR: 5.79; CI 1.76-20.2; P = 0.004) increased the odds for IA. Propensity score matching yielded 18 IA and 18 NIA patients matched for age, gender, race, prior stroke, and family history of aneurysms. History of CAD (IA = 11 [61.1%] vs. NIA = 4 (22.2%), P = 0.043) and current smoker status (IA = 6 [33.3%] vs. NIA = 0 (0%), P < 0.01) were significantly higher in the IA cohort. Body mass index (P = 0.622), diabetes (P = 0.180), hypertension (P = 0.732), prior stroke (P = 1.00), family history (P = 0.732), alcohol (P = 0.314), recreational drugs (P = 1.00), and other aneurysms (P = 0.585) were not statistically significant. CONCLUSIONS: Prevalence of IAs in our series of MFS patients was 11.3%, and de novo formation was 5.5%. MFS patients with a history of CAD and current smoker status had an increased risk of IA. Neurovascular radiographic screening should be considered in all patients with MFS, particularly in patients who smoke or have a history of heart disease.

Telestroke in the Time of COVID-19: The Mayo Clinic Experience.

On March 11, 2020, the World Health Organization declared the coronavirus disease 2019 (COVID-19) a pandemic, and in the weeks following, public health organizations, medical associations, and governing bodies throughout the world recommended limiting contact with others to "flatten the curve" of COVID-19. Although both ischemic and hemorrhagic strokes have been reported with COVID-19, there has been anecdotal suggestion of an overall decrease in stroke admissions. To date, the effects of any pandemic on telestroke service lines have not been described. The purpose of this cross-sectional analysis of telestroke activations in the 30 days before and after the declaration of the COVID-19 pandemic is to describe the difference in case volumes of telestroke activations, the characteristics of patients, and treatment recommendations between the 2 time frames. We found a 50.0% reduction in total telestroke activations between the predeclaration group (142 patients) and the postdeclaration group (71 patients). There were no statistically significant differences in age (P=.95), sex (P=.10), diagnosis (P=.26), or regional variations (P=.08) in activation volumes. The percentage of patients for whom we recommended urgent stroke treatment with intravenous alteplase, mechanical thrombectomy, or both decreased from 44.4% (28 of 63) to 33.3% (11 of 33). The reasons for the sunstantial decrease in telestroke activations and urgent stroke treatment recommendations are likely multifactorial but nevertheless underscore the importance of continued public health measures to encourage patients and families to seek emergency medical care at the time of symptom onset.

Clinical presentation, natural history and outcomes of intramedullary spinal cord cavernous malformations.

OBJECTIVE: There is a paucity of literature investigating the clinical course of patients with spinal intramedullary cavernous malformations (ISCMs). We present a large case series of ISCMs to describe clinical presentation, natural history and outcomes of both surgical and conservative management. METHODS: We retrospectively reviewed the clinical course of patients diagnosed with ISCMs at our institution between 1995 and 2016. Haemorrhage was defined as clinical worsening in tandem with imaging changes visualised on follow-up MRI. Outcomes assessed included neurological status and annual haemorrhage rates. RESULTS: A total of 107 patients met inclusion criteria. Follow-up data were available for 85 patients. While 21 (24.7%) patients underwent immediate surgical resection, 64 (75.3%) were initially managed conservatively. Among this latter group, 16 (25.0%) suffered a haemorrhage during follow-up and 11 (17.2%) required surgical resection due to interval bleeding or neurological worsening. The overall annual risk of haemorrhage was 5.5% per person year. The rate among patients who were symptomatic and asymptomatic on presentation was 9.5% and 0.8%, respectively. Median time to haemorrhage was 2.3 years (0.1-12.3). Univariate analysis identified higher ISCM size (p=0.024), history of prior haemorrhage (p=0.013) and presence of symptoms (p=0.003) as risk factors for subsequent haemorrhage. Multivariable proportional hazards analysis revealed presence of symptoms to be independently associated with haemorrhage during follow-up (HR 9.39, CI 1.86 to 170.8, p=0.013). CONCLUSION: Large, symptomatic ISCMs appear to be at increased risk for subsequent haemorrhage. Surgery may be considered in such lesions to prevent rebleeding and subsequent neurological worsening.

Mild Carotid Stenosis with Recurrent Symptoms Triggered by Eating.

BACKGROUND: Carotid atherosclerosis is a known risk factor for acute ischemic stroke. Although severe luminal narrowing is a well-established risk factor for future ischemic events, 10% of patients with acute ischemic stroke or transient ischemic attacks are thought to have ischemic events secondary to vulnerable carotid plaques in the setting of mild to moderate carotid artery stenosis. CASE DESCRIPTION: A 78-year-old man presented with multiple recurrent strokes and transient ischemic attacks that consistently occurred with eating. Based on the anatomic relationships of the proximal internal carotid artery harboring the plaque, we hypothesized that multiple recurrent emboli occurred secondary to the mechanical effect of the esophagus "squeezing" the vulnerable plaque against the surrounding fixed tissues despite a mild degree of stenosis (approximately 20% stenosis on magnetic resonance angiography and angiography). Despite the mild stenosis, a carotid endarterectomy was performed, which resulted in immediate resolution of the ischemic events. CONCLUSIONS: We postulate that the likely mechanical "squeeze" of the vulnerable plaque involving the retropharyngeal proximal internal carotid artery was responsible for the multiple recurrent transient ischemic attacks, which characteristically occurred only while eating. Resolution of the episodes after carotid endarterectomy supports this hypothesis.

Diagnostic Dilemma in Primary Blastomyces dermatitidis Meningitis: Role of Neurosurgical Biopsy.

A 52-year-old male on chronic prednisone for polymyalgia rheumatica presented with a subacute history of headaches, nausea, phonophobia, intermittent diplopia and gait instability. He was hospitalized 2 weeks prior to presentation with extensive evaluations only notable for leptomeningeal inflammation on MRI. His symptoms progressively worsened and he developed aphasia. He was transferred to our facility where extensive spinal fluid examinations were repeated and were again nondiagnostic. Ultimately, a diagnostic skull-based biopsy was performed which demonstrated Blastomyces dermatitidis fungal meningitis. Despite extensive sampling and cultures, only 1 of the intraoperative samples yielded diagnostic results. This underscores the low sensitivity of current methods to diagnose CNS blastomycosis. This case suggests that a neurosurgical biopsy may be necessary and should be considered early in the diagnostic process, especially if a definitive diagnosis is elusive. If a biopsy is performed, sampling should be ample and from multiple areas. Following the diagnosis, our patient was treated with liposomal amphotericin B and then voriconazole with a good clinical response.

Failure of dabigatran and rivaroxaban to prevent thromboembolism in antiphospholipid syndrome: a case series of three patients.

Direct oral factor inhibitors (DOFIs) are an attractive alternative to vitamin K antagonists (VKA) for the treatment of patients with antiphospholipid syndrome (APS). In the absence of prospective, randomised trial data, reports of therapeutic failures in clinical practice alert clinicians to potential limitations of DOFI therapy for this indication. Data for all cases were collected from a centralised system that contains complete medical records of all patients treated and followed at Mayo Medical Center. We present here three consecutive APS patients who had had no thromboembolism recurrence on warfarin but were switched to DOFIs. The diagnosis of APS was established according to currently recommended criteria. The three cases were as follows: A woman with primary APS developed thrombotic endocarditis with symptomatic cerebral emboli after transition to dabigatran. A second woman with primary APS experienced ischemic arterial strokes and right transverse-sigmoid sinus thrombosis after conversion to rivaroxaban. A man with secondary APS suffered porto-mesenteric venous thrombosis after switching to rivaroxaban. None of these patients had failed warfarin prior to the transition to DOFIs. Based on these three cases, we advocate caution in using DOFIs for APS patients outside of a clinical trial setting, until further data becomes available.

Medication events on a tertiary neurology inpatient service.

This study aimed to determine the prevalence and attributes of medication events in a tertiary inpatient neurology setting. These findings were compared with the results of a physician survey designed to assess general knowledge of medication events. We reviewed all medication events reported through an established voluntary event recording system for adult neurology inpatients at a tertiary referral center over a 2 year period. An electronic survey was sent to all neurology residents and hospital staff regarding medication events. One hundred and forty-three medication events were reported, for a rate of 8.6 events per 1000 patient-days. The medication event rate was comparable to published rates on medicine services. There was no evidence of events being more prevalent at the beginning of the academic year or on nights. One hundred and seventeen events (81.8%) reached the patient, but most (92.7%, 108/117) did not result in harm. The most common medication types involved were anticoagulants (24 events, 16.8%), anticonvulsants (17 events, 11.9%), and antibiotics (16 events, 11.2%). Nurses were involved in reporting 133 events (93%), while physicians were involved in reporting 20 events (14%). Physicians completed the survey at a rate of 60.8% (48/79). There were notable differences between physician presumptions as to medication event attributes and actual results. No trend referable to time of year or day was identified, which contrasted with physician beliefs in our survey. Nursing staff reported the majority of events.

Management of unruptured intracranial aneurysms.

Unruptured intracranial aneurysms (UIA) are a common finding, occurring in about 2% of the population, making them very likely to be seen by most practitioners, and present a challenge in the recommendations for optimal management and screening. The consequences of aneurysm rupture are dire, with high likelihood of significant morbidity and mortality. Most aneurysms do not rupture and patients harboring these lesions often remain asymptomatic. There are effective surgical and endovascular interventions to prevent rupture, but these procedures carry a risk of adverse complications. This article addresses the challenges of screening and management of UIAs.

Laterality, region, and type of motor dysfunction correlate with cognitive impairment in Parkinson's disease.

We studied the relationship between two screening cognitive measures and off motor Unified Parkinson's Disease Rating Scale (UPDRS) scores in 108 Parkinson's disease patients. Multiple regressions were conducted to examine the UPDRS subscores' unique contributions to cognitive function. When including bradykinesia, rigidity, and postural/gait instability subscores, only bradykinesia predicted Mini Mental Status Examination (MMSE), normalized beta = -0.57, t(104) = -3.31, P < 0.01, and Dementia Rating Scale-2 (DRS-2), normalized beta = -0.45, t(104) = -2.55, P < 0.05. Tremor was not included in the regression analyses because it did not correlate with cognitive function. When including axial and appendicular subscores, only the axial subscore predicted MMSE, normalized beta = -0.39, t(105) = -3.19, P < 0.01, and DRS-2 scores, normalized beta = -0.40, t(106) = -3.28, P < 0.01. When including left-sided and right-sided subscores, only the right-sided symptoms predicted DRS-2 scores, normalized beta = -0.28, t(105) = -2.45, P < 0.05, and showed a trend toward predicting MMSE scores, normalized beta = -0.22, t(105) = -1.95, P = 0.054. We therefore found that right-sided symptoms (for laterality), axial symptoms (for region), and bradykinesia (for type of symptoms) were the best predictors of cognitive function.