Superior Cerebellar Artery Dissection in a Patient Diagnosed with Reversible Cerebral Vasoconstriction Syndrome: A Case Report

Arterial dissection is an uncommon complication of reversible cerebral vasocon-striction syndrome (RCVS). We describe the case of a 35-year-old woman with a migraine history who presented with recurrent thunderclap headache and focal neurological signs, including right hemiataxia. She had been diagnosed with COVID-19 disease two weeks earlier. Neuroimaging revealed multifocal stenosis of the posterior circulation arteries and dissection of the right superior cerebellar artery. She improved significantly throughout her one-week hospitalization and maintained only mild ataxia. The interplay between COVID-19 disease, RCVS, and arterial dissection requires further investigation.Copyright © Author(s) (or their employer(s)) and Sinapse 2022.

Transcranial Doppler Ultrasonography-Related Research in the Caribbean Region.

Transcranial Doppler (TCD) ultrasonography is a non-invasive ultrasound technique that uses high-frequency sound waves to measure blood flow velocities in the cerebral vasculature. This review analyzes TCD research in the Caribbean region using a bibliometric analysis of 29 articles from PubMed. The articles were analyzed using Microsoft Excel 2016 and the VOSviewer software (Van Eck and Waltman, Leiden University, Centre for Science and Technology Studies (CWTS), www.vosviewer.com) and characterized various aspects of TCD research, including countries, research themes, authorship, journals, affiliations, and keywords. The majority of the 29 publications came from Cuba (38%), followed by the French West Indies (22%) and Jamaica (20%). Most TCD research focused on sickle cell disease (SCD), accounting for 45% of the studies, followed by 21% of articles on vasospasm and subarachnoid hemorrhage. The use of TCD in brain death and neuro-intensive care was also explored, constituting 17% of the studies. Alternative TCD-monitored treatment options for SCD, such as stem cell transplantation and hydroxyurea, were also frequently investigated. The most productive institutions were Hospital Clínico-Quirúrgico Hermanos Ameijeiras in Havana, Cuba, the Sickle Cell Unit at the University of West Indies (UWI) Mona in Jamaica, the Medical-Surgical Research Center (CIMEQ) in Havana, Cuba, and the SCD Reference Center in Guadeloupe and Martinique in the French West Indies. TCD has been identified as a cost-effective tool for real-time monitoring of cerebral blood flow in many clinical settings, including stroke and SCD, which are prevalent in the Caribbean. Although there is an increase in the trend of using TCD for neuromonitoring in the Caribbean, gaps still exist. Capacity-building initiatives, such as training programs for healthcare providers and the development of local TCD research networks, can improve access to TCD in resource-constrained settings to treat and neuromonitor patients cost-effectively.

[Prehospital ultrasound and cardiological emergencies]. / Échographie préhospitalière et urgences cardiologiques.

Technological advances over the past two decades have paved the way for the prehospital use of ultrasound. This practice was first developed in traumatology and then in a multitude of other indications, including cardiology. The development of pulmonary ultrasound is certainly the most visible illustration of this. Firstly, because it is an extra-cardiac examination that provides the answer to a cardiac question. Secondly because from a theoretical point of view this ultrasound indication was a bad indication for the use of ultrasound due to the air contained in the thorax. Thirdly, because this indication has become a 'standard of care' when caring for a patient with dyspnea - a practice that has become widespread during the COVID epidemic. In patients with heart failure, ultrasound has a high diagnostic power (including for alternative diagnoses) which is all the more precise since the technique is non-invasive, the response is obtained quickly, the examination can be repeated at desire to follow the evolution of the patient. The main other indications for prehospital ultrasound are cardiac arrest to search for a curable cause, identification of residual mechanical cardiac activity, monitoring of cerebral perfusion; chest pain, for both positive and negative diagnoses; shock for the search for an etiology and therapeutic follow-up or even pulmonary embolism or ultrasound for the search for dilation of the right ventricle which is now at the forefront of the recommendation algorithm.

Cerebral vasospasm after subarachnoid hemorrhage from intracranial aneurysm rupture in SARS-CoV-2 positive patients. A retrospective review from two Bulgarian hospitals

Intracranial aneurysms are acquired lesions resulting from hemodynamic stress on the vascular wall. Their rupture usually presents as a subarachnoid hemorrhage (SAH) with a high mortality rate. Cerebral vasospasm follows, which leads in many cases to delayed brain ischemia and even death. We aimed to explore the relationship between cerebral perfusion and coronavirus disease (COVID-19) in aneurysmal SAH. We analyzed 42 cases that underwent treatment for proven SAH due to ruptured cerebral aneurysms between January 2020 and December 2021. The patients were treated adhering to a standard protocol. The cerebral vasospasm was assessed by measuring the difference in the mean arterial pressure of the carotid artery relative to the internal cerebral artery (Lindegaard's ratio) by transcranial Doppler ultrasound for 12 consecutive days. Twenty-three patients showed no signs of an acute respiratory syndrome associated with COVID-19 but tested positive for the SARS-coronavirus 2 (SARS-CoV-2). The control group included 19 SARS-CoV-2 negative cases. The mean age was 59.7 +/- 8.4 years (range 44-72), with 29 males and 13 females. The mean arterial pressure was without a significant difference of 89.3 +/- 3.3 to 89.7 +/- 3.7 mmHg in SARS-CoV-2 negative to positive patients. When viral infection was evident, we observed a higher Lindegaard's ratio of 2.12 +/- 0.36 than the control, with a value of 1.43 +/- 0.33 (p < 0.01). Thus, brain perfusion was 32.5% better in negative patients. We suggest that SARS-CoV-2 positive patients, without acute COVID-19, are more likely to have worse brain perfusion after SAH from cerebral aneurysm rupture.

MANAGEMENT OF SICKLE CELL DISEASE: A CLINICAL PRACTICE SURVEY

Background: Therapeutic options for Sickle Cell Disease (SCD) have increased recently as well as the development of updated national guidelines. It is not known how these options are being offered or to what degree guidelines are incorporated into clinical practice. Objectives: To assess the clinical practice patterns of providers treating children with SCD. Design/Method: A survey study was performed which included nine sections: clinic structure, prophylaxis, immunizations, hydroxyurea, splenic sequestration, stroke, novel therapies, potential curative therapies, and transition. Survey was disseminated over a three-month period via SurveyMonkey, to members of the American Society of Pediatric Hematology-Oncology Hemoglobinopathy Special Interest Group. Results: There were 86 respondents;most were attending/faculty (85%, 73/86) who were part of a university/academic practice (65%, 56/86). Program size was most commonly 50-250 patients (44%, 37/86). Accessibility to support staff in clinic included 95% (81/86) social work;76% (65/86) child life;68% (58/86) nurse coordinator and 34% (29/86) school liaison and 15% (13/86) transition navigator. For preventive care, 72% prescribe penicillin prophylaxis before 2 months of age recommending 100% (83) for HbSS and Sβnull, 72% (60/83) for HbSC and 70% (58/83) for HbSβplus. Influenza was the most common vaccine offered in clinic at 96% (76/79) with 91% (72/79) offering pneumococcal vaccines, 84% (67/79) offering meningococcal vaccines and 50% (40/79) offering COVID vaccines. Transcranial doppler screening was offered in 95% (69/73) but only 42% (31/73) performed MRI screening for silent stroke. Transfusion therapy was recommended for primary stroke prevention by 90% (65/72) and 84% (59/70) attempt to transition to hydroxyurea following TWITCH guidelines. For secondary stroke prevention, 88% (63/72) recommend chronic transfusion therapy. Regarding disease-modifying therapy, 90% (70/78) report starting hydroxyurea routinely in patients with HbSS and Sβnull;initiated at 9 months of age by 69% (54/78). Laboratory monitoring recommended every 3 months for stable dosing by 62% (49/78) and hydroxyurea held by 56% (44/78) if platelets <75,000, 73% (56/78) for neutrophils <1000. New therapies were recommended for patients on hydroxyurea who were still experiencing SCD complications: L-glutamine 68% (37/54;crizanlizumab 93% (54/58). Voxelotor was recommended for patients on hydroxyurea with low hemoglobin 65% (43/66). Matched sibling transplant was considered for any disease severity by 55% (38/69). Gene therapy trial is offered on-site by 29% (20/69). Transition programs were endorsed by 61% (42/69), but only 45% (31/68) had dedicated staff. Conclusion: This survey is the only assessment of the application of SCD guidelines in clinical practice.

Platypnea-orthodeoxia syndrome years after acute pulmonary embolism: Two consecutive cases

Platypnea-Orthodeoxia syndrome (POS) is a rare condition in which dyspnoea and arterial oxygen desaturation are present in the upright position, while in the supine position, they are alleviated. It is observed in the presence of an anatomical (intra-or extracardiac) communication between the right and left heart causing a right-to-left shunt. POS is most frequently caused by a patent foramen ovale (PFO) and usually, the clinical assessment and a transthoracic echocardiograms with bubble study are enough to reach the diagnosis. The only possible treatment of POS is the percutaneous closure of the defect. We describe two cases of POS due to a PFO which manifested itself years after an episode of acute pulmonary embolism (PE), a finding never reported to date in the literature. Few cases describe the relationship between PE and POS, but these conditions may be more closely related than we currently think.

Persistent impairment in peripheral but not cerebral vascular function in young healthy adults with long-term symptoms from covid-19

Vascular dysfunction and inflammation are precursors to cardiovascular disease (CVD). Notably, young adults who were symptomatic from COVID-19 during the acute phase of illness (within 4 weeks from diagnosis) have shown to exhibit peripheral vascular dysfunction. Importantly, many young adults report persistent symptoms from COVID-19 for several months, including cognitive difficulties. However, it remains unknown whether vascular dysfunction persists beyond the acute phase of COVID-19 in symptomatic young adults. We tested the hypothesis that peripheral and cerebral vascular function would be blunted in symptomatic (SYM) young adults who are beyond 4 weeks from a COVID-19 diagnosis, compared to asymptomatic adults (ASYM). Since COVID-19 causes inflammation that may negatively impact vascular function, we also hypothesized that serum hsCRP would be elevated in SYM compared to ASYM. We studied 15 otherwise healthy adults (age = 23 ± 1 years;mean ± SE) with a positive lab diagnosis of COVID-19. Eight were SYM (14 ± 1 weeks from diagnosis) while seven were ASYM (13 ± 2 weeks from diagnosis) at time of testing. Brachial artery flow-mediated dilation (FMD;duplex Doppler ultrasound) was performed, and macroand microvascular function were quantified as FMD% and peak blood velocity after cuff release, respectively. Cerebral vasomotor reactivity (CVMR) was quantified as percent increase in middle cerebral artery blood velocity (transcranial Doppler ultrasound) to rebreathing induced hypercapnia. Serum hsCRP level was measured. FMD was lower in SYM (3.81 ± 0.60%) compared to ASYM (7.10 ± 0.94%, P = 0.010). Likewise, peak blood velocity after cuff release was lower in SYM (47 ± 3 cm/s vs. ASYM: 65 ± 8 cm/s, P = 0.037). However, CVMR was not different between the two groups (P = 0.91). Serum hsCRP was higher in SYM (3.4 ± 1.0 mg/L vs. ASYM: 0.7 ± 0.1 mg/L, P = 0.036). These preliminary results indicate that peripheral macro-and microvascular function remain blunted beyond the acute phase in young adults with persistent symptoms from COVID-19, whereas cerebral vascular function appears unaffected. The extent to which this sustained vascular impairment and elevated hsCRP contributes to increased CVD risk in these otherwise healthy young adults remains to be determined.

Comparative Study of Haiti and Miami Cohorts of Sickle Cell Disease (CSHSCD): Methods, Accomplishments, and Implementation

[Formula presented] Background: There are significant limitations in Haiti for the diagnosis and management of sickle cell disease (SCD), including the non-availability of universal newborn screening (NBS) and transcranial Doppler (TCD) ultrasound screening, and the lack of diagnostic laboratory resources, oral penicillin and hydroxyurea (HU). Methods: Beginning in September 2019, CSHSCD (R01HL149121), a 5-year NIH-sponsored observational comparative study of children with SCD from Haitian ethnicity in Miami and in Haiti compared to children of African American ethnicity with SCD, was designed to increase access to care in Haiti. The study aims are 1) to compare the incidence of SCD among newborns from Haitian and African American ethnicity in Miami, 2) to establish NBS programs for hemoglobinopathies in Haiti, and 3) to compare cohorts of children in SCD at the study sites. The participating sites are the University of Miami (UM, Miami, Florida), Hôpital Saint Damien (HSD, Tabarre, Haiti), Hôpital de l'Université d'Etat d'Haïti (HUEH, Port-au-Prince, Haiti), Hôpital Universitaire Justinien (HUJ, Cap Haitien, Haiti), and Hôpital Sacré Coeur (HSC, Milot, Haiti). HUJ and HSC use two NBS screening methods (isoelectric focusing and Sickle SCAN rapid test) and HSD and HUEH use isoelectric focusing only. CSHSCD supplies penicillin and HU and trains TCD examiners to implement stroke risk screening. Data are collected in REDCap. Results: During the first 2 years and despite the COVID-19 pandemic, we established NBS sites with a cohesive network of physicians and nurses trained in the care of children with SCD in Haiti. This capacity building will support sustainability of the program. We successfully identified at least 15 new cases of SCD via newborn screening, trained six TCD examiners, and enrolled 130 children with SCD in follow up, providing them with penicillin prophylaxis and hydroxyurea for severe cases according to local protocols. Implementation activities which have helped are close communications between the investigators, monthly Zoom meetings to coordinate efforts with enrollment updates every month, the availability of rapid tests (Sickle SCAN and Gazelle miniature cellulose acetate electrophoresis) for the diagnosis of SCD, especially when there is no laboratory equipment on site. Implementation challenges we have faced are mostly two. The first is the timely completion of DUNS and SAM registration for the two public hospitals, with one site achieving this after 9 months and the other site taking 18 months to complete. The reasons for the delay are the inability for the UM site to direct these efforts, following strict rules, and the Haitian hospital officers' lack of familiarity with website requirements. We were able to achieve these registrations with the assistance of one Haitian study staff who is very acquainted with internet navigation and became familiarized with requirements. Outsourcing materials to Haiti is another major challenge, with either gaps in the delivery of supplies because of multiple steps involved in ordering and shipping or with delays in releasing equipment once it is at the Port-au-Prince customs, resulting in gaps in NBS in one of the sites for 8 weeks. We have minimized these issues by opening a one-year ticket to order materials from the different companies involved. Also, Haiti's lack of infrastructure, available materials and medications, and political instability limit health care delivery. Conclusion: Since its inception, we have achieved major milestones, including capacity building and implementation of NBS, TCD training, and enrollment of children with SCD into the prospective cohorts despite the current COVID-19 pandemic. Material outsourcing challenges have been the major implementation problem we have faced due to systemic factors. We anticipate that these factors will be corrected or minimized as we have learned how to handle them. These problems were expected as part of conducting an international study in a low-resource setting. Acknowledgment: We acknowledg NHLBI for supporting this work. Disclosures: Alvarez: Forma Therapeutics: Membership on an entity's Board of Directors or advisory committees;GBT: Membership on an entity's Board of Directors or advisory committees. Romano: Genentech: Research Funding;Vycor: Current holder of individual stocks in a privately-held company;NovaVision: Consultancy.

Cardiac index is associated with oxygenation in COVID-19 acute respiratory distress syndrome.

Eleven participants with COVID-19 acute respiratory distress syndrome requiring mechanical ventilation underwent pulmonary artery catheterization for clinical indications. Clinical interventions or events concurrent with hemodynamic were recorded. Increased cardiac index was associated with worse hypoxemia. Modulation of cardiac index may improve hypoxemia in patients with COVID-19 acute respiratory distress syndrome.

Powered Air Purifying Respirator (PAPR) restores the N95 face mask induced cerebral hemodynamic alterations among Healthcare Workers during COVID-19 Outbreak.

BACKGROUND AND AIM: COVID-19 pandemic has resulted in an unprecedented increased usage of Personal protective equipment (PPE) by healthcare-workers. PPE usage causes headache in majority of users. We evaluated changes in cerebral hemodynamics among healthcare-workers using PPE. METHODS: Frontline healthcare-workers donning PPE at our tertiary center were included. Demographics, co-morbidities and blood-pressure were recorded. Transcranial Doppler (TCD) monitoring of middle cerebral artery was performed with 2-MHz probe. Mean flow velocity (MFV) and pulsatility index (PI) were recorded at baseline, after donning N95 respirator-mask, and after donning powered air-purifying respirator (PAPR), when indicated. End-tidal carbon-dioxide (ET-CO2) pressure was recorded for participants donning PAPR in addition to the N95 respirator-mask. RESULTS: A total of 154 healthcare-workers (mean age 29 ± 12 years, 67% women) were included. Migraine was the commonest co-morbidity in 38 (25%) individuals while 123 (80%) developed de-novo headache due to N95 mask. Donning of N95 respirator-mask resulted in significant increase in MFV (4.4 ± 10.4 cm/s, p < 0.001) and decrease in PI (0.13 ± 0.12; p < 0.001) while ET-CO2 increased by 3.1 ± 1.2 mmHg (p < 0.001). TCD monitoring in 24 (16%) participants donning PAPR and N95 respirator mask together showed normalization of PI, accompanied by normalization of ET-CO2 values within 5-min. Combined use of N95 respirator-mask and PAPR was more comfortable as compared to N95 respirator-mask alone. CONCLUSION: Use of N95 respirator-mask results in significant alterations in cerebral hemodynamics. However, these effects are mitigated by the use of additional PAPR. We recommend the use of PAPR together with the N95 mask for healthcare-workers doing longer duties in the hospital wards.