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The coronavirus disease (COVID-19) pandemic has impacted many lives globally. Neurologic manifestations have been observed among individuals at various stages and severity of the disease, the most common being stroke. Prompt identification of these neurologic diagnoses can affect patient management and prognosis. This article discusses the acute neuroradiological features typical of COVID-19, including cerebrovascular disease, intracerebral hemorrhage, leukoencephalopathy, and sensory neuropathies.

Sex as a variable of survival

One-way ANOVA analysis comparing expression of cytokines between Avelumab treated and Avelumab combined with Bevacizumab

Purpose:The treatment of glioblastoma (GBM) poses challenges. The use of immune checkpoint inhibition (ICI) has been disappointing as GBM is characterized by low mutational burden and low T-cell infiltration. The combination of ICI with other treatment modalities may improve efficacy.Patient and Methods:Patients with recurrent GBM were treated with avelumab, a human IgG1 antibody directed against PD-L1 (part A), or avelumab within a week after laser interstitial thermal therapy (LITT) and continuation of avelumab (part B). Bevacizumab was allowed to be combined with ICI to spare steroid use. The primary objective was to characterize the tolerability and safety of the regimens. The secondary objectives included overall survival, progression-free survival (PFS), signatures of plasma analytes, and immune cells.Results:A total of 12 patients (median age 64; range, 37–73) enrolled, five in part A and seven in part B. Two serious adverse events occurred in the same patient, LITT treated, not leading to death. The median survival from enrollment was 13 months [95% confidence interval (CI), 4–16 months] with no differences for part A or B. The median PFS was 3 months (95% CI, 1.5–4.5 months). The decrease in MICA/MICB, γδT cells, and CD4+ T cell EMRA correlated with prolonged survival.Conclusions:Avelumab was generally well tolerated. Adding bevacizumab to ICI may be beneficial by lowering cytokine and immune cell expression. The development of this combinatorial treatment warrants further investigation. Exploring the modulation of adaptive and innate immune cells and plasma analytes as biomarker signatures may instruct future studies in this dismal refractory disease.Significance:Our phase I of PD-L1 inhibition combined with LITT and using bevacizumab to spare steroids had a good safety profile for recurrent GBM. Developing combinatory treatment may help outcomes. In addition, we found significant immune modulation of cytokines and immune cells by bevacizumab, which may enhance the effect of ICI.

Wernicke’s encephalopathy (WE) is a life-threatening neurologic disorder resulting from thiamine (vitamin B1) deficiency that can be secondary to chronic alcohol abuse, gastrointestinal surgery, systemic infectious and non-infectious diseases, and chemotherapy. WE is classically characterized on MRI by reduced diffusion and T2 prolongation along the mammillothalamic tracts, periaqueductal gray and tectal plate. We present two patients with acute WE who had baseline arterial spin labeling (ASL) perfusion at the time of presentation, demonstrating increase in cerebral blood flow (CBF) within the classically involved brain regions and concurrent global cerebral cortical hypoperfusion. Both patients were successfully treated with intravenous thiamine infusion. Post-treatment MRI demonstrated improvement of reduced diffusion and normalization of CBF within the involved structures. Prior histopathological studies have documented prominent undulation and luminal dilatation of arteries and arterioles in acute WE lesions, likely explaining the increased perfusion shown by imaging. The root of this pathophysiologic process may trace back to thiamine’s biochemical role in maintaining osmotic gradients and glucose metabolism, that if failed can lead to arterial hyper-perfusion. Our findings show that ASL-CBF can highlight the underlying pathophysiology in patients with acute WE by demonstrating increased CBF in involved central structures. This luxury perfusion may be a compensatory or protective mechanism by which increased metabolic demand is met in the acute setting and which, if treated timely, will show normalization of CBF on ASL imaging.

Contains fulltext : 290934.pdf (Publisher’s version ) (Open Access) DDX3X syndrome is a surprisingly common newly discovered genetic neurodevelopmental disorder associated with intellectual disability, autism spectrum disorder, language delays, attention-deficit/hyperactivity disorder, and medical comorbidities. Two hundred individuals with DDX3X syndrome have been described in the literature to date, with varied levels of detail. Individuals with DDX3X syndrome often have complex presentations including symptoms in the neurological, psychiatric/psychological, ophthalmologic, and gastrointestinal domains. Owing to this complex presentation, an overview of symptom prevalence, medical recommendations, and suggested medical surveillance is vital for the care and health of individuals with DDX3X syndrome. In this article, we summarize the present clinical knowledge of DDX3X syndrome and provide recommendations for clinical assessments and care based on a comprehensive review of the existing literature and of new, not yet published DDX3X syndrome cohorts. As more is learned about DDX3X syndrome, we anticipate that these recommendations will evolve. 01 januari 2023

Background: Reduced cerebral blood flow in parieto-occipital regions has been reported in neurodegenerative disorders using ASL. We aimed to investigate neuropsychiatric and neurodegenerative comorbidities that may associate with parieto-occipital region hypoperfusion. Methods: This was a retrospective single-center study. Between March 2017 to May 2018, adult patients who underwent brain MRI with the inclusion of ASL perfusion and who had bilateral reductions of CBF in the parieto-occipital regions were included. ASL was performed using a pseudo-continuous arterial spin labeling (pCASL) technique on 1.5T MR system. Age and gender-matched patients with no perfusion defect were concurrently collected. Comorbidity data was collected from EMR, including major depressive disorder, Alzheimer’s disease, Parkinson’s disease, Schizophrenia, anxiety disorder, hypertension, diabetes mellitus type II, coronary artery disease, and chronic kidney disease. A Pearson’s Chi-Square test was performed to assess for comorbidities associated with hypoperfusion of the parieto-occipital lobes. Results: Our patient cohort consisted of 93 patients with bilateral hypoperfusion in the parieto-occipital lobes and 93 age and gender-matched patients without corresponding perfusion defects based on ASL-CBF. Among the comorbidities assessed, there was a statistically significant association between hypoperfusion of the parieto-occipital lobes and major depressive disorder (p=0.004) and Parkinson’s disease (p=0.044). There was no statistically significant association for Alzheimer’s disease, generalized anxiety disorder, diabetes mellitus type II, hypertension, coronary artery disease, or chronic kidney disease. Conclusion: Major depressive disorder may be linked to regional parieto-occipital hypoperfusion on ASL.

Coronavirus disease 2019 (COVID-19), a clinical manifestation of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), was declared a global pandemic by the World Health Organization on March 11, 2020. Hypercoagulable state has been described as one of the hallmarks of SARS-CoV-2 infection and has been reported to manifest as pulmonary embolisms, deep vein thrombosis, and arterial thrombosis of the abdominal small vessels. Here we present cases of arterial and venous thrombosis pertaining to the head and neck in COVID-19 patients., Highlights • We present cases: intracranial and extracranial large vessel occlusions, hemorrhagic strokes, and venous sinus thrombosis. • It is important to recognize possible neurovascular complications that can be seen in COVID-19 patients on imaging as early detection and management can contribute to effective response.

We present a case of brain death in a vaccinated, immunocompromised patient who presented with COVID-19 pneumonia. Imaging was characterized by diffuse cerebral edema, pseudo-subarachnoid hemorrhage, and no antegrade flow above the terminal internal carotid arteries. To our knowledge, this is the first case report with such findings in a vaccinated patient.

Occlusion of foramen of Monro is an uncommon clinical entity that usually presents in children. Common causes are obstructing mass, infectious etiologies or vascular malformation. Rarely, it may be an idiopathic stricture or membrane. We report a case of idiopathic membranous obstruction of the foramen of Monro in a 45-year-old male with no past medical or surgical history. He presented with new intermittent dull and burning bifrontal severe headache for 2 d, which was alleviated slightly by non-steroidal anti-inflammatory medication. Imaging showed marked dilation of the lateral ventricles with normal third and fourth ventricles. The patient was discharged initially with conservative medical management and close follows up; however, the headache continued to progress and neurosurgical intervention was offered. The patient underwent endoscopic exploration, fenestration of the septum pellucidum, and right ventriculoperitoneal shunt placement. Bilateral membranous obstruction of foramina of Monro and an auto-fenestrated cavum septum pellucidum were identified intraoperatively. The patient reported resolution of headache post-operatively without recurrence on 1-month follow up. This case is unusual in that the patient presented without any known neurologic history or prior intracranial infections. It became apparent at the time of surgery that chronic obstruction of the bilateral foramina with collapse of the third ventricle had developed, and the safest durable treatment for him was septostomy and cerebrospinal fluid (CSF) shunt placement.

Background DDX3X syndrome is a recently identified genetic disorder that accounts for 1–3% of cases of unexplained developmental delay and/or intellectual disability (ID) in females, and is associated with motor and language delays, and autism spectrum disorder (ASD). To date, the published phenotypic characterization of this syndrome has primarily relied on medical record review; in addition, the behavioral dimensions of the syndrome have not been fully explored. Methods We carried out multi-day, prospective, detailed phenotyping of DDX3X syndrome in 14 females and 1 male, focusing on behavioral, psychological, and neurological measures. Three participants in this cohort were previously reported with limited phenotype information and were re-evaluated for this study. We compared results against population norms and contrasted phenotypes between individuals harboring either (1) protein-truncating variants or (2) missense variants or in-frame deletions. Results Eighty percent (80%) of individuals met criteria for ID, 60% for ASD and 53% for attention-deficit/hyperactivity disorder (ADHD). Motor and language delays were common as were sensory processing abnormalities. The cohort included 5 missense, 3 intronic/splice-site, 2 nonsense, 2 frameshift, 2 in-frame deletions, and one initiation codon variant. Genotype–phenotype correlations indicated that, on average, missense variants/in-frame deletions were associated with more severe language, motor, and adaptive deficits in comparison to protein-truncating variants. Limitations Sample size is modest, however, DDX3X syndrome is a rare and underdiagnosed disorder. Conclusion This study, representing a first, prospective, detailed characterization of DDX3X syndrome, extends our understanding of the neurobehavioral phenotype. Gold-standard diagnostic approaches demonstrated high rates of ID, ASD, and ADHD. In addition, sensory deficits were observed to be a key part of the syndrome. Even with a modest sample, we observe evidence for genotype–phenotype correlations with missense variants/in-frame deletions generally associated with more severe phenotypes.

BackgroundDDX3X syndrome is a recently identified genetic disorder that accounts for 1-3% of cases of unexplained developmental delay (DD) and/or intellectual disability (ID) in females and is associated with motor and language delays, and autism spectrum disorder (ASD). To date, the published phenotypic characterization of this syndrome has primarily relied on medical record review; in addition, the behavioral dimensions of the syndrome have not been fully explored.MethodsWe carried out multi-day, prospective, detailed phenotyping of DDX3X syndrome in 14 females and 1 male, focusing on behavioral, psychological, and neurological measures; three participants in this cohort have been previously reported. We compared results against population norms and contrasted phenotypes between individuals harboring either (i) protein-truncating variants or (ii) missense variants and in-frame deletions.ResultsEighty percent of individuals met criteria for ID, 60% for ASD and 53% for attention-deficit/hyperactivity disorder (ADHD). Motor and language delays were common as were sensory processing abnormalities. The cohort included 5 missense, 3 intronic/splice-site, 2 nonsense, 2 frameshift, 2 in-frame deletions, and one initiation codon variant. Genotype-phenotype correlations indicated that missense variants/in-frame deletions were associated with more severe language, motor, and adaptive deficits in comparison to protein-truncating variants.LimitationsSample size is modest, however, DDX3X is a rare and underdiagnosed disorder.ConclusionThis study, representing a first, prospective, detailed characterization of DDX3X syndrome, extends our understanding of the neurobehavioral phenotype. Gold-standard diagnostic approaches demonstrated high rates of ID, ASD, and ADHD. In addition, sensory deficits were observed to be a key part of the syndrome. Even with a modest sample, we observe evidence for genotype-phenotype correlations with missense variants/in-frame deletions yielding a more severe phenotype.

Coronavirus disease 2019 (COVID-19) is a global pandemic, and it is increasingly important that physicians recognize and understand its atypical presentations. Neurological symptoms such as anosmia, altered mental status, headache, and myalgias may arise due to direct injury to the nervous system or by indirectly precipitating coagulopathies. We present the first COVID-19 related cases of carotid artery thrombosis and acute PRES-like leukoencephalopathy with multifocal hemorrhage., Highlights • COVID-19 may manifest with neurological symptoms • SARS-CoV-2 may have direct neurologic effects due to endothelial invasion, such as our case of PRES-like leukoencephalopathy. • Indirect effects may include coagulation cascade disruption, resulting in unusual thrombosis and large vessel occlusions.

Background Physiologic changes quantified by diffusion and perfusion MRI have shown utility in predicting treatment response in glioblastoma (GBM) patients treated with cytotoxic therapies. We aimed to investigate whether quantitative changes in diffusion and perfusion after treatment by immune checkpoint inhibitors (ICIs) would determine 6-month progression-free survival (PFS6) in patients with recurrent GBM. Methods Inclusion criteria for this retrospective study were: (i) diagnosis of recurrent GBM treated with ICIs and (ii) availability of diffusion and perfusion in pre and post ICI MRI (iii) at ≥6 months follow-up from treatment. After co-registration, mean values of the relative apparent diffusion coefficient (rADC), Ktrans (volume transfer constant), Ve (extravascular extracellular space volume) and Vp (plasma volume), and relative cerebral blood volume (rCBV) were calculated from a volume-of-interest of the enhancing tumor. Final assignment of stable/improved versus progressive disease was determined on 6-month follow-up using modified Response Assessment in Neuro-Oncology criteria. Results Out of 19 patients who met inclusion criteria and follow-up (mean ± SD: 7.8 ± 1.4 mo), 12 were determined to have tumor progression, while 7 had treatment response after 6 months of ICI treatment. Only interval change of rADC was suggestive of treatment response. Patients with treatment response (6/7: 86%) had interval increased rADC, while 11/12 (92%) with tumor progression had decreased rADC (P = 0.001). Interval change in rCBV, Ktrans, Vp, and Ve were not indicative of treatment response within 6 months. Conclusions In patients with recurrent GBM, interval change in rADC is promising in assessing treatment response versus progression within the first 6 months following ICI treatment. Key Points • In recurrent GBM treated with ICIs, interval change in rADC suggests early treatment response. • Interval change in rADC can be used as an imaging biomarker to determine PFS6. • Interval change in MR perfusion and permeability measures do not suggest ICI treatment response.

BACKGROUND. An increase in frequency of acute ischemic strokes has been observed among patients presenting with acute neurologic symptoms during the coronavirus disease (COVID-19) pandemic. OBJECTIVE. The purpose of this study was to investigate the association between COVID-19 and stroke subtypes in patients presenting with acute neurologic symptoms. METHODS. This retrospective case-control study included patients for whom a code for stroke was activated from March 16 to April 30, 2020, at any of six New York City hospitals that are part of a single health system. Demographic data (age, sex, and race or ethnicity), COVID-19 status, stroke-related risk factors, and clinical and imaging findings pertaining to stroke were collected. Univariate and multivariate analyses were conducted to evaluate the association between COVID-19 and stroke subtypes. RESULTS. The study sample consisted of 329 patients for whom a code for stroke was activated (175 [53.2%] men, 154 [46.8%] women; mean age, 66.9 ± 14.9 [SD] years). Among the 329 patients, 35.3% (116) had acute ischemic stroke confirmed with imaging; 21.6% (71) had large vessel occlusion (LVO) stroke; and 14.6% (48) had small vessel occlusion (SVO) stroke. Among LVO strokes, the most common location was middle cerebral artery segments M1 and M2 (62.0% [44/71]). Multifocal LVOs were present in 9.9% (7/71) of LVO strokes. COVID-19 was present in 38.3% (126/329) of the patients. The 61.7% (203/329) of patients without COVID-19 formed the negative control group. Among individual stroke-related risk factors, only Hispanic ethnicity was significantly associated with COVID-19 (38.1% of patients with COVID-19 vs 20.7% of patients without COVID-19; p = 0.001). LVO was present in 31.7% of patients with COVID-19 compared with 15.3% of patients without COVID-19 (p = 0.001). SVO was present in 15.9% of patients with COVID-19 and 13.8% of patients without COVID-19 (p = 0.632). In multivariate analysis controlled for race and ethnicity, presence of COVID-19 had a significant independent association with LVO stroke (odds ratio, 2.4) compared with absence of COVID-19 (p = 0.011). CONCLUSION. COVID-19 is associated with LVO strokes but not with SVO strokes. CLINICAL IMPACT. Patients with COVID-19 presenting with acute neurologic symptoms warrant a lower threshold for suspicion of large vessel stroke, and prompt workup for large vessel stroke is recommended.

We present a case of an infundibular dilation at the origin of an accessory middle cerebral artery emanating from the distal A1 segment of the anterior cerebral artery. There was also partial vessel wall enhancement along this infundibulum. To our knowledge, this is the first case report with such findings.

Background and purpose Despite increasing demand for fluoroscopy-guided lumbar puncture (FG-LP), there is limited quantitative and epidemiological data on patients undergoing this procedure. Additionally, data are scarce on the correlation of iliac crest landmarks to the actual anatomical lumbar level (intercristal line). The aim of this study is to determine if (1) body mass index (BMI) correlates with skin to spinal canal distance (SCD) and (2) the iliac crest landmark correlates with the presumed anatomical landmark on cross-sectional imaging. Methods In this retrospective, single-center IRB-approved study, we assessed 495 patients who underwent FG-LP and had lumbar computed tomography/magnetic resonance imaging within 6 months of presentation. SCD was measured on the sagittal view at the L3-L4, L4-L5, and L5-S1 intervertebral levels. Results In our cohort of 495 adults (mean age ± standard deviation [SD], 53.2 ± 16.4 years), there was a statistically significant linear correlation between BMI and SCD at each intervertebral level. Mean ± SD (R2 ) SCD at L3-4, L4-5, and L5-S1 was 6.7 ± 1.6 cm (.5486), 7.4 ± 1.9 cm (.5894), and 7.8 ± 1.9 cm (.5968), respectively. The intercristal line aligned with L3-L4, L4-L5, and L5-S1 in 2.1%, 72.4%, and 6.2% of patients, respectively. Conclusions There was direct, positive linear correlation between BMI and SCD at clinically relevant lumbar disc levels. Furthermore, there is considerable anatomical variance in the intervertebral space that aligns with the superior aspect of the iliac crest.

ObjectiveThe usage of arterial spin labelling (ASL) perfusion has exponentially increased due to improved and faster acquisition time and ease of postprocessing. We aimed to report potential additional findings obtained by adding ASL to routine unenhanced brain MRI for patients being scanned in a hospital setting for various neurological indications.DesignRetrospective.SettingLarge tertiary hospital.Participants676 patients.Primary outcomeAdditional findings from ASL sequence compared with conventional MRI.ResultsOur patient cohorts consisted of 676 patients with 257 with acute infarcts and 419 without an infarct. Additional findings from ASL were observed in 13.9% (94/676) of patients. In the non-infarct group, additional findings from ASL were observed in 7.4% (31/419) of patients, whereas in patients with an acute infarct, supplemental information was obtained in 24.5% (63/257) of patients.ConclusionThe addition of an ASL sequence to routine brain MRI in a hospital setting provides additional findings compared with conventional brain MRI in about 7.4% of patients with additional supplementary information in 24.5% of patients with acute infarct.

BackgroundAuthors have noticed an increase in lung apex abnormalities on CT angiography (CTA) of the head and neck performed for stroke workup during the coronavirus disease 2019 (COVID-19) pandemic.ObjectiveTo evaluate the incidence of these CTA findings and their relation to COVID-19 infection.MethodsIn this retrospective multicenter institutional review board-approved study, assessment was made of CTA findings of code patients who had a stroke between March 16 and April 5, 2020 at six hospitals across New York City. Demographic data, comorbidities, COVID-19 status, and neurological findings were collected. Assessment of COVID-19 related lung findings on CTA was made blinded to COVID-19 status. Incidence rates of COVID-19 related apical findings were assessed in all code patients who had a stroke and in patients with a stroke confirmed by imaging.ResultsThe cohort consisted of a total of 118 patients with mean±SD age of 64.9±15.7 years and 57.6% (68/118) were male. Among all code patients who had a stroke, 28% (33/118) had COVID-19 related lung findings. RT-PCR was positive for COVID-19 in 93.9% (31/33) of these patients with apical CTA findings.Among patients who had a stroke confirmed by imaging, 37.5% (18/48) had COVID-19 related apical findings. RT-PCR was positive for COVID-19 in all (18/18) of these patients with apical findings.ConclusionThe incidence of COVID-19 related lung findings in stroke CTA scans was 28% in all code patients who had a stroke and 37.5% in patients with a stroke confirmed by imaging. Stroke teams should closely assess the lung apices during this COVID-19 pandemic as CTA findings may be the first indicator of COVID-19 infection.

BACKGROUND AND PURPOSE: Coronavirus disease 2019 (COVID-19) is an active worldwide pandemic with diverse complications. Stroke as a presentation has not been strongly associated with COVID-19. The authors aimed to retrospectively review a link between COVID-19 and acute stroke. MATERIALS AND METHODS: We conducted a retrospective case-control study of 41 cases and 82 control subjects matched by age, sex, and risk factors. Cases were patients who underwent stroke alert imaging with confirmed acute stroke on imaging between March 16 and April 5, 2020, at 6 hospitals across New York City. Control subjects were those who underwent stroke alertimaging during the same timeframe without imaging evidence of acute infarction. Data pertaining to diagnosis of COVID-19 infection, patient demographics, and risk factors were collected. A univariate analysis was performed to assess the covariate effect of risk factors and COVID-19 status on stroke imaging with positive findings. RESULTS: The mean age for cases and controls was 65.5 ± 15.3 years and 68.8 ± 13.2 years, respectively. Of patients with acute ischemic stroke, 46.3% had COVID-19 infection compared with 18.3% of controls (P = .001). After adjusting for age, sex, and risk factors, COVID-19 infection had a significant independent association with acute ischemic stroke compared with control subjects (OR, 3.9; 95% CI, 1.7–8.9; P = .001). CONCLUSIONS: We demonstrated that COVID-19 infection is significantly associated with imaging confirmation of acute ischemic stroke, and patients with COVID-19 should undergo more aggressive monitoring for stroke.

Objectives To determine whether children with white coat hypertension (WCH) have evidence of target-organ damage by comparing the left ventricular mass index (LVMI) of subjects with WCH with that of matched normotensive and hypertensive controls. Study design Each subject in the WCH group was matched by body mass index (BMI; ± 10%), age (± 1 year), and sex to a normotensive control and a hypertensive control. Echocardiograms were reviewed to determine the LVMI for each subject. These triple matches were analyzed using repeated-measures analysis of variance to detect differences in LVMI among the 3 groups. Results A total of 27 matched triplets were established. The groups were comparable for sex, age, and BMI. Mean LVMI was 29.2 g/m 2.7 for the normotensive group, 32.3 g/m 2.7 for the WCH group, and 35.1 g/m 2.7 for the sustained hypertensive group (normotensive vs WCH, P = .028; WCH vs sustained hypertension, P = .07). Left ventricular hypertrophy was not present in any subject in the normotensive or WCH groups, but was found in 26% of the sustained hypertensive subjects ( P Conclusions After controlling closely for BMI, the LVMI in the subjects with WCH was between that of the normotensives and sustained hypertensives, suggesting that WCH may be associated with hypertensive end-organ effects.