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.

RCVS: by clinicians for clinicians-a narrative review.

BACKGROUND/OBJECTIVE: Reversible cerebral vasoconstriction syndrome may be underdiagnosed. It can be accompanied by various complications, mainly intracerebral hemorrhage and ischemic stroke. The clinical presentation of this condition varies according to its localization. The aims of this review are to raise awareness of the disease, especially in the presence of corresponding risk factors; to connect its precipitating factors, pathophysiology, and complications; and to compare various differential diagnoses of vasoconstriction. METHODS: A review of the literature in PubMed/MEDLINE and Google Scholar was conducted from May 1997 until May 2022. RESULTS: Reversible cerebral vasoconstriction syndrome, which is a clinical-radiological syndrome, is mainly characterized by the occurrence of thunderclap headache and widespread vasoconstriction. The most common precipitating factors are the use of vasoactive substances and postpartum status. The pathophysiology is currently assumed to include two mechanisms: sympathetic overactivity and endothelial dysfunction. From these mechanisms, it is possible to derive potential complications as well as the most important differential diagnoses: posterior reversible encephalopathy syndrome, convexity subarachnoid hemorrhage, ischemic and hemorrhagic stroke, and primary angiitis of the central nervous system. CONCLUSION: In general, the outcome of reversible cerebral vasoconstriction syndrome is very good. Vasospasm as well as thunderclap headache attacks can be fully reversible, and > 90% of patients are functionally independent at discharge.

Pathophysiology of reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndrome (RCVS) is a complex neurovascular disorder being recognized during the past two decades. It is characterized by multiple abrupt severe headaches and widespread cerebral vasoconstrictions, with potential complications such as ischemic stroke, convexity subarachnoid hemorrhage, intracerebral hemorrhage and posterior reversible encephalopathy syndrome. The clinical features, imaging findings, and dynamic disease course have been delineated. However, the pathophysiology of RCVS remains elusive. Recent studies have had substantial progress in elucidating its pathogenesis. It is now believed that dysfunction of cerebral vascular tone and impairment of blood-brain barrier may play key roles in the pathophysiology of RCVS, which explains some of the clinical and radiological manifestations of RCVS. Some other potentially important elements include genetic predisposition, sympathetic overactivity, endothelial dysfunction, and oxidative stress, although the detailed molecular mechanisms are yet to be identified. In this review, we will summarize what have been revealed in the literature and elaborate how these factors could contribute to the pathophysiology of RCVS.

TELEMEDICAL EVALUATION OF ACUTE HEADACHE IN SETTING OF ONGOING COVID PANDEMIC

CASE: 50 yo generally healthy female with two sudden “throbbing” frontal headaches (HA) over the 7 days. First episode (rated 9 out of 10) was preceded by abd pain and emesis. The HA worsened in the laying down, which decreased after 5 hrs of “pacing around the house." Second HA preceded by neck pain and nausea. HA persist (2 out of 10) after the episodes with 'persistent brain fog.' Positive for recent life stressors. Has Mirena. During the video visit, she appears alert, not in distress, speech and mentation at baseline. Face symmetrical. However, no traditional intake such as vital signs were not available and the physical exam was limited. Due to the red flags symptoms, imaging was indicated. CT is the first pass work up for intracranial hemorrhage. Differential diagnoses include migraines, benign HA, hemorrhage, thrombosis, dissection, and neoplasms. There were logistical limitations as this occurred over the holiday with reduced clinic hours, no urgent care and the ED on diversion. An urgent head CT ordered with the plan for follow up in person visit after the holiday for further assessment, and likely consultation with neuro. Findings concerning for acute SAH. Radiology sends patient to ED. Repeat CT angio, again, shows “multifocal beaded and narrowing in circulation. Suggestive of cerebral vasoconstriction syndrome (RCVS).” After admission, pt is evaluated by neuro and undergoes angio, which finds mild diffused artery luminal stenosis consistent with RVCS. Intra-arterial verapamil administered into 3 cerebral arteries had marked improvement. Discharged on 90 days of oral verapamil with close PCP follow up. IMPACT/DISCUSSION: RVCS is evolving neurological condition. Given the low incidence of 3 in 1million patients, the understanding of RVCS continues to grow. RVCS commonly presents as severe thunderclap HA. Triggered by use of vasoconstricting medication, illicit drug, postpartum and grief. However, acute HA have a relatively large differential. Primarily diagnosed through imaging. As in this case, RVCS requires urgent interventions. To differentiate from benign etiologies of HA particularly as health services are limited or overwhelmed by COVID health epidemic, telehealth can play a pivotal role in increasing accessibility to reduce pt harm and potentially negative outcomes. Impact on practice: Red flag symptoms associated with thunderclap HA, even after improvement necessitate urgent evaluation of address the risk of RCVS. Thorough limited neuro examinations through video can assist in diagnostic differential development. As the COVID continues and impacts burden of healthcare, post pandemic incorporation of telehealth can play in acute settings with limited resources that can significantly reduce poor pt outcomes. CONCLUSION: Thorough investigations of presenting illness and medical history supply critical details in distinguishing atypical HA In the setting of limited resources and time constraints, virtual assessments provide sufficient information to support expedited workup.

RCEM Annual Scientific Conference 2022

The proceedings contain 51 papers. The topics discussed include: prognostic accuracy of triage tools for adults with suspected covid-19 in a pre-hospital setting: an observational cohort study;thunderclap headache syndrome presenting to the emergency department: an international multicenter observational cohort study;changing nature of major trauma from 2000 to 2019 in England and Wales: observational registry study;characteristics of patients with COVID-19 undergoing CT pulmonary angiography in the emergency department: a retrospective observational study;improving the accuracy of frontline clinicians in detecting SARS-COV-2 on chest x-rays using a bespoke virtual training platform;the prognostic accuracy of neutrophil-lymphocyte ratio in COVID-19 patients;evaluation of the role of anal tone and perianal sensation examination in the assessment of suspected cauda equina syndrome;and changing nature of major trauma from 2000 to 2019 in England and Wales: observational registry study.

Reversible Cerebral Vasoconstriction Syndrome and Multisystem Inflammatory Syndrome in Children With COVID-19.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) involves multiple organs and shows increased inflammatory markers. Since the onset of the coronavirus disease 2019 (COVID-19) pandemic, several studies have reported the association between severe COVID-19 and MIS-C. Reversible cerebral vasoconstriction syndrome (RCVS) presents with thunderclap headaches and multifocal reversible vasoconstriction on imaging. RCVS is very rare in children. This article reports two cases of pediatric COVID-19 with severe MIS-C and clinical and imaging features indicative of RCVS. METHODS: Clinical, laboratory, and imaging data of the patients were reviewed. The diagnosis of RCVS was confirmed based on clinical symptomatology and brain magnetic resonance imaging findings. RESULTS: Two pediatric patients with clinical findings compatible with severe MIS-C and hemodynamic compromise presented to the hospital. During their hospitalization course, they developed thunderclap headaches and neurological deficits. Both were receiving vasoactive agents, intravenous immunoglobulin, and immunosuppressants. Imaging studies showed marked multifocal cerebral vasoconstriction in both cases and infarcts in one. The course and management of the patients will be presented. After controlling inflammation and elimination of triggers, both patients were ultimately symptom free upon discharge. Cerebral vasoconstriction had completely resolved on follow-up imaging. CONCLUSIONS: Although a variety of symptoms including headaches may be seen in pediatric COVID-19 patients with MIS-C, RCVS should be considered as a differential diagnosis in cases of thunderclap headache accompanied by neurological signs in these patients. Imaging findings and follow-up are also key in establishing the diagnosis.

SARS-CoV-2 vaccine-related neurological complications.

OBJECTIVE: To describe three cases with neurological symptoms after SARS-CoV-2 vaccination. METHODS: A case series followed by a review of the literature, describing hypotheses on how neurological symptoms might develop after vaccination. RESULTS: The different temporal relationship between the onset or worsening of different neurological symptoms suggests different pathophysiological mechanisms. Progression of post-infectious myoclonus, caused by a previous SARS-CoV-2-infection, shortly after vaccination suggests a renewed auto-immune mediated crossreaction of antibodies to both viral epitopes and central nervous system components. Thunderclap headache after vaccination suggests a similar pathophysiological mechanism to the headache and other flu-like symptoms described after vaccination against other viruses. This might be ascribed to the activation of immunoinflammatory mediators or accompanying fever. Although headache accompanied by encephalopathy and focal neurological deficit might occur as part of a cytokine release syndrome, this is clinically less likely. CONCLUSIONS: A variety of symptoms, including thunderclap headache, focal deficits and movement disorders, can occur after SARS-CoV-2 vaccination, and an activation or reactivation of the immune system is suggested as most likely cause. However, one should be careful about claiming a direct correlation. It remains important to exclude other causes, such as structural lesions, infections or subarachnoid hemorrhage, and future research is required to understand possible pathophysiological mechanisms and associations with the SARS-CoV-2 vaccine.