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OBJECTIVES: To describe the characteristics and causes of non-aneurysmal thunderclap headache (TCH) and compare serious from benign underlying causes. METHODS: Retrospective cohort study of consecutive adult patients with TCH presenting to a tertiary care academic medical center between 2010 and 2020. Aneurysmal subarachnoid hemorrhage cases were excluded. Cases were categorized into serious or benign; serious TCH was defined as any condition in which delayed diagnosis and treatment could result in neurological disability or death. Risk factors for serious TCH were analyzed. We adhere to standardized guidelines for reporting observational studies. RESULTS: A total of 932 patients presented with TCH. After exclusion of 393 patients with aneurysmal-type subarachnoid hemorrhage, 539 were included in the analysis. One-half (n = 275, 51.0 %) had a serious cause. Median age was 51 years, 69.0 % were female. Most frequent diagnoses were intracranial hemorrhage (n = 102, 18.9 %), reversible cerebral vasoconstriction syndrome (n = 97, 18.0 %), and idiopathic TCH (n = 102, 38.6 %). A multivariable logistic regression model for prediction of serious TCH included age, hypertension, migraines, recurrent TCH, level of consciousness and other clinical exam findings, and achieved an AUROC of 0.732. This score had a sensitivity of 79.9 % (95 % CI 73.5-83.5 %) for the identification of serious TCH. A 0.5-point increase in the risk score was associated with a 73 % increase in the odds of serious TCH (odds ratio 1.73, 95 % CI 1.53-1.95, p < 0.001). CONCLUSION: Our study describes the relative frequency of presentation and etiologies among patients with TCH This score can aide clinicians in recognising patients with potentially serious cause of TCH, for whom additional imaging and neurological consultation is necessary.
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INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) can present with hemorrhage, ischemia, or both. We aim to compare the differences in presentation and outcomes between patients with RCVS. METHODS: The hemorrhagic presentation group had 58 patients, and the non-hemorrhagic presentations had 30 patients. Subgroup analysis compared patients with evidence of one or more types of hemorrhage (n = 53), no evidence of hemorrhage or infarction (n = 23), evidence of infarction only (n = 7), and combination of hemorrhage and infarction (n = 5). Clinical and radiographic data were analyzed. RESULTS: Migraine (p = 0.030) and intracranial tumors (p = 0.004) were more frequent in non-hemorrhagic presentation. Seizures on admission (p = 0.047) and higher than average C-reactive protein (CRP) (p = 0.037) were seen at a higher rate in patients with hemorrhagic presentation. RCVS2 scores were not unexpectedly higher in patients with hemorrhage than non-hemorrhagic presentations (p = 0.010). Outcomes between the hemorrhagic and non-hemorrhagic groups were comparable. Subgroup analysis found a higher subset of patients with opiate use (p = 0.046) in the hemorrhage-only group. Patients with hemorrhage presented with a thunderclap headache (p < 0.001) more often when compared to the other three groups. RCVS2 score was not unexpectedly higher in the hemorrhage-only group compared with the other groups (p = 0.004). CONCLUSION: A history of migraines was associated with ischemia, while intracranial tumor was significantly associated with evidence of either an infarct, or no changes on imaging. Exposure to opiates, and seizures or thunderclap headache on presentation were associated with hemorrhage. If our data are reproducible, the RCVS2 score may benefit from inclusion of other, small hemorrhages as criterion for diagnosis for RCVS.
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Acute intermittent porphyria is a rare autosomal dominant metabolic disorder. It can affect the autonomic, peripheral, and central nervous system. The present study reports on the case of 28-year-old Chinese female patient with posterior reversible encephalopathy syndrome, reversible cerebral vasoconstriction syndrome and myocardial ischemia which have been very rarely reported in patients with acute intermittent porphyria.
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BACKGROUND: Data on drug-induced reversible cerebral vasoconstriction syndrome (RCVS) are scarce. We aimed to describe RCVS characteristics with drugs previously identified as associated with RCVS and investigate potential signals related to other drugs. METHODS: VigiBase® was queried for all reports of RCVS until 31 May 2023. A descriptive study was performed on reports concerning drug classes known to precipitate RCVS. To identify new drugs, a disproportionality analysis was conducted. RESULTS: In total, 560 reports were included. RCVS occurred in patients aged between 45-64 years (40%) and 18-44 years (35%), mainly in females (72.5%). Drugs were antidepressants (38.4%), triptans (6.4%), nasal decongestants (3.7%) and immunosupressants (8.7%). In 50 cases, antidepressants were in association with drugs known to precipitate RCVS. The median time to onset was 195 days for antidepressants and much shorter (1-10 days) for triptans, nasal decongestants and immunosuppressants. The outcome was favorable in 87% of cases, and fatal in 4.4%. We found a disproportionality signal with 14 drugs: glucocorticoids, bupropion, varenicline, mycophenolic acid, aripiprazole, trazodone, monoclonal antibodies (erenumab, ustekinumab and tocilizumab), leuprorelin and anastrozole. CONCLUSIONS: The present study confirms the role of vasoconstrictors in the onset of RCVS, particularly when used in combination and found potential signals, which may help clinicians envisage an iatrogenic etiology of RCVS.
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Farmacovigilancia , Humanos , Femenino , Persona de Mediana Edad , Masculino , Adulto , Adolescente , Adulto Joven , Vasoespasmo Intracraneal/inducido químicamente , Vasoespasmo Intracraneal/epidemiología , Antidepresivos/efectos adversos , Descongestionantes Nasales/efectos adversos , Inmunosupresores/efectos adversos , Triptaminas/efectos adversos , AncianoRESUMEN
BACKGROUND: Reversible Cerebral Vasoconstriction Syndrome (RCVS) involves cerebral vasculature constriction and dilation. While the exact pathophysiology of RCVS is still not fully understood, there are multiple etiological factors suggested to be implicated in triggering RCVS. We report two RCVS cases potentially linked to teprotumumab. Case 1: A 59-year-old female with Graves' eye disease (GED) developed leg weakness and headache after initiating teprotumumab, and neuroimaging studies revealed multifocal cerebral vasospasm (CVS). Verapamil mitigated vasospasm and the patient overall improved. Case 2: A 71-year-old female with GED developed thunderclap headache two months after starting teprotumumab, with subarachnoid hemorrhage (SAH) and CVS revealed on neuroimaging studies. The patient improved on verapamil and was discharged without deficits. CONCLUSIONS: The temporal correlation between teprotumumab initiation and RCVS's symptom onset raises concern for the potential involvement of teprotumumab in triggering RCVS via disrupting cerebrovascular modulation. Further research is needed to investigate this proposed association.
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Reversible cerebral vasoconstriction syndrome (RCVS) is a complex neurovascular disorder characterized by repetitive thunderclap headaches and reversible cerebral vasoconstriction. The pathophysiological mechanism of this mysterious syndrome remains underexplored and there is no clinically available molecular biomarker. To provide insight into the pathogenesis of RCVS, this study reported the first landscape of dysregulated proteome of cerebrospinal fluid (CSF) in patients with RCVS (n = 21) compared to the age- and sex-matched controls (n = 20) using data-independent acquisition mass spectrometry. Protein-protein interaction and functional enrichment analysis were employed to construct functional protein networks using the RCVS proteome. An RCVS-CSF proteome library resource of 1054 proteins was established, which illuminated large groups of upregulated proteins enriched in the brain and blood-brain barrier (BBB). Personalized RCVS-CSF proteomic profiles from 17 RCVS patients and 20 controls reveal proteomic changes involving the complement system, adhesion molecules, and extracellular matrix, which may contribute to the disruption of BBB and dysregulation of neurovascular units. Moreover, an additional validation cohort validated a panel of biomarker candidates and a two-protein signature predicted by machine learning model to discriminate RCVS patients from controls with an area under the curve of 0.997. This study reveals the first RCVS proteome and a potential pathogenetic mechanism of BBB and neurovascular unit dysfunction. It also nominates potential biomarker candidates that are mechanistically plausible for RCVS, which may offer potential diagnostic and therapeutic opportunities beyond the clinical manifestations.
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Biomarcadores , Proteoma , Humanos , Femenino , Proteoma/metabolismo , Masculino , Adulto , Biomarcadores/líquido cefalorraquídeo , Biomarcadores/metabolismo , Vasoconstricción , Persona de Mediana Edad , Cefaleas Primarias/líquido cefalorraquídeo , Cefaleas Primarias/metabolismo , Proteómica/métodos , Estudios de Casos y Controles , Mapas de Interacción de Proteínas , SíndromeRESUMEN
Reversible cerebral vasoconstriction syndrome (RCVS) poses a complex neurological challenge characterized by sudden, severe headaches and multifocal cerebral vasoconstriction. While our understanding of its clinical aspects and underlying mechanisms has advanced, the focus of investigation remains on radiological manifestations. This systematic review aims to comprehensively analyze the existing literature on radiological findings in RCVS, synthesizing evidence from diverse imaging modalities to enhance the understanding of imaging features associated with the syndrome. Accurate diagnosis based on radiological findings is pivotal for initiating appropriate management and preventing complications. Specific markers may facilitate the differentiation of RCVS from other conditions, thereby enhancing patient care. This review explores a wide range of radiological presentations, from vasoconstriction to infarctions and hemorrhages, thereby refining diagnostic criteria and guiding clinical practice. By consolidating current knowledge, the review sheds light on areas of consensus, controversies, and gaps, with the aim of serving as a comprehensive resource for evidence-based decision-making.
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Females are at higher risk than males for a multitude of cerebrovascular conditions, both common and rare; partially resulting from a complex interplay between differing process involving genetics, hormonal influences, common cerebrovascular risk factors among others. Specific topics including cervical artery dissection, cerebral dural sinus venous thrombosis, reversible cerebral vasoconstriction syndrome, migraine, along with these disorders in the setting of pregnancy, puerperium and oral contraceptive utilization. Epidemiology, pathophysiology, presentation, basics of management and outcomes are presented, with sex differences throughout.
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Trastornos Migrañosos , Trombosis de los Senos Intracraneales , Vasoconstricción , Humanos , Femenino , Embarazo , Trastornos Migrañosos/fisiopatología , Trastornos Migrañosos/diagnóstico , Factores de Riesgo , Masculino , Trombosis de los Senos Intracraneales/fisiopatología , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/complicaciones , Factores Sexuales , Anticonceptivos Orales/efectos adversos , Accidente Cerebrovascular/diagnóstico , Accidente Cerebrovascular/fisiopatología , Accidente Cerebrovascular/etiología , Periodo Posparto , Disparidades en el Estado de Salud , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/complicaciones , Disección Aórtica/fisiopatología , Medición de Riesgo , Pronóstico , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Circulación CerebrovascularRESUMEN
This report describes the case of an 18-year-old Micronesian pregnant woman at 32 weeks gestation, initially presumed to have eclampsia but later diagnosed with reversible cerebral vasoconstriction syndrome (RCVS). She presented with seizures, altered mental status, nystagmus, lower extremity weakness, and absent reflexes. An extensive workup ruled out infectious and autoimmune causes, but a computed tomography angiogram (CTA) revealed severe cerebral vasoconstriction. Treatment included levetiracetam, intravenous magnesium, and nimodipine. The case highlights the challenge of differentiating RCVS from eclampsia in the postpartum period, emphasizing the importance of considering alternative diagnoses and brain CTA when RCVS is suspected, with calcium channel blockers potentially contributing to favorable neurological outcomes.
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Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular disorder highlighted by diffuse and multifocal vasoconstriction of the cerebral circulation. This syndrome has been reported to be associated with provoking vasoactive agents, and the identification of such offenders is quite challenging. In our case, the patient's RCVS was caused by the ingestion of loperamide. Although being reported in the cardiac literature, cerebral vasoconstriction due to loperamide has not been reported yet.
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Thunderclap headache is a sudden severe headache with onset to peak within one minute. Multiple excruciating, short-lived thunderclap headaches over a few days to weeks are highly suggestive of reversible cerebral vasoconstriction syndrome (RCVS). RCVS can be primary or secondary to several factors, but it is rarely described after neuro-endovascular procedures using onyx material. A 10-year-old child presented with RCVS heralded by recurrent thunderclap headache following endovascular embolization of pial arteriovenous malformation with onyx material (contains organic solvent dimethyl sulfoxide). Dimethyl sulfoxide is an angiotoxic material that can cause dysregulation of cerebral vascular tone triggering reversible cerebral vasoconstriction syndrome. Recurrent thunderclap headache after embolization procedures using onyx material should prompt for the diagnosis of reversible cerebral vasoconstriction syndrome.
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Dimetilsulfóxido , Embolización Terapéutica , Cefaleas Primarias , Malformaciones Arteriovenosas Intracraneales , Polivinilos , Humanos , Embolización Terapéutica/métodos , Niño , Cefaleas Primarias/etiología , Cefaleas Primarias/terapia , Dimetilsulfóxido/efectos adversos , Malformaciones Arteriovenosas Intracraneales/terapia , Malformaciones Arteriovenosas Intracraneales/diagnóstico por imagen , Malformaciones Arteriovenosas Intracraneales/complicaciones , Masculino , Vasoespasmo Intracraneal/diagnóstico por imagen , Vasoespasmo Intracraneal/etiología , Vasoespasmo Intracraneal/terapia , Femenino , RecurrenciaAsunto(s)
Glomerulonefritis , Infecciones Estreptocócicas , Humanos , Infecciones Estreptocócicas/complicaciones , Glomerulonefritis/complicaciones , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/etiología , Niño , Masculino , Femenino , Vasoespasmo Intracraneal/diagnóstico por imagen , Vasoespasmo Intracraneal/etiología , Vasoespasmo Intracraneal/complicaciones , Imagen por Resonancia MagnéticaRESUMEN
Objectives: This study aimed to analyze the Vaccine Adverse Event Reporting System (VAERS) database and systematically review the literature to provide a comprehensive analysis of reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES) secondary to vaccination. Methods: The authors analyzed the VAERS database and conducted a systematic review following PRISMA guidelines. The inclusion criteria for VAERS data were a score of ≥3 on the RCVS2 score and/or radiographic findings consistent with the diagnosis of RCVS or PRES. The systematic review was registered with PROSPERO. Results: Our combined data set included 29 cases (9 RCVS and 20 PRES). Most cases were women (72.4%) with a mean age of 50.7 years (SD 19.4 years). Most cases were associated with COVID-19 mRNA vaccines (58.6% Moderna, 20.7% Pfizer). Hypertension (37.9%), hyperlipidemia (13.7%), chronic kidney disease (CKD) (10.3%), and end-stage renal disease (6.8%) were common comorbidities. Furthermore, 20.6% (6/29) of cases were on immunosuppression therapy for various reasons. The mean time to symptom onset was 10.49 days after vaccination (SD 18.60), and the mean duration of hospitalization was 7.42 days (SD 5.94). The symptoms reported the most frequently were headache (41.3%), elevated blood pressure (31.0%), and emesis (17.2%). Typical radiographic findings included T2/FLAIR hyperintensities affecting the parieto-occipital lobes, indicative of vasogenic and/or cytotoxic edema. Conclusions: This study provides a comprehensive analysis of postvaccine RCVS and PRES. Both disease states were seen most often in those with pre-existing risk factors such as female sex, age over 50, hypertension, renal disease, and immunosuppression. Vaccines and their associated immune response may cause endothelial dysfunction leading to cerebral vasospasm and loss of cerebral autoregulation. However, further research is required to understand the underlying pathophysiological mechanisms. Despite the associations found, the absolute risk of these syndromes remains extremely low compared to the immense benefits of vaccination.
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OBJECTIVE: With the development of diagnostic imaging, a new clinical entity called reversible cerebral vasoconstriction syndrome (RCVS), which is considered to be a cause of secondary headache, has emerged. We herein present two cases of RCVS with different patterns of clinical progression. CASE REPORT: Case 1 occurred during labor, whereas case 2 occurred after delivery. Neither case presnted thunderclap headache at the onset of symptoms. Hypertensive disorders of pregnancy did not occur during the pregnancy or the puerperium in either case. Neurological symptoms following mild headache (Case 1: coma; Case 2: paralysis of the right extremities) were observed. CONCLUSION: Even when a patient has no risk factors for RCVS and had no severe headache, it is important not to miss any of the neurological symptoms. Magnetic resonance imaging (MRI) strongly supports the diagnosis, even during pregnancy. In addition, the diagnosis should always be reviewed while excluding eclampsia.
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Trastornos Cerebrovasculares , Vasoconstricción , Embarazo , Femenino , Humanos , Imagen por Resonancia Magnética , Periodo Posparto , CefaleaRESUMEN
BACKGROUND: Pregnancy-associated spontaneous coronary artery dissection (SCAD) and reversible cerebral vasoconstriction syndrome (RCVS) are rare conditions that may occur during pregnancy and the postpartum period. The coexistence of both diseases may pose a risk to patients, potentially resulting in a variety of complications and clinical manifestations. This is considered the first case of a patient who successfully recovered from a critical condition in the postpartum period, with contemporaneous SCAD and RCVS. CASE PRESENTATION: A 33-year-old female with no known medical history was referred to the emergency department after experiencing cardiac arrest, which occurred 1 week after giving birth to her third child. She complained of sudden anterior squeezing chest pain, accompanied by a headache, and eventually collapsed due to ventricular fibrillation with seizure. She was successfully resuscitated after receiving basic life support. She showed an alert mentality and did not experience any further seizure events or additional neurological symptoms. Although vital sign remained stable, the level of highly sensitive troponin I was significantly elevated. Electrocardiography revealed sinus rhythm with T-wave inversion at V1-4, while chest computed tomography (CT) demonstrated severe aspiration pneumonia. The patient was admitted to the intensive care unit due to a high requirement of O2 supply. A consultation with the neurologic department and a brain magnetic resonance angiography (MRA) were conducted for the thunderclap headache. The brain MRA demonstrated stenosis in the basilar artery, the right M2 segment, and bilateral A1 segments, along with sulcal hyperintensity on post-contrast fluid-attenuated inversion recovery (FLAIR) suggesting blood-brain barrier breakdown due to vasoconstriction. Formal echocardiography showed regional wall motion abnormality in the left anterior descending artery (LAD) territory. After the improvement of pneumonia, a coronary angiography was performed, revealing diffuse luminal narrowing from the mid to distal LAD due to a long segmental, extensive dissection. We decided to maintain the medical therapy. A follow-up coronary CT angiography performed 6 months later revealed complete remission of the dissected coronary vessel, and a brain MRA checked 3 months later showed resolved vasoconstriction of the relevant cerebral vessels. CONCLUSIONS: The physicians must be aware of pregnancy-associated complications in certain patients. Clear diagnoses and proper treatments are required in pregnant patients who may be exposed to multiple acute conditions, in order to reduce complications and achieve favorable outcomes.
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INTRODUCTION: Reversible cerebral vasoconstriction syndrome is a complex neurovascular syndrome that presents with varying neurological deficits as well as segmental vasoconstriction of the small and medium cerebral arteries. There is limited literature on pathologies that mimic reversible cerebral vasoconstriction syndrome, so this study aims to understand what factors may impact the angiographic confirmation of reversible cerebral vasoconstriction syndrome on follow-up and play a role in establishing the diagnosis. METHODS: The Clinical Research Data Warehouse at this institution was employed to search the medical records for patients with diagnosis and treatment of reversible cerebral vasoconstriction syndrome between January 2010 and May 2021. After screening, 32 patients met the inclusion criteria for a presumed diagnosis of reversible cerebral vasoconstriction syndrome with both angiography on presentation and at three-month follow-up after treatment. Patients were divided into two categories: those with complete angiographic resolution, versus partial or no improvement on follow-up. Clinical and radiographic data were analyzed. RESULTS: Patients who had partial or no resolution were more likely to have a history of hypertension (p = 0.001), higher systolic blood pressure on admission (p = 0.047), and present with a recurrent thunderclap headache (p = 0.038). Binary logistic regression selected for hypertension (odds ratio [OR] 18.35 [95% CI, 1.37-245.1]) as predictive of not having reversible cerebral vasoconstriction syndrome, as can be seen by partial or no resolution on follow-up angiography (p = 0.028). CONCLUSION: Complete resolution on follow-up angiography is a distinguishing factor of reversible cerebral vasoconstriction syndrome. Our analysis revealed that a history of hypertension is the most significant predictor of confirming that a patient may not have reversible cerebral vasoconstriction syndrome. This is due, in part, to increased atherosclerotic or hypertensive cerebral arterial changes, which can mimic reversible cerebral vasoconstriction syndrome and present as partial or no resolution on angiography.
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Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is most commonly characterized by focal or diffuse severe thunderclap headache with or without focal neurological deficits and associated transient focal vasoconstriction of the intracerebral arteries lasting up to three months. We present six patients diagnosed as RCVS, with three patients presenting with focal neurological deficits without headache and the remaining three with severe headache alone. Neuroimaging revealed focal subarachnoid bleed with or without segmental intracerebral vasospasm, which resolved over three months. Despite thunderclap headache being the most prevalent symptom associated with RCVS, the absence of this symptom should not preclude the diagnosis, especially in the presence of cortical subarachnoid hemorrhage (SAH) or focal segmental intracerebral arterial narrowing. Methods This case series is a retrospective analysis of all patients diagnosed with RCVS between 2018 and 2022, focusing on clinical symptoms, imaging findings, and management. Results Six patients (three males and three females) were diagnosed with RCVS between 2018 and 2022. Three patients presented with typical symptoms, while the remaining three presented with atypical symptoms. Neuroimaging findings ranged from normal to focal SAH with or without arterial narrowing. Conclusion This case series underscores the diverse clinical presentations of RCVS, emphasizing that while thunderclap headache is the predominant symptom, its absence should not exclude the possibility of RCVS, especially when accompanied by focal neurological deficits or cortical SAH. Neuroimaging played a crucial role in identifying the spectrum of findings. These findings highlight the importance of comprehensive evaluation and consideration of RCVS in patients presenting with neurological symptoms, even in the absence of typical headache features.
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BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) may cause ischaemic stroke and intracranial haemorrhage. The aim of our study was to assess the frequency of the afore-mentioned outcomes. METHODS: We performed a PROSPERO-registered (CRD42022355704) systematic review and meta-analysis accessing PubMed until 7 November 2022. The inclusion criteria were: (1) original publication, (2) adult patients (≥18 years), (3) enrolling patients with PRES and/or RCVS, (4) English language and (5) outcome information. Outcomes were frequency of (1) ischaemic stroke and (2) intracranial haemorrhage, divided into subarachnoid haemorrhage (SAH) and intraparenchymal haemorrhage (IPH). The Cochrane Risk of Bias tool was used. RESULTS: We identified 848 studies and included 48 relevant studies after reviewing titles, abstracts and full text. We found 11 studies on RCVS (unselected patients), reporting on 2746 patients. Among the patients analysed, 15.9% (95% CI 9.6%-23.4%) had ischaemic stroke and 22.1% (95% CI 10%-39.6%) had intracranial haemorrhage. A further 20.3% (95% CI 11.2%-31.2%) had SAH and 6.7% (95% CI 3.6%-10.7%) had IPH. Furthermore, we found 28 studies on PRES (unselected patients), reporting on 1385 patients. Among the patients analysed, 11.2% (95% CI 7.9%-15%) had ischaemic stroke and 16.1% (95% CI 12.3%-20.3%) had intracranial haemorrhage. Further, 7% (95% CI 4.7%-9.9%) had SAH and 9.7% (95% CI 5.4%-15%) had IPH. CONCLUSIONS: Intracranial haemorrhage and ischaemic stroke are common outcomes in PRES and RCVS. The frequency reported in the individual studies varied considerably.
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Hemorragias Intracraneales , Accidente Cerebrovascular Isquémico , Síndrome de Leucoencefalopatía Posterior , Humanos , Síndrome de Leucoencefalopatía Posterior/epidemiología , Accidente Cerebrovascular Isquémico/epidemiología , Accidente Cerebrovascular Isquémico/complicaciones , Hemorragias Intracraneales/epidemiología , Hemorragias Intracraneales/etiología , Hemorragias Intracraneales/complicaciones , Vasoespasmo Intracraneal/epidemiologíaRESUMEN
We systematically reviewed case reports of posterior reversible encephalopathy syndrome (PRES), and investigated the characteristics of PRES in pregnant Japanese women and the clinical relevance of reversible cerebral vasoconstriction syndrome (RCVS) in pregnant women with PRES. Articles were collected using the PubMed/Medline and Ichushi-Web databases. This review was ultimately conducted on 121 articles (162 patients). The clinical characteristics of PRES, individual sites of PRES lesions, edema types, and clinical characteristics of RCVS in PRES cases were examined. The most common individual site of PRES lesion was the occipital lobe (83.3%), followed by the basal ganglia, parietal lobe, frontal lobe, brain stem, cerebellum, temporal lobe, thalamus, and splenium corpus callosum (47.5, 42.6, 24.7, 16.1, 9.3, 5.6, 4.3, and 0.0%, respectively). Edema types in 79 cases with PRES were mainly the vasogenic edema type (91.1%), with very few cases of the cytotoxic edema type (3.8%) and mixed type (5.1%). Among 25 PRES cases with RCVS, RCVS was not strongly suspected in 17 (68.0%) before magnetic resonance angiography. RCVS was observed at the same time as PRES in 13 cases (approximately 50%), and between days 1 and 14 after the onset of PRES in the other 12. These results suggest that the basal ganglia is a frequent site of PRES lesions in pregnant women. RCVS may occur at or after the onset of PRES, even if there are no symptoms to suggest RCVS.
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Síndrome de Leucoencefalopatía Posterior , Adulto , Femenino , Humanos , Embarazo , Encéfalo/diagnóstico por imagen , Encéfalo/irrigación sanguínea , Relevancia Clínica , Japón/epidemiología , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Vasoconstricción/fisiología , Vasoespasmo Intracraneal/diagnóstico por imagen , Informes de Casos como AsuntoRESUMEN
BACKGROUND: The pathophysiology of the reversible cerebral vasoconstriction syndrome (RCVS) remains enigmatic and the role of glymphatics in RCVS pathophysiology has not been evaluated. We aimed to investigate RCVS glymphatic dynamics and its clinical correlates. METHODS: We prospectively evaluated the glymphatic function in RCVS patients, with RCVS subjects and healthy controls (HCs) recruited between August 2020 and November 2023, by calculating diffusion-tensor imaging along the perivascular space (DTI-ALPS) index under a 3-T MRI. Clinical and vascular (transcranial color-coded duplex sonography) investigations were conducted in RCVS subjects. RCVS participants were separated into acute (≤ 30 days) and remission (≥ 90 days) groups by disease onset to MRI interval. The time-trend, acute stage and longitudinal analyses of the DTI-ALPS index were conducted. Correlations between DTI-ALPS index and vascular and clinical parameters were performed. Bonferroni correction was applied to vascular investigations (q = 0.05/11). RESULTS: A total of 138 RCVS patients (mean age, 46.8 years ± 11.8; 128 women) and 42 HCs (mean age, 46.0 years ± 4.5; 35 women) were evaluated. Acute RCVS demonstrated lower DTI-ALPS index than HCs (p < 0.001) and remission RCVS (p < 0.001). A continuously increasing DTI-ALPS trend after disease onset was demonstrated. The DTI-ALPS was lower when the internal carotid arteries resistance index and six-item Headache Impact test scores were higher. In contrast, during 50-100 days after disease onset, the DTI-ALPS index was higher when the middle cerebral artery flow velocity was higher. CONCLUSIONS: Glymphatic function in patients with RCVS exhibited a unique dynamic evolution that was temporally coupled to different vascular indices and headache-related disabilities along the disease course. These findings may provide novel insights into the complex interactions between glymphatic transport, vasomotor control and pain modulation.