Advances in Management of the Stroke Etiology One-Percenters.

PURPOSE OF REVIEW: Uncommon causes of stroke merit specific attention; when clinicians have less common etiologies of stoke in mind, the diagnosis may come more easily. This is key, as optimal management will in many cases differs significantly from "standard" care. RECENT FINDINGS: Randomized controlled trials (RCT) on the best medical therapy in the treatment of cervical artery dissection (CeAD) have demonstrated low rates of ischemia with both antiplatelet and vitamin K antagonism. RCT evidence supports the use of anticoagulation with vitamin K antagonism in "high-risk" patients with antiphospholipid antibody syndrome (APLAS), and there is new evidence supporting the utilization of direct oral anticoagulation in malignancy-associated thrombosis. Migraine with aura has been more conclusively linked not only with increased risk of ischemic and hemorrhagic stroke, but also with cardiovascular mortality. Recent literature has surprisingly not provided support the utilization of L-arginine in the treatment of patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS); however, there is evidence at this time that support use of enzyme replacement in patients with Fabry disease. Additional triggers for reversible cerebral vasoconstriction syndrome (RCVS) have been identified, such as capsaicin. Imaging of cerebral blood vessel walls utilizing contrast-enhanced MRA is an emerging modality that may ultimately prove to be very useful in the evaluation of patients with uncommon causes of stroke. A plethora of associations between cerebrovascular disease and COVID-19 have been described. Where pertinent, authors provide additional tips and guidance. Less commonly encountered conditions with updates in diagnosis, and management along with clinical tips are reviewed.

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.

Eclampsia with hypothyroidism complicated with posterior reversible encephalopathy syndrome-a case report.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disorder with complex physiopathological mechanisms that have not been fully understood. Early identification is of great prognostic significance, of which the symptoms and radiological abnormalities can be completely reversed. If the diagnosis and treatment are delayed, ischemia and massive infarction may be developed in some patients. Posterior reversible encephalopathy syndrome (PRES) has been reported mainly in association with postpartum eclampsia, which have been rarely reported, while the association with hypothyroidism has not been reported at home or abroad. CASE PRESENTATION: Here we report on a pregnant 29-year-old with multipara and a chief complication of hypothyroidism. She presented in the emergency department with frequent attacks of severe headache symptoms resulting from reversible cerebral vasoconstriction syndrome (RCVS), accompanied with prenatal eclampsia. PRES was determined by radiological examination. CONCLUSION: To the best of our knowledge, this is the first case of PRES complicated by hypothyroidism and prepartum eclampsia.Clinicians should be alert for the co-occurence of eclampsia, PRES, and RCVS when patients have convulsions after a typical throbbing headache. Moreover, regular monitoring of thyroid function during pregnancy should also occupy certain special attention.

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.

Diagnosis and treatment of vasculitis

Primary angiitis of the central nervous system (PACNS) is an inflammatory disease affecting exclusively small and medium-sized vessels of the central nervous system. CNSvasculitis may also occur in systemic diseases like giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis, or Behçet syndrome. The most common presenting symptoms of CNS vasculitis are multifocal symptoms associated with recurrent episodes of ischemia or hemorrhage, encephalopathy-related cognitive and affective abnormalities, and headaches. Diagnostic work up of CNS vasculitis includes MRI, CSF examination, digital subtraction angiography and brain biopsy. High-resolution, contrast-enhanced, compensated and fat-saturated MRI imaging of the cerebral vessel walls (black-blood imaging) may be of some value for the detection of CNS-vasculitis. Patients with normal CSF findings are unlikely to have CNS vasculitis. Brain biopsy should be performed in suspected PACNS. Important differential diagnoses include reversible cerebral vasoconstriction syndrome, moyamoya angiopathy and infectious vasculopathies (VZV, SarsCoV2, borreliosis, bacterial endocarditis). The adherence to diagnostic criteria and the avoidance of inappropriate therapies are essential. Treatment recommendations for CNS-vasculitis include glucocorticoids in combination with cyclophosphamide or rituximab;however, randomized clinical trials of PACNS treatment do not exist. Induction therapy is recommended for 6 to 12 months. After remission is achieved, treatment may be continued with substances as mycophenolate mofetil, methotrexate, or azathioprine. Repeated clinical, CSF- and neuroradiological monitoring is needed to determine the individual duration of maintenance therapy.

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.

Reversible cerebral vasoconstriction syndrome in a patient with COVID-19

COVID-19 infection is well-known to produce different neurological complications, including cerebrovascular diseases. Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by transient segmental vasoconstriction of the cerebral vasculature, has been rarely reported in association with COVID-19 infection. The causative agent, the novel coronavirus (SARS-CoV-2), binds to the angiotensin-converting enzyme 2 (ACE-2) receptors for its entry into the host cell. This leads to downregulation of the ACE-2 and increased activity of the renin-angiotensin-aldosterone (RAAS) axis resulting in sympathetic overactivity and vasoconstriction. This might be the possible mechanism of RCVS in COVID-19. We hereby report a case of RCVS occurring in a SARS-CoV-2infected patient. This was a 38-year-old male without any comorbidities or risk factors, who presented with headache and confusion. His SARS-CoV-2 RT-PCR was positive. MRI of the brain was normal but cerebral angiography revealed segmental vasoconstriction in bilateral middle cerebral arteries and the terminal part of the internal carotid arteries, which resolved almost completely after 2 weeks. He was treated with oral nimodipine 60 mg every 6 hourly. A database search revealed 2 previous cases of RCVS associated with COVID-19. In conclusion, RCVS is a rare complication of COVID-19. It is possibly under-recognized as only a few COVID-19 patients with headaches undergo cerebral angiography especially when parenchymal brain imaging is normal.

Intracerebral Hemorrhage and Reversible Cerebral Vasoconstriction Syndrome in a Patient With COVID-19.

Neurological manifestations, such as encephalopathy, intracranial neuropathy, headache, and cognitive decline, are often presented in patients with COVID-19 infection. Since the onset of the pandemic, acute ischemic stroke associated with a hypercoagulable state caused by COVID-19 is increasingly being reported. Hemorrhagic stroke is also reported via poorly understood mechanisms. We report one of the first-ever cases of intraparenchymal hemorrhage, subarachnoid hemorrhage secondary to reversible cerebral vasoconstriction syndrome in a patient with COVID-19 infection.

Neurological complications in COVID-19 - a diagnostic challenge.

With the exponential growth of COVID-19 cases, the neurological complications reported during or after the infection became more common. There is limited knowledge regarding the pathophysiological mechanisms that are responsible for these complications. Recent data provides compelling evidence for the neurotropic nature of SARS-CoV-2, based on neurological manifestations reported during the current pandemic, as well as on previous experience with other coronaviruses. We present the case of a patient who developed headaches, motor deficit and dysphasia after respiratory COVID-19. Imaging tests showed heterogeneous central nervous system lesions (multiple subarachnoid hemorrhages and two ischemic strokes). Given the plethora of atypical neurological complications of COVID-19 described in the current literature, establishing a positive diagnosis and deciding on a treatment plan proved to be particularly challenging. We set to discuss some of the possible pathologies, hypothesized to be associated with COVID-19, that could lead to concomitant neurological lesions, similar to those noticed in our patient.

COVID-19 Associated Reversible Cerebral Vasoconstriction Syndrome Successfully Treated with Nimodipine and Aspirin.

There have been limited cases linking SARS-CoV-2 infection with the development of reversible cerebral vasoconstriction syndrome (RCVS). We hereby report a rare case of RCVS in the setting of mild SARS-CoV-2 respiratory infection successfully treated with nimodipine and aspirin. SARS-CoV-2 attacks the ACE2-receptors, which are expressed in various body organs including the lungs, kidneys, and blood vessels. Vasoconstriction can result from down-regulation of the ACE2-receptors that can lead to sympathetic hypertonia of the cerebral blood vessel walls and/or over-activation of the renin-angiotensin axis.

Reversible cerebral vasoconstriction syndrome and dissection in the setting of COVID-19 infection.

The current COVID-19 pandemic has recently brought to attention the myriad of neuro- logic sequelae associated with Coronavirus infection including the predilection for stroke, particularly in young patients. Reversible cerebral vasoconstriction syndrome (RCVS) is a well-described clinical syndrome leading to vasoconstriction in the intracra- nial vessels, and has been associated with convexity subarachnoid hemorrhage and oc- casionally cervical artery dissection. It is usually reported in the context of a trigger such as medications, recreational drugs, or the postpartum state; however, it has not been described in COVID-19 infection. We report a case of both cervical vertebral ar- tery dissection as well as convexity subarachnoid hemorrhage due to RCVS, in a pa- tient with COVID-19 infection and no other triggers.