Reversible Cerebral Vasoconstriction Syndrome Research Articles

Update on the efficiency and safety of orally administered nasal decongestants

Nasal congestion leading to obstruction is one of the main symptoms in acute rhinosinusitis (common cold), other upper respiratory infections and allergic rhinitis. The EPOS 2020 guidelines place oral decongestants as an efficient therapy for the relief of nasal obstruction. In Romania, there are more than 50 available nasal decongestants, so it is very important for practitioners from all medical domains to be aware of recent data regarding their effectiveness and safety profile. Recent concerns raised by both the Food and Drug Administration (FDA) and the European Medicines Agency (EMA) regarding the efficacy and safety of pseudoephedrine (PSE) and phenylephrine (PHE) emphasize the need for informed decision-making in prescribing. Notably, there were concerns raised about the association between PSE, reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES). EMA’s safety committee, Pharmacovigilance Risk Assessment Committee (PRAC), recently recommended measures to minimize risks of serious side effects when using medicines containing PSE. PRAC recommends that medicines containing PSE should not be used in patients with severe or uncontrolled hypertension and severe acute or chronic kidney disease or failure. Additionally, PRAC recommends healthcare professionals to counsel patients to discontinue the use of such medicines promptly and seek medical assistance if they experience symptoms suggesting PRES or RCVS, such as sudden onset of severe headache, confusion, vomiting, visual disturbances or seizures. While effectiveness of oral PSE is confirmed by clinical studies, expert consensus is unfavorable to PHE. Both in vitro and in vivo clinical pharmacology data indicate that neither the recommended doses nor higher doses of oral PHE demonstrate efficacy in alleviating symptoms of nasal congestion.

Atypical Manifestation of Zoster Meningitis Presented with Headache and Multiple Cerebral Vasoconstrictions

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by headaches and multiple transient cerebral vasoconstrictions. Central nervous system (CNS) infections related to the varicella zoster virus (VZV) can complicate various neurological disorders, including cerebral vasculopathy. We present the case of a patient who complained of a severe headache without any other symptoms or focal neurological signs. Brain magnetic resonance angiography showed multiple focal stenoses, especially in the right middle cerebral artery (MCA), and transcranial Doppler sonography revealed increased velocities in the MCA and right anterior cerebral artery. Initially, the headache was attributed to RCVS, and nimodipine infusion was initiated; however, the headache worsened. A cerebrospinal fluid study showed pleocytosis, increased protein levels, and positive polymerase chain reaction markers for VZV. No skin lesions or fever were observed during treatment. Our case emphasizes considering VZV meningitis as a cause of cerebral vasoconstriction, even in the absence of typical signs of CNS infection.

The Music Within: A Doppler Harmonic Pattern Report in Non-Subarachnoid Hemorrhage Related Cerebral Vasospasm

A 40-year-old male presented an episode of severe thunderclap headache with right hemiparesis. Imaging revealed severe stenosis in the left middle cerebral artery, associated with increased mean velocities detected by Transcranial Doppler Ultrasound (TCD). Notably, a distinctive "tin whistle" sound, known as a doppler harmonic effect, near the highest velocity point was observed during the examination. Extensive workup was performed, including CSF analysis and laboratory investigations, and the diagnosis of Reversible Cerebral Vasoconstriction Syndrome (RCVS) was established. Treatment involving medication and daily monitoring led to a decline in the left MCA's velocity, enabling the patient's discharge with no recurrent headaches or neurological deficits. Notably, a follow-up MRI exhibited the disappearance of the left MCA stenosis. This report discusses the rare occurrence of "musical murmurs" in cerebrovascular diseases, underscoring the potential for early diagnosis using techniques like TCD in emergency settings, particularly in conditions like RCVs.

Association between impaired dynamic cerebral autoregulation and BBB disruption in reversible cerebral vasoconstriction syndrome

BackgroundHalf of the sufferers of reversible cerebral vasoconstriction syndrome (RCVS) exhibit imaging-proven blood-brain barrier disruption. The pathogenesis of blood-brain barrier disruption in RCVS remains unclear and mechanism-specific intervention is lacking. We speculated that cerebrovascular dysregulation might be associated with blood-brain barrier disruption in RCVS. Hence, we aimed to evaluate whether the dynamic cerebral autoregulation is altered in patients with RCVS and could be associated with blood-brain barrier disruption.MethodsA cross-sectional study was conducted from 2019 to 2021 at headache clinics of a national tertiary medical center. Dynamic cerebral autoregulation was evaluated in all participants. The capacity of the dynamic cerebral autoregulation to damp the systemic hemodynamic changes, i.e., phase shift and gain between the cerebral blood flow and blood pressure waveforms in the very-low- and low-frequency bands were calculated by transfer function analysis. The mean flow correlation index was also calculated. Patients with RCVS received 3-dimensional isotropic contrast-enhanced T2 fluid-attenuated inversion recovery imaging to visualize blood-brain barrier disruption.ResultsForty-five patients with RCVS (41.9 ± 9.8 years old, 29 females) and 45 matched healthy controls (41.4 ± 12.5 years old, 29 females) completed the study. Nineteen of the patients had blood-brain barrier disruption. Compared to healthy controls, patients with RCVS had poorer dynamic cerebral autoregulation, indicated by higher gain in very-low-frequency band (left: 1.6 ± 0.7, p = 0.001; right: 1.5 ± 0.7, p = 0.003; healthy controls: 1.1 ± 0.4) and higher mean flow correlation index (left: 0.39 ± 0.20, p = 0.040; right: 0.40 ± 0.18, p = 0.017; healthy controls: 0.31 ± 0.17). Moreover, patients with RCVS with blood-brain barrier disruption had worse dynamic cerebral autoregulation, as compared to those without blood-brain barrier disruption, by having less phase shift in very-low- and low-frequency bands, and higher mean flow correlation index.ConclusionsDysfunctional dynamic cerebral autoregulation was observed in patients with RCVS, particularly in those with blood-brain barrier disruption. These findings suggest that impaired cerebral autoregulation plays a pivotal role in RCVS pathophysiology and may be relevant to complications associated with blood-brain barrier disruption by impaired capacity of maintaining stable cerebral blood flow under fluctuating blood pressure.Graphical

Reversible cerebral vasoconstriction syndrome: A systematic review of reports and case series

Reversible cerebral vasoconstriction syndrome: A systematic review of reports and case series

When less is more: A case of reversible cerebral vasoconstriction syndrome

When less is more: A case of reversible cerebral vasoconstriction syndrome

Post blood transfusion reversible cerebral vasoconstriction syndrome in sickle cell disease: Two case reports

Post blood transfusion reversible cerebral vasoconstriction syndrome in sickle cell disease: Two case reports

Transfusion-Associated Reversible Cerebral Vasoconstriction Syndrome in Pediatrics: A Case Report and Review of Literature

Background Reversible cerebral vasoconstriction syndrome (RCVS) is a rare complication of packed red blood cell (pRBC) transfusion primarily reported in adults with severe chronic anemia due to menorrhagia. It is characterized by a triad of rapidly changing and reversible segmental cerebral artery narrowing and dilatation, thunderclap headache, and benign cerebrospinal fluid findings. 1,2 More than 50% of patients experience blurring of vision or cortical vision loss, and a subset of patients develop hemiparesis or aphasia. 1 There are emerging case reports of RCVS in pediatric patients, however the literature examining this transfusion complication in children is limited. The rarity and potential severity of RCVS warrants better understanding by pediatric hematology and transfusion providers. Results We describe a previously healthy 4 year-old male who presented with pallor and laboratory parameters consistent with severe iron deficiency anemia. Chest x-ray showed cardiomegaly, and echocardiogram demonstrated pericardial effusion, moderate left ventricular dilation, and moderate coronary artery dilatation. His history was notable for an extremely selective diet and at least 50 ounces of cow's milk daily. The patient was admitted, and over the course of 3 days, he received a total of 43 mL/kg of pRBCs (602 mL total) in 8 aliquots, and his hemoglobin (Hgb) increased from 1.4 g/dL to 11.4 g/dL (Fig. 1). He then re-presented 5 days after discharge with altered mental status and malignant hypertension. Brain MRI/MRA was consistent with RCVS, with luminal irregularities in multiple anterior and posterior circulation intracranial arteries creating a beaded appearance. He was treated supportively with aggressive blood pressure control and made a complete neurologic recovery 8 days after presentation. At hematology clinic follow-up 2 months post-discharge, he remained at his typical neurologic baseline. Conclusions The connection between blood transfusion and RCVS is postulated to be due to a rapid increase in hematocrit and blood viscosity, which alters cerebral autoregulation and leads to hypertensive encephalopathy despite systemic normotension. 3 The increased viscosity may also lead to vasospasm. 3 When a patient with severe chronic anemia receives a pRBC transfusion, there is increased cerebral blood flow and viscosity, resulting in decreased vasodilation, increased vascular resistance, and cerebral vessel constriction. However, these are hypothesized mechanisms with little supportive experimental data. Vasculopathies and vasoconstrictive medications 2 are also risk factors for RCVS. There are 4 previous reported cases of pediatric RCVS associated with pRBC transfusion. 2 patients were acutely ill with complications of sickle cell anemia. 7,9 Another case occurred in a patient with systemic lupus erythematosus 8, and the fourth occurred in the setting of severe iron deficiency anemia. 5 Several features of our patient's clinical course are similar to the reported cases of transfusion-related RCVS 5 and warrant highlighting: 1) severe chronic anemia with cardiac dysfunction and 2) the large volume of transfused pRBCs over a short period resulting in a significant increase in Hgb. However, our patient developed symptoms of RCVS sooner than other published cases and did not have seizure or stroke. Pediatric guidelines recommend pRBC transfusion for Hgb levels between 5-10 g/dL, depending on the clinical scenario. 4 There are few data to guide Hgb targets for pediatric patients presenting with severe anemia. Over-transfusion does not offer physiological benefit and poses risk of transfusion-related adverse events. A case report from Dou et al 6 proposed that a >5 g/dL Hgb increase was associated with a greater likelihood of developing RCVS. For this patient with cardiac dysfunction and social concerns including lack of primary care, the potential benefits of achieving a higher Hgb with pRBC transfusions were prioritized. RCVS should be considered in pediatric and adult patients recently transfused for chronic severe anemia presenting with headaches or other neurological symptoms. Prompt recognition of RCVS may prevent unnecessary treatment and potentially limit risk of neurologic compromise. The role of the volume and rapidity of the pRBC transfusions in the development of RCVS warrants further study. Fig 1. Timeline of initial presentation, detailing blood transfusions

Reversible cerebral vasoconstriction syndrome related to extracranial dissection associated with COVID-19: an immunological trigger?

Reversible cerebral vasoconstriction syndrome related to extracranial dissection associated with COVID-19: an immunological trigger?

A case report of reversible cerebral vasoconstriction syndrome with thunderclap headache significantly exacerbated in the supine position and alleviated in the standing position

BackgroundReversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden onset thunderclap headache and multiple segmental reversible cerebral vasoconstrictions that improve within 3 months. The postpartum period is a well-known precipitating factor for the onset of RCVS. Cerebral venous thrombosis (CVT) causes thunderclap headaches in the postpartum period. While headache in CVT is sometimes exacerbated in the supine position, the severity of the headache in RCVS is usually independent of body position. In this study, we report a case of RCVS with thunderclap headache exacerbated in the supine position, and headache attacks that resolved quickly in the standing position during the postpartum period.Case presentationA 33-year-old woman presented with a sudden increase in blood pressure and thunderclap headache on the fifth postpartum day (day 1: the first sick day). The headache was severe and pulsatile, with onset in the supine position in bed, and peaked at approximately 10 s. It was accompanied by nausea and chills but there were no scintillating scotomas or ophthalmic symptoms. The headache resolved in the standing or sitting position but was exacerbated and became unbearable within a few seconds when the patient was in the supine position. Therefore, she was unable to lie supine at night. Computed tomography angiography (CTA) of the head on day 2 and magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) on day 3 showed no abnormalities. However, considering the possibility of RCVS, verapamil was initiated on day 3. The headache resolved the following day. MRA of the head on day 10 revealed diffuse and segmental stenoses in the bilateral middle and posterior cerebral arteries and basilar artery. Therefore, the patient was diagnosed with RCVS. The headache gradually resolved and disappeared completely on day 42. Cerebral vasoconstriction was also improved on MRA on day 43.ConclusionsThis postpartum RCVS case was notable for the exacerbation of headaches in the supine position. For the diagnosis of thunderclap headache in the postpartum period, RCVS should be considered in addition to CVT when the patient presents with a headache that is exacerbated in the supine position.

Misdiagnosis of Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome in the Emergency Department.

Background Cerebrovascular dysregulation syndromes, posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), are challenging to diagnose because they are rare and require advanced neuroimaging for confirmation. We sought to estimate PRES/RCVS misdiagnosis in the emergency department and its associated factors. Methods and Results We conducted a retrospective cohort study of PRES/RCVS patients using administrative claims data from 11 states (2016-2018). We defined patients with a probable PRES/RCVS misdiagnosis as those with an emergency department visit for a neurological symptom resulting in discharge to home that occurred ≤14 days before PRES/RCVS hospitalization. Proportions of patients with probable misdiagnosis were calculated, characteristics of patients with and without probable misdiagnosis were compared, and regression analyses adjusted for demographics and comorbidities were performed to identify factors affecting probable misdiagnosis. We identified 4633 patients with PRES/RCVS. A total of 210 patients (4.53% [95% CI, 3.97-5.17]) had a probable preceding emergency department misdiagnosis; these patients were younger (mean age, 48 versus 54 years; P<0.001) and more often female (80.4% versus 69.3%; P<0.001). Misdiagnosed patients had fewer vascular risk factors except prior stroke (36.3% versus 24.2%; P<0.001) and more often had comorbid headache (84% versus 21.4%; P<0.001) and substance use disorder (48.8% versus 37.9%; P<0.001). Facility-level factors associated with probable misdiagnosis included smaller facility, lacking a residency program (62.2% versus 73.7%; P<0.001), and not having on-site neurological services (75.7% versus 84.3%; P<0.001). Probable misdiagnosis was not associated with higher likelihood of stroke or subarachnoid hemorrhage during PRES/RCVS hospitalization. Conclusions Probable emergency department misdiagnosis occurred in ≈1 of every 20 patients with PRES/RCVS in a large, multistate cohort.

Ultrasonographic Vasospasm and Outcome of Posterior Reversible Encephalopathy and Cerebral Vasoconstriction Syndromes.

Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are often complicated by vasospasm and ischemia. Monitoring with transcranial color-coded Doppler (TCCD) could be useful, but its role is not established. We studied the incidence of ultrasonographic vasospasm (uVSP) in PRES/RCVS and its relationship with ischemic lesions and clinical outcome. We conducted a multicenter retrospective study of all patients with PRES/RCVS from 2008 to 2020 who underwent TCCD and magnetic resonance imaging (MRI). TCCD exams were analyzed for uVSP. Diffusion-weighted MRI was analyzed for positive lesions (DWI-positive). Functional outcome was assessed by modified Rankin scale (mRS) at 90 days. The associations with outcomes were determined by logistic regression. We included 80 patients (mean age of 46 (standard deviation, 17) years; 66% females; 41 with PRES, 28 with RCVS and 11 with overlap phenotype). uVSP was detected in 25 (31%) patients. DWI-positive lesions were more often detected in uVSP-positive than uVSP-negative patients (36% vs. 15%; adjusted odds ratio [aOR] 4.05 [95% CI 1.06 - 15.5], P=0.04). DWI-positive lesions were independently associated with worse functional prognosis (mRS 2-6, 43% vs. 10%; aOR, 10 [95% CI 2.6 - 43], P<0.01). Having additional uVSP further increased the odds of a worse outcome (P interaction=0.03). Ultrasonographic vasospasm was detected in a third of patients with PRES/RCVS and was associated with brain ischemic lesions. TCCD bedside monitoring can help to stratify patients at risk for cerebral ischemia, a strong predictor of functional outcome.

Headache Attributed to Reversible Cerebral Vasoconstriction Syndrome (RCVS).

Reversible cerebral vasoconstriction syndrome (RCVS) is a condition with variable outcomes presenting a new onset thunderclap headache accompanied by focal neurological symptoms or seizures. It can be idiopathic or arise secondarily to a variety of trigger factors. The condition is increasingly recognized in clinical practice, but many facets remain poorly understood. This article aims to clarify the headache characteristics in RCVS, the temporal association of angiographic findings, the potential association of the condition with SARS-CoV-2 infection, and the clinical presentation of RCVS in children and is based on a systematic PRISMA search for published analytical or large descriptive observational studies. Data from 60 studies that fulfilled specific criteria were reviewed. Most people with RCVS exhibit a typical thunderclap, explosive, or pulsatile/throbbing headache, or a similar acute and severe headache that takes longer than 1 min to peak. Atypical presentations or absence of headaches are also reported and may be an underrecognized phenotype. In many cases, headaches may persist after resolution of RCVS. Focal deficits or seizures are attributed to associated complications including transient ischemic attacks, posterior reversible encephalopathy syndrome, ischemic stroke, cerebral edema, and intracranial hemorrhage. The peak of vasoconstriction occurs usually within two weeks after clinical onset, possibly following a pattern of centripetal propagation, and tends to resolve completely within 3 months, well after symptoms have subsided. There are a few reports of RCVS occurring in relation to SARS-CoV-2 infection, but potential underlying pathophysiologic mechanisms and etiological associations have not been confirmed. RCVS occurs in children most often in the context of an underlying disease. Overall, the available data in the literature are scattered, and large-scale prospective studies and international collaborations are needed to further characterize the clinical presentation of RCVS.

Guillain-Barré Syndrome Complicated by Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome.

Guillain-Barré syndrome is an immune-mediated inflammatory polyneuritis characterised by rapidly progressive flaccid paralysis. Guillain-Barré syndrome may present with posterior reversible encephalopathy syndrome or reversible cerebral vasoconstriction syndrome in rare cases. A woman in her 60s with a history of follicular lymphoma presented with a one-week history of difficulty walking and thunderclap headaches. The patient was diagnosed with Guillain-Barré syndrome based on neurological examination, cerebrospinal fluid analysis and nerve conduction findings. Further diagnosis of posterior reversible encephalopathy and reversible cerebral vasoconstriction syndromes was based on imaging findings and headache history. The patient was treated with intravenous immunoglobulin and amlodipine, and symptoms improved. We reviewed the literature on Guillain-Barré syndrome associated with posterior reversible encephalopathy and/or reversible cerebral vasoconstriction syndrome. The underlying pathophysiology may involve dysautonomia resulting in unstable blood pressure, and hyponatraemia causing endothelial dysfunction. The SNOOP mnemonic highlights the 'red flags'. This SNOOP mnemonic suggests the possibility of secondary headaches that require imaging studies. In this case, the patient exhibited three SNOOP symptoms: S (history of malignancy: follicular lymphoma), O (sudden-onset headache) and O (over 50 years old). This case highlights the importance of considering coexisting central neurological disorders in patients with Guillain-Barré syndrome. Guillain-Barré syndrome (GBS) alone rarely causes headaches; therefore, when GBS patients complain of severe headaches, especially when the headache is associated with 'red flags', other complications and differential diagnosis should be considered.Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) can be triggered by GBS.Hyponatraemia, age over 50 years and female gender may be risk factors for developing PRES and RCVS in GBS patients.

Lomerizine Improved Bath-related Headache Presenting as Reversible Cerebral Vasoconstriction Syndrome.

Lomerizine Improved Bath-related Headache Presenting as Reversible Cerebral Vasoconstriction Syndrome.

Disrupted Brain Functional Status in Patients with Reversible Cerebral Vasoconstriction Syndrome.

The aim of this study was to investigate the functional networks in subjects with reversible cerebral vasoconstriction syndrome (RCVS) using resting-state functional magnetic resonance imaging (rs-fMRI). We prospectively recruited patients with RCVS and healthy controls (HCs) between February 2017 and April 2021. The rs-fMRI data were analyzed using graph theory methods. We compared node-based global and regional topological metrics (Bundle 1) and network-based intranetwork and internetwork connectivity (Bundle 2) between RCVS patients and HCs. We also explored the associations of clinical and vascular (ie, the Lindegaard index, LI) parameters with significant rs-fMRI metrics. A total of 104 RCVS patients and 93 HCs were included in the final analysis. We identified significantly decreased local efficiency of the left dorsal anterior insula (dAI; p = 0.0005) in RCVS patients within 30 days after disease onset as compared to HCs, which improved 1 month later. RCVS patients also had increased global efficiency (p = 0.009) and decreased average degree centrality (p = 0.045), clustering coefficient (p = 0.033), and assortativity values (p = 0.003) in node-based analysis. In addition, patients with RCVS had increased internetwork connectivity of the default mode network (DMN) with the salience (p = 0.027) and dorsal attention (p = 0.016) networks. Significant correlations between LI and regional local efficiency in left dAI (rs  = -0.418, p = 0.042) was demonstrated. The significantly lower local efficiency of the left dAI, suggestive of impaired central autonomic modulation, was negatively correlated with vasoconstriction severity, which is highly plausible for the pathogenesis of RCVS. ANN NEUROL 2023;94:772-784.

Adult-onset Leigh Syndrome with a m.9176 T>C Mutation Manifested as Reversible Cerebral Vasoconstriction Syndrome.

A 26-year-old woman developed a sudden headache, ptosis, and diplopia. Magnetic resonance imaging and angiography demonstrated a symmetrical lesion from the midbrain to the brainstem, involving the solitary nucleus and multifocal cerebral artery narrowing. Reversible cerebral vasospasm syndrome (RCVS) was suspected, and the patient improved after vasodilatation. Leigh syndrome was suspected due to the elevated serum lactate levels, so mitochondrial DNA was analyzed, and an m.9176 T>C mutation was detected. The final diagnosis was adult-onset Leigh syndrome manifesting as RCVS. An uncontrolled baroreflex due to a solitary nuclear lesion or endothelial dysfunction may have contributed to her unique presentation.

Cerebrovascular Pathologies in Women during Pregnancy and Postpartum In the Neurology Departments of the Yalgado Ouédraogo and Bogodogo University Hospitals

Introduction: Pregnancy and post-partum are periods of risk for cerebrovascular complications in women. These pathologies can affect the vital prognosis of the woman. Objectives: To describe the determinants of cerebrovascular pathologies during pregnancy and postpartum in the neurology departments of the CHU-YO and CHU-B. Method: This was a descriptive cross-sectional study collected retrospectively over a period from January 1, 2019 to December 31, 2021. Results: We included 113 patients, mean age 23.35 years with extremes of 15 and 45 years. Eclampsia predominated in 64.60% of cases. Other etiologies were stroke (18.58%), cerebral venous thrombosis (11.51%), reversible posterior encephalopathy syndrome (3.54%) and reversible cerebral vasoconstriction syndrome (1.77%). Factors associated with the occurrence of cerebrovascular pathology were young age [15-19 years] (p=0.001), high blood pressure (p=0.001). The mortality rate was 8.85%. Conclusion: Cerebrovascular pathologies during pregnancy and postpartum with regard to their morbidity and mortality constitute a reproductive health problem and therefore require a multidisciplinary approach.

Posterior Reversible Leucoencephalopathy Syndrome: Case Series, Comments, and Diagnostic Dilemma.

To report a series of patients with clinical and radiological features suggestive of posterior reversible encephalopathy syndrome (PRES) related to diverse etiologies emphasizing its pathophysiological basis. Posterior reversible encephalopathy syndrome (PRES) may present with a broad range of clinical symptoms from headache and visual disturbances to seizure and altered mentation. Typical imaging findings include posterior-circulation predominant vasogenic edema. Although there are many well-documented diseases associated with PRES, the exact pathophysiologic mechanism has yet to be fully elucidated. Generally accepted theories revolve around disruption of the blood-brain barrier secondary to elevated intracranial pressures or endothelial injury induced by ischemia from a vasoconstrictive response to rising blood pressure or toxins/cytokines. While clinical and radiographic reversibility is common, long-standing morbidity and mortality can occur in severe forms. In patients with malignant forms of PRES, aggressive care has markedly reduced mortality and improved functional outcomes. Various factors that have been associated with poor outcome include altered sensorium, hypertensive etiology, hyperglycemia, longer time to control the causative factor, elevated C reactive protein, coagulopathy, extensive cerebral edema, and hemorrhage on imaging. Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. Diagnosis of PRES in some circumstances can be challenging and structural imaging may not be sufficient to distinguish it from other differential diagnostic considerations like ADEM. Advanced imaging techniques, such as MR spectroscopy or positron emission tomography (PET) can provide additional information to determine the diagnosis. Such techniques are more useful to understand the underlying vasculopathic changes in PRES and may answer some of the unresolved controversies in pathophysiology of this complex disease. Eight patients with PRES resulting from different etiologies varying from pre-eclampsia/eclampsia, post-partum headache with seizures, neuropsychiatric systemic lupus erythematosus, snake bite, Dengue fever with encephalopathy, alcoholic liver cirrhosis with hepatic encephalopathy, and lastly reversible cerebral vasoconstriction syndrome (RCVS). Additionally, a diagnostic dilemma between PRES and acute disseminated encephalomyelitis (ADEM) was notable in one patient. Some of these patients did not have or only very transiently had arterial hypertension. PRES may underlie the clinical conundrum of headache, confusion, altered sensorium, seizures, and visual impairment. PRES need not necessarily be always associated with high blood pressure. Imaging findings may also be variable. Both clinicians and radiologists need to familiarize themselves with such variabilities.

Management of postoperative cerebral vasospasm in skull base surgeries: A systematic review of case reports and series.

This study provides a comprehensive overview of the management of postoperative vasospasm after skull base surgeries. This phenomenon is rare but can be of serious sequelae. Medline, Embase, and PubMed Central were searched, along with examining the references of the included studies. Only case reports and series that reported vasospasm following a skull base pathology were incorporated. Cases with pathologies other than skull base, subarachnoid hemorrhage, aneurysm, and reversible cerebral vasoconstriction syndrome were excluded from the study. Quantitative data were presented as mean (Standard Deviation) or median (range), accordingly, while qualitative data were presented as frequency (percentage). Chi- square test and one-way analysis of variance were used to assess for any association between the different factors and patient outcomes. We had a total of 42 cases extracted from the literature. The mean age was 40.1 (±16.1) with approximately equal males and females (19 [45.2%] and 23 [54.8%], respectively). The time to develop vasospasm after the surgery was 7 days (±3.7). Most of the cases were diagnosed by either angiogram or magnetic resonance angiography. Seventeen of the 42 patients had pituitary adenoma as the pathology. Anterior circulation was nearly affected in all patients. For management, most patients received pharmacological with supportive management. Twenty-three patients had an incomplete recovery as a result of vasospasm. Vasospasm following skull base operations can affect males and females, and most patients in this review were middle-aged adults. The outcome of patients varies; however, most patients did not achieve a full recovery. There was no correlation between any factors and the outcome.