RESUMEN
BACKGROUND AND PURPOSE: Coma is an independent predictor of poor clinical outcomes in cerebral venous thrombosis (CVT). We aimed to describe the association of age, sex, and radiological characteristics of adult coma patients with CVT. METHODS: We used data from the international, multicentre prospective observational BEAST (Biorepository to Establish the Aetiology of Sinovenous Thrombosis) study. Only positively associated variables with coma with <10% missing data in univariate analysis were considered for the multivariate logistic regression model. RESULTS: Of the 596 adult patients with CVT (75.7% women), 53 (8.9%) patients suffered coma. Despite being a female-predominant disease, the prevalence of coma was higher among men than women (13.1% vs. 7.5%, p = 0.04). Transverse sinus thrombosis was least likely to be associated with coma (23.9% vs. 73.3%, p < 0.001). The prevalence of superior sagittal sinus thrombosis was higher among men than women in the coma sample (73.6% vs. 37.5%, p = 0.01). Men were significantly older than women, with a median (interquartile range) age of 51 (38.5-60) versus 40 (33-47) years in the coma (p = 0.04) and 44.5 (34-58) versus 37 (29-48) years in the non-coma sample (p < 0.001), respectively. Furthermore, an age- and superior sagittal sinus-adjusted multivariate logistic regression model found male sex (odds ratio = 1.8, 95% confidence interval [CI] = 1.0-3.4, p = 0.04) to be an independent predictor of coma in CVT, with an area under the receiver operating characteristic curve of 0.61 (95% CI = 0.52-0.68, p = 0.01). CONCLUSIONS: Although CVT is a female-predominant disease, men were older and nearly twice as likely to suffer from coma than women.
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Coma , Humanos , Masculino , Femenino , Coma/etiología , Coma/epidemiología , Adulto , Persona de Mediana Edad , Trombosis Intracraneal/epidemiología , Trombosis Intracraneal/complicaciones , Estudios Prospectivos , Trombosis de la Vena/epidemiología , Trombosis de la Vena/complicaciones , Trombosis de los Senos Intracraneales/epidemiología , Trombosis de los Senos Intracraneales/complicaciones , Factores Sexuales , Factores de Edad , PrevalenciaRESUMEN
BACKGROUND: Idiopathic nephrotic syndrome (NS) presents as a hypercoagulable state, of which thromboembolism (TE) is a well-known life-threatening complication. Although TE is more likely to occur in venous vessels than arterial vessels, arterial TE is important because it may cause after-effects, including tissue necrosis and cerebral infarction (CI); therefore, prompt diagnosis and appropriate treatment are required. We report a pediatric NS case with multiple CIs. CASE PRESENTATION: A 14-year-7-month-old Japanese girl was diagnosed with frequent relapsing NS, accompanied by headache and disturbance of consciousness during the second relapse. Brain magnetic resonance imaging (MRI) and four-dimensional computed tomography revealed multiple CIs, vasogenic edema, and cerebral venous sinus thrombosis (CVST). The patient had no underlying thrombophilia other than hypercoagulability due to NS and prednisolone (PSL), and no cardiac arrhythmia; however, a right-to-left shunt through the patent foramen ovale (PFO) was observed with the Valsalva maneuver by echocardiography. Therefore, we assumed that a potential cause of multiple CIs might be an embolic stroke, caused by thrombosis formed from a hypercoagulable state due to NS and PSL treatment and reached through PFO. Antiplatelet and anticoagulant therapies were administered for TE. She was treated with PSL and mycophenolate mofetil (MMF) for NS. Rituximab (RTX) was administered to prevent NS relapse after complete remission (CR). She underwent transcatheter PFO closure at age 14 years and 9 months because we considered that the right-to-left shunt through the PFO would be one of the risks for recurrent cerebral embolism when NS relapses. One year after the onset of CIs, an MRI indicated that the CVST had resolved, leaving no neurological sequelae due to CI; therefore, anticoagulant therapy was discontinued. And then she has been in CR for NS with only MMF therapy. CONCLUSIONS: CI is a serious complication in patients with NS. The pathogenesis of multiple CIs is various, including right-to-left shunt through PFO, in addition to the hypercoagulability due to NS. It is important to investigate and manage underlying risks such as PFO, besides preventing the relapses of NS by aggressive treatments using MMF and RTX, in patients with NS.
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Infarto Cerebral , Foramen Oval Permeable , Síndrome Nefrótico , Recurrencia , Trombosis de los Senos Intracraneales , Humanos , Femenino , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/etiología , Trombosis de los Senos Intracraneales/tratamiento farmacológico , Síndrome Nefrótico/complicaciones , Adolescente , Foramen Oval Permeable/complicaciones , Foramen Oval Permeable/diagnóstico por imagen , Infarto Cerebral/etiología , Infarto Cerebral/diagnóstico por imagenRESUMEN
PURPOSE: Cerebral sinovenous thrombosis is an increasingly recognized cause of stroke in children and neonates. Its true incidence appears to be underestimated. Despite being a rare event, certain studies have found a correlation between subdural hemorrhage and cerebral sinus thrombosis. The literature suggests that spontaneous cerebral sinovenous thrombosis in the pediatric population may lead to the occurrence of a subdural hemorrhage. In this report, we present a case of cerebral venous thrombosis associated with chronic subdural hematoma and review the literature to highlight the importance of these conditions. CASE REPORT: An 11-year-old boy was admitted in the neurosurgery department with headache and a neurological examination without changes. The imaging studies identified a heterogeneous subdural collection in the fronto-temporo-parietal region. The patient underwent surgical drainage of the subdural hematoma, and the procedure was performed without complications. The magnetic resonance and angiography showed an extensive thrombosis of the superior sagittal sinus, extending downward to the occipital sinus and partially to the right transverse sinus. CONCLUSIONS: Appropriate management in the diagnosis and an early treatment of dural sinus thrombosis associated with subdural hemorrhage can reduce the risk of recurrence and improve the clinical outcome.
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Senos Craneales , Trombosis de los Senos Intracraneales , Niño , Masculino , Recién Nacido , Humanos , Hematoma Subdural/complicaciones , Hematoma Subdural/diagnóstico por imagen , Hematoma Subdural/cirugía , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/cirugía , Imagen por Resonancia Magnética/efectos adversos , Seno Sagital Superior/patologíaRESUMEN
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
We describe a rare and complex case of septic cavernous sinus thrombosis (SCST) in a 70-year-old patient who initially presented with ocular symptoms that rapidly progressed to severe intracranial vascular complications, including subarachnoid hemorrhage (SAH). Despite the use of broad-spectrum antibiotics and anticoagulants, the patient's condition deteriorated. SCST, often caused by sinus infections, presents a significant diagnostic and therapeutic dilemma, with mortality rates exceeding 20%. This report underscores the diversity of clinical presentations, ranging from mild headaches to severe cranial nerve deficits, that complicate diagnosis and treatment. The inability to detect any aneurysms in our patient using magnetic resonance imaging (MRI) and computed tomography angiography (CTA) may indicate an alternative pathogenesis. This could involve venous hypertension and endothelial hyperpermeability. This case illustrates the need for personalized treatment approaches, as recommended by the European Federation of Neurological Societies, and the importance of a multidisciplinary perspective when managing such intricate neurological conditions. Our findings contribute to the understanding of SCST coexisting with SAH.
Asunto(s)
Trombosis del Seno Cavernoso , Trombosis de los Senos Intracraneales , Hemorragia Subaracnoidea , Humanos , Anciano , Trombosis del Seno Cavernoso/complicaciones , Trombosis del Seno Cavernoso/diagnóstico , Hemorragia Subaracnoidea/complicaciones , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/tratamiento farmacológico , Anticoagulantes/uso terapéutico , Imagen por Resonancia Magnética/efectos adversosRESUMEN
Whole exome sequencing (WES) has been used to detect rare causative variants in neurological diseases. However, the efficacy of WES in genetic diagnosis of clinically heterogeneous familial stroke remains inconclusive. We prospectively searched for disease-causing variants in unrelated probands with defined familial stroke by candidate gene/hotspot screening and/or WES, depending on stroke subtypes and neuroimaging features at a referral center. The clinical significance of each variant was determined according to the American College of Medical Genetics guidelines. Among 161 probands (mean age at onset 53.2 ± 13.7 years; male 63.4%), 33 participants (20.5%) had been identified with 19 pathogenic/likely pathogenic variants (PVs; WES applied 152/161 = 94.4%). Across subtypes, the highest hit rate (HR) was intracerebral hemorrhage (ICH, 7/18 = 38.9%), particularly with the etiological subtype of structural vasculopathy (4/4 = 100%, PVs in ENG, KRIT1, PKD1, RNF213); followed by ischemic small vessel disease (SVD, 15/48 = 31.3%; PVs in NOTCH3, HTRA1, HBB). In contrast, large artery atherosclerosis (LAA, 4/44 = 9.1%) and cardioembolism (0/11 = 0%) had the lowest HR. NOTCH3 was the most common causative gene (16/161 = 9.9%), presenting with multiple subtypes of SVD (n = 13), ICH (n = 2), or LAA (n = 1). Importantly, we disclosed two previously unreported PVs, KRIT1 p.E379* in a familial cerebral cavernous malformation, and F2 p.F382L in a familial cerebral venous sinus thrombosis. The contribution of monogenic etiologies was particularly high in familial ICH and SVD subtypes in our Taiwanese cohort. Utilizing subtype-guided hotspot screening and/or subsequent WES, we unraveled monogenic causes in 20.5% familial stroke probands, including 1.2% novel PVs. Genetic diagnosis may enable early diagnosis, management and lifestyle modification. Among 161 familial stroke probands, 33 (20.5%) had been identified pathogenic or likely pathogenic monogenic variants related to stroke. The positive hit rate among all subtypes was high in intracerebral hemorrhage (ICH) and ischemic small vessel disease (SVD). Notably, two previously unreported variants, KRIT1 p.E379* in a familial cerebral cavernous malformation and F2 p.F382L in familial cerebral venous sinus thrombosis, were disclosed. CVT cerebral venous thrombosis; HTN Hypertensive subtype; LAA large artery atherosclerosis; SV structural vasculopathy; U Undetermined.
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Aterosclerosis , Accidente Cerebrovascular Isquémico , Trombosis de los Senos Intracraneales , Accidente Cerebrovascular , Humanos , Masculino , Adulto , Persona de Mediana Edad , Anciano , Secuenciación del Exoma , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/genética , Accidente Cerebrovascular/diagnóstico , Hemorragia Cerebral/complicaciones , Hemorragia Cerebral/diagnóstico por imagen , Hemorragia Cerebral/genética , Aterosclerosis/complicaciones , Isquemia/complicaciones , Trombosis de los Senos Intracraneales/complicaciones , Adenosina Trifosfatasas , Ubiquitina-Proteína LigasasRESUMEN
OBJECTIVE: This study aimed to assess the clinical characteristics of cerebral venous sinus thrombosis (CVT) patients with new-onset headache and to identify the risk factors for headache in this population. METHODS: We retrospectively reviewed the demographic and clinical data of 69 CVT patients recruited between September 2017 and September 2019. Patients were classified into two groups, the headache group and the non-headache group, according to the presence or absence of new-onset headache symptoms at admission. The following characteristics and parameters were measured and analyzed, including gender, age, amount of thromboembolic cerebral venous sinus(ATCVS), and other relevant indicators. RESULTS: The incidence of headache was 75% in this cohort. The proportion of female patients in the headache group was higher than that in the non-headache group. Patients in the headache group were younger than those without headache. CVT patients of headache group showed higher lymphocyte ratio (LR), blood urea nitrogen (BUN), and intracranial pressure (ICP) compared to the non-headache group, whereas mean corpuscular volume (MCV) and levels of protein (cerebrospinal fluid, CSF) and lactic dehydrogenase (LDH) in CSF were lower in headache patients. The data also revealed younger age and the increased level of chloride ion CI-(CSF) were the risk factors for the occurrence of headache in CVT patients. CONCLUSION: Age, LR, MCV, BUN levels, ICP, protein (CSF), and LDH (CSF) in patients with headache were significantly different from those in the non-headache group at admission. Younger age and a level of CI- (CSF) were risk factors for headache in CVT patients. These findings may provide guidance for clinical diagnosis and treatment of CVT.
Asunto(s)
Venas Cerebrales , Trombosis Intracraneal , Trombosis de los Senos Intracraneales , Trombosis de la Vena , Humanos , Femenino , Estudios Retrospectivos , Trombosis de la Vena/diagnóstico , Cefalea/etiología , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/epidemiología , Trombosis Intracraneal/complicacionesRESUMEN
BACKGROUND: The Dural Arteriovenous Fistulas (DAVFs) secondary to cerebral venous sinus thrombosis (CVST) are rather rare. The aim of present study is to investigate the clinical and radiological features, and treatment outcome of DAVFS in patients following CVST. METHODS: Data about demographic information, clinical presentations, radiological findings, as well as treatment and outcome of DAVFs sequence to CVST were collected to analysis from January 2013 to September 2020 in this retrospective study. RESULTS: Fifteen patients with DAVFs after CVST were included in the study. The median age was 41 years (range17-76 years). Ten patients (66.67%) were male and 6 patients (33.33%) were female. The median duration of presenting CVST was 182 days (Range 20-365). Mean time from diagnosis of CVST to confirmation of DAVFs was 97 days (range 36-370 days). The most common manifestations of DAVFs following CVST were headache and visual disturbance seen in 7 patients respectively. Five patients had pulsatile tinnitus (%) and 2 had nausea/vomiting. The DAVFs are most frequently located at the transverse/sigmoid sinus (7/15, 46.67%), followed by the superior sagittal the sinus and confluence sinus (6/15, 40.00%) respectively. Angiography of DAVFs revealed Board type I in seven (46.7%) patients, Board type II and III in 4(26.7%) patients, respectively. The Cognard I was noted in seven (46.7%), Cognard IIa and IV in 3 patients, IIb and III in one patient, respectively. The main feeding arteries of DAVFs most commonly originate from the branches of the external carotid artery in 6 (40.0%) patients. The other DAVFs are conjointly supplied by multiple feeders from internal and external carotid artery and vertebral arteries. Fourteen (93.33%) patients were treated with endovascular embolization and none of the patients had permanent deficits during follow-up. CONCLUSION: Intracranial DAVFs following CVST are rare presentations. Most patients have a good outcome after timely interventional therapy. Continued observation and follow-up of (DSA) are important to find DAVFs secondary to CVST.
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Malformaciones Vasculares del Sistema Nervioso Central , Embolización Terapéutica , Trombosis de los Senos Intracraneales , Humanos , Masculino , Femenino , Adulto , Estudios Retrospectivos , Senos Craneales , Resultado del Tratamiento , Malformaciones Vasculares del Sistema Nervioso Central/complicaciones , Malformaciones Vasculares del Sistema Nervioso Central/diagnóstico por imagen , Malformaciones Vasculares del Sistema Nervioso Central/terapia , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/terapia , Angiografía CerebralRESUMEN
BACKGROUND: Cerebral venous sinus thrombosis (CVST) is typically associated with a prothrombotic state of the blood, with its causative factors varying widely. Prior research has not reported the simultaneous occurrence of CVST and dural arteriovenous fistulas (DAVFs) as potentially resulting from genetic mutations. In this case report, we introduce a unique occurrence wherein a patient with a heterozygous mutation of the low-density lipoprotein receptor (LDLR) gene presented with CVST in conjunction with DAVFs. CASE: Presentation: A male patient, aged 51, sought treatment at our facility due to a consistent decline in cognitive functions accompanied by recurrent headaches. Comprehensive evaluations were administered, including neurological examinations, laboratory tests, magnetic resonance imaging, digital subtraction angiography, and whole exome sequencing. Digital subtraction angiography identified DAVFs in the patient's right sigmoid sinus and an occlusion within the left transverse sinus. The whole exome sequencing of blood samples pinpointed a heterozygous mutation in the LDLR gene (NM_000527:exon12:c.C1747T:p.H583Y). Following the confirmed diagnosis of CVST and DAVFs, the patient underwent anticoagulant therapy combined with endovascular procedures - these comprised embolization of the arteriovenous fistula in the right sigmoid sinus and balloon dilation with stent implantation in the left transverse sinus. A six-month follow-up indicated a significant abatement in the patient's symptoms. CONCLUSIONS: This report marks the first documented case of an LDLR gene mutation that could be associated with the onset of CVST and DAVFs. The mutation in the LDLR gene might foster a prothrombotic environment, facilitating the gradual emergence of CVST and the subsequent genesis of DAVFs.
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Malformaciones Vasculares del Sistema Nervioso Central , Embolización Terapéutica , Trombosis de los Senos Intracraneales , Humanos , Masculino , Malformaciones Vasculares del Sistema Nervioso Central/complicaciones , Malformaciones Vasculares del Sistema Nervioso Central/diagnóstico por imagen , Malformaciones Vasculares del Sistema Nervioso Central/genética , Angiografía Cerebral , Senos Craneales , Embolización Terapéutica/métodos , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/genética , Persona de Mediana EdadRESUMEN
BACKGROUND: Cerebral venous and sinus thrombosis (CVST) can cause increased intracranial pressure, often leading to papilledema. In this study, we investigated the association between papilledema and venous stasis on susceptibility weighted imaging (SWI) in CVST. METHODS: Patients with CVST between 2008 and 2020 were reviewed. Patients without fundoscopic examination or SWI were excluded in this study. Venous stasis was evaluated and scored for each cerebral hemisphere: each hemisphere was divided into 5 regions according to the venous drainage territories (superior sagittal sinus, Sylvian veins, transverse sinus and vein of Labbé, deep cerebral veins, and medullary veins) and 1 point was added if venous prominence was confirmed in one territory on SWI. The venous stasis score on SWI between cerebral hemispheres with and without papilledema was compared. RESULTS: Eight of 19 patients with CVST were excluded because of the absence of fundoscopic examination or SWI. Eleven patients (26.5 ± 2.1 years) were included in this study. Papilledema was identified in 6 patients: bilateral papilledema in 4 patients and unilateral papilledema in 2 patients. The venous stasis score on SWI was significantly higher (P = 0.013) in the hemispheres with papilledema (median, 4.0; 95% CI, 3.038-4.562) than in the hemispheres without papilledema (median, 2.5; 95% CI, 0.695-2.805). CONCLUSIONS: This study shows that higher score of venous stasis on SWI is associated with papilledema. Therefore, the venous stasis on SWI may be an imaging surrogate marker of increased intracranial pressure in patients with CVST.
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Venas Cerebrales , Hipertensión Intracraneal , Papiledema , Trombosis de los Senos Intracraneales , Humanos , Venas Cerebrales/diagnóstico por imagen , Papiledema/diagnóstico por imagen , Papiledema/etiología , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Imagen por Resonancia Magnética , Hipertensión Intracraneal/complicacionesRESUMEN
BACKGROUND: Tuberculous meningitis (TBM), complicated with cerebral venous thrombosis (CVT), has been sparsely reported and needs to be investigated further. METHODS: Among those with tuberculous meningitis in Haihe Hospital, Tianjin University, 3 patients with venous sinus thrombosis were identified retrospectively. "Tuberculous meningitis" and "cerebral venous thrombosis" were used as keywords, and the retrieved literature was summarized and analyzed. Our data were combined with previously reported case data to describe this new condition. RESULTS: Among 28 patients with a median onset age of 31 years for TBM, 17 were females. The manifestations were fever, headache, and seizure. Magnetic resonance imaging (MRI) venography showed that the most common site of venous sinus thrombosis involved superior sagittal sinus, left transverse sinus, left sigmoid sinus, cavernous sinus, and straight sinus. The abnormalities found on MRI include hydrocephalus, exudates, hemorrhage, meningeal enhancement, infarction, and tuberculoma. In the acute phase, all patients received standard anti-TB treatment, and 14/28 patients received anticoagulant treatment. The mortality rate of these patients was 17.9%, and 21/28 (75%) became functionally independent. CONCLUSIONS: CVT is one of the rare complications of TMB and must be considered a differential diagnosis in patients with TBM who show poor clinical features and/or develop new neurological signs.
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Trombosis Intracraneal , Trombosis de los Senos Intracraneales , Tuberculosis Meníngea , Trombosis de la Vena , Femenino , Humanos , Adulto , Masculino , Tuberculosis Meníngea/complicaciones , Tuberculosis Meníngea/diagnóstico por imagen , Tuberculosis Meníngea/tratamiento farmacológico , Estudios Retrospectivos , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/tratamiento farmacológico , Imagen por Resonancia Magnética , Trombosis Intracraneal/complicacionesRESUMEN
BACKGROUND: Bacillus cereus infections in immunocompetent patients are uncommon and mainly observed in fragile patients. It can cause lethal infections with multiple organ dysfunction syndrome (MODS). However, a patient presenting as venous sinus thrombosis and survival without sequela has not been reported. CASE PRESENTATION: A 20-year-old previously healthy male developed gastroenteritis after a meal, followed by fever, convulsions, and severe disturbance of consciousness. The patient had significant leukocytosis with a mildly elevated D-dimer, creatinine level, and respiratory failure. The CT(computed tomography) revealed fatal brain edema and subarachnoid hemorrhage. Previous blood culture in a local hospital revealed B. cereus, which was confirmed by mNGS(metagenomic next-generation sequencing) using blood and urine in our hospital. Accordingly, B. cereus sepsis with MODS were considered. Later, cerebral venous sinus thrombosis was proved. After anti-infection (linezolid 0.6 g, Q12h; and meropenem 1.0 g, Q8h), anti-coagulant (enoxaparin 6000U, Q12h), and other symptomatic treatments, the patient recovered completely without sequela at the 6-month follow-up. CONCLUSIONS: This case suggests that in immunocompetent adults, there is still a risk of infection with B. cereus, causing severe MODS. Special attention should be paid to venous sinus thrombosis and subarachnoid hemorrhage in such cases, while, anti-coagulant is essential therapy.
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Sepsis , Trombosis de los Senos Intracraneales , Hemorragia Subaracnoidea , Humanos , Masculino , Adulto , Adulto Joven , Hemorragia Subaracnoidea/etiología , Bacillus cereus , Sepsis/complicaciones , Sepsis/diagnóstico , Sepsis/tratamiento farmacológico , Trombosis de los Senos Intracraneales/complicacionesRESUMEN
AIM: To compare paediatric patients with cerebral sinovenous thrombosis (CSVT) with and without head/neck infection to improve management of the condition. METHOD: We conducted a bicentric retrospective study of consecutive children (neonates excluded) with radiologically confirmed CSVT, comparing children with a concurrent head/neck infection and children with other causes. RESULTS: A total of 84 consecutive patients (46 males and 38 females) with a median age of 4 years 6 months (range 3 months-17 years 5 months) were included. Associated head/neck infection was identified in 65.4% of cases and represented the main identified CSVT aetiology. Children in the head/neck infection group displayed a milder clinical presentation and less extensive CSVT. Median time to complete recanalization was significantly shorter in this group (89 days [interquartile range 35-101] vs 112.5 days [interquartile range 83-177], p = 0.005). These findings were even more pronounced in the subgroup of patients with otogenic infection and no neurological sign. INTERPRETATION: As CSVT in the setting of an otogenic infection and no neurological sign seems to represent a milder condition with a shorter course, these results suggest adapting current recommendations: consider earlier control imaging in paediatric otogenic CSVT, and shorter anticoagulant treatment if recanalization is obtained. WHAT THIS PAPER ADDS: Children with cerebral sinovenous thrombosis related to head/neck infections have a milder clinical presentation. They also have a shorter recanalization time, especially if there is otogenic infection without neurological symptoms.
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Trombosis de los Senos Intracraneales , Trombosis de la Vena , Niño , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Factores de Riesgo , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis/complicacionesRESUMEN
BACKGROUND: Dural venous sinus thrombosis (DVST) is an important cause of papilledema. Patients diagnosed with DVST should undergo work-up for underlying hypercoagulable state, including genetic causes. One important prothrombotic mutation is in the JAK2 gene, which is a driver of myeloproliferative neoplasms including polycythemia vera (PV). We aimed to determine the prevalence of JAK2 mutation in patients in presenting to neuro-ophthalmology clinic with DVST and papilledema. METHODS: Retrospective case series of patients seen in a tertiary neuro-ophthalmology practice who presented with papilledema due to DVST and were investigated for presence of JAK2 mutation. RESULTS: Four out of 15 patients with DVST (26%) were found to have JAK2 V617F mutation which led to subsequent diagnosis of PV in 2. One additional patient had a known diagnosis of essential thrombocytosis. We describe the clinical presentation of these four patients with papilledema and JAK2 mutation. CONCLUSIONS: A significant proportion of patients with papilledema secondary to DVST will harbor mutations in the JAK2 gene. Clinicians should be aware of this mutation as early testing will facilitate timely diagnosis and treatment of myeloproliferative disease to improve prognosis and reduce risk of recurrent thrombotic events.
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Trastornos Mieloproliferativos , Papiledema , Policitemia Vera , Trombosis de los Senos Intracraneales , Humanos , Estudios Retrospectivos , Papiledema/genética , Janus Quinasa 2/genética , Policitemia Vera/diagnóstico , Policitemia Vera/tratamiento farmacológico , Policitemia Vera/genética , Trastornos Mieloproliferativos/complicaciones , Trastornos Mieloproliferativos/genética , Mutación/genética , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/genética , Trombosis de los Senos Intracraneales/complicacionesRESUMEN
SIGNIFICANCE: This case highlights ocular adverse effects of a rare, potentially life-threatening complication from coronavirus disease 2019 (COVID-19). Papilledema can occur because of increased intracranial pressure caused by cerebral venous sinus thrombosis, the incidence of which may be more likely in patients with a history of COVID-19 because of an induced hypercoagulable state. PURPOSE: This case report presents a case of papilledema secondary to cerebral venous sinus thrombosis in a patient with a recent history of severe coronavirus disease (COVID-19). CASE REPORT: A 29-year-old man hospitalized with a complicated course of coronavirus disease (COVID-19) was referred to the ophthalmology department for episodic blurry vision of both eyes and intermittent binocular diplopia. Clinical examination revealed diffuse bilateral optic disc edema. Magnetic resonance venography of the brain during his admission revealed subtotal occlusion of the right transverse sinus by thrombosis. At the time of diagnosis, the patient was already taking systemic anticoagulation therapy for treatment of a recent pulmonary embolism also thought to be induced by COVID-19. After additional treatment with acetazolamide, there was improvement in his optic nerve edema. CONCLUSIONS: Cerebral venous sinus thrombosis, a serious and potentially life-threatening condition, can occur as a rare complication of COVID-19. In such cases, patients may develop increased intracranial pressure, papilledema, and subsequent vision loss. Magnetic resonance venography should be ordered in patients with suspected papilledema to help rule out the presence of cerebral venous sinus thrombosis.
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COVID-19 , Papiledema , Trombosis de los Senos Intracraneales , Masculino , Humanos , Adulto , Papiledema/diagnóstico , Papiledema/etiología , Papiledema/tratamiento farmacológico , Trombosis de los Senos Intracraneales/etiología , Trombosis de los Senos Intracraneales/complicaciones , COVID-19/complicaciones , Imagen por Resonancia Magnética , Trastornos de la Visión/etiología , DiplopíaRESUMEN
BACKGROUND: In March 2021, several European countries suspended the use of the AZD1222 (Oxford-AstraZeneca) COVID-19 vaccine because of thromboembolic safety concerns. Reports from Norway and Germany subsequently described patients with venous thrombosis and thrombocytopenia within 5 to 16 days of vaccination. OBJECTIVE: To evaluate the risk for outcomes related to thrombosis and thrombocytopenia after AZD1222 or BNT162b2 (Pfizer-BioNTech) COVID-19 vaccination. DESIGN: Nationwide exploratory retrospective cohort study. SETTING: Danish linkable registers on vaccinations, hospitalizations, occupation, and other covariates. PARTICIPANTS: 355 209 Danish frontline personnel designated for priority COVID-19 vaccination followed from 27 December 2020 (the day of the first COVID-19 vaccination in Denmark) to 13 April 2021. MEASUREMENTS: Study outcomes were cerebral venous sinus thrombosis, splanchnic vein thrombosis, pulmonary embolism, deep venous thrombosis, arterial thrombosis, thrombocytopenia, and death. Cumulative incidences of study outcomes within 28 days of vaccination and unvaccinated risk time were compared using adjusted survival curves resulting in risk differences (RDs) at day 28 after vaccination. Adjustment for birth cohort, sex, calendar period, occupation, comorbid conditions, and prescription drug use was included. RESULTS: Vaccination with AZD1222 versus no vaccination was associated with a significant RD at day 28 for deep venous thrombosis (RD, 8.35 [95% CI, 0.21 to 16.49] per 100 000 vaccinations). The RDs for cerebral venous sinus thrombosis (RD, 1.68 [CI, -0.64 to 4.00] per 100 000 vaccinations) and thrombocytopenia (RD, 2.39 [CI, -1.09 to 5.87] per 100 000 vaccinations) were not significant. No adverse associations were seen for BNT162b2 vaccination. LIMITATION: No medical record review; surveillance bias. CONCLUSION: In this exploratory retrospective cohort study among frontline personnel in Denmark, receipt of the AZD1222 vaccine was associated with a small excess risk for deep venous thrombosis. Although the corresponding risks for the more rare and severe thrombotic outcomes (such as cerebral venous sinus thrombosis) were not statistically significantly increased, statistical precision was low, and clinically relevant risks could not be excluded with certainty. There was no statistically significant association of BNT162b2 vaccination with thrombotic or thrombocytopenic events. PRIMARY FUNDING SOURCE: Lundbeck Foundation.
Asunto(s)
COVID-19 , Trombosis de los Senos Intracraneales , Trombocitopenia , Tromboembolia , Trombosis , Trombosis de la Vena , Vacuna BNT162 , COVID-19/epidemiología , COVID-19/prevención & control , Vacunas contra la COVID-19/efectos adversos , ChAdOx1 nCoV-19 , Humanos , Ocupaciones , Estudios Retrospectivos , SARS-CoV-2 , Trombosis de los Senos Intracraneales/complicaciones , Trombocitopenia/inducido químicamente , Trombocitopenia/epidemiología , Tromboembolia/epidemiología , Tromboembolia/etiología , Trombosis/etiología , Vacunación/efectos adversos , Trombosis de la Vena/complicacionesRESUMEN
BACKGROUND: Cerebral sinus venous thrombosis (CSVT) is an uncommon condition in children with potentially serious outcomes. Large epidemiological studies in children with CSVT are few. The objective of this study is to evaluate the epidemiology and in-hospital outcomes of hospitalized children with CSVT in the United States. METHODS: We performed a retrospective cross-sectional analysis of the Healthcare Cost and Utilization Project Kids' Inpatient Database for the combined years 2016 and 2019. The database was queried using the diagnoses for intracranial and intraspinal phlebitis and thrombophlebitis, nonpyogenic thrombosis of the intracranial venous system, and cerebral infarction due to cerebral venous thrombosis. Sample weighting was employed to produce national estimates. RESULTS: Of 12,165,621 discharges, 3202 had CSVT (in-hospital prevalence 26.3 per 100,000 discharges). Male patients accounted for 57% of CSVT discharges. The median age was 8 years (interquartile range 1-16), with a U-shaped distribution with peaks in patients younger than 4 years and patients aged between 18 and 20 years. A total of 19.3% of children with CSVT had either hemorrhagic or ischemic stroke. Patients with stroke were more likely to require mechanical ventilation (odds ratio [OR] 2.7; 95% confidence interval [CI] 2.1-3.3; p < 0.001) and have higher mortality (OR 2.3; 95% CI 1.6-3.4; p < 0.001). Mechanical ventilation was necessary for 25.2% of patients with CSVT, of whom the majority were neonates and young children. The need for mechanical ventilation was associated with increased mortality (OR 16.6; 95% CI 9.9-27.9; p < 0.001). The overall mortality rate for CSVT was 4.1%, and 16.5% of patients with CSVT were discharged with home health care or to a skilled nursing facility. CONCLUSIONS: CSVT, which has a U-shaped age distribution, is an uncommon condition in children. Stroke is common in children with CSVT, and it is associated with an increased need for mechanical ventilation and increased mortality. The need for mechanical ventilation is more common in infants, and it is associated with increased mortality across all age groups.
Asunto(s)
Trombosis de los Senos Intracraneales , Accidente Cerebrovascular , Trombosis de la Vena , Lactante , Recién Nacido , Humanos , Masculino , Niño , Preescolar , Adolescente , Adulto Joven , Adulto , Factores de Riesgo , Estudios Retrospectivos , Trombosis de los Senos Intracraneales/epidemiología , Trombosis de los Senos Intracraneales/terapia , Trombosis de los Senos Intracraneales/complicaciones , Estudios Transversales , Accidente Cerebrovascular/complicaciones , Trombosis de la Vena/epidemiología , Trombosis de la Vena/terapiaRESUMEN
INTRODUCTION: Raised intracranial pressure (ICP) can be due to varied etiology. Differentiating among these various etiologies is crucial in making appropriate therapeutic decisions. A patient with a known past history of the primary or secondary headache of any etiology, when presenting with new onset severe headache, needs to be evaluated with imaging to rule out an alternative diagnosis. DISCUSSION: Here, we describe the case details of a young lady who presented with recurrent raised ICP headaches due to three different etiologies. At her third visit, isolated intracranial hypertension (IH) was the only manifestation of cerebral venous sinus thrombosis (CVST), which could have been missed if a repeat magnetic resonance imaging (MRI) brain and venogram were not done. CONCLUSION: Our case highlights the importance of having a high degree of suspicion for CVST in the clinical setting of raised ICP headache in view of its crucial therapeutic implications.
Asunto(s)
Cefalea , Hipertensión Intracraneal , Trombosis de los Senos Intracraneales , Femenino , Humanos , Cefalea/etiología , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/etiología , Presión Intracraneal/fisiología , Imagen por Resonancia Magnética , Recurrencia , Trombosis de los Senos Intracraneales/diagnóstico , Trombosis de los Senos Intracraneales/complicacionesRESUMEN
BACKGROUND: Current methods to evaluate the severity of cerebral venous sinus thrombosis (CVST) lack patient-specific indexes. Herein, a novel scoring method was investigated to estimate the thrombus burden and the intracranial pressure (ICP) of CVST. METHODS: In this retrospective study from January 2019 through December 2021, we consecutively enrolled patients with a first-time confirmed diagnosis of CVST by contrast-enhanced magnetic resonance venography (CE-MRV) or computed tomography venography (CTV). In these patients, a comprehensive CVST-Score was established using magnetic resonance black-blood thrombus imaging (MRBTI) to estimate the thrombus burden semi-quantitatively. The relationship between CVST-Score and ICP was explored to assess the potential of using the CVST-score to evaluate ICP noninvasively and dynamically. RESULTS: A total of 87 patients were included in the final analysis. The CVST-Scores in different ICP subgroups were as follows: 4.29±2.87 in ICP<250mmH2O subgroup, 11.36±3.86 in ICP =250-330mmH2O subgroup and 14.99±3.15 in ICP>330mmH2O subgroup, respectively (p<0.001). For patients with ICP ≤330mmH2O, the CVST-Score was linearly and positively correlated with ICP (R2=0.53). The receiver operating characteristic (ROC) curves showed the optimal CVST-Score cut-off values to predict ICP ≥250mmH2O and >330mmH2O were 7.15 and 11.62, respectively (P<0.001). Multivariate analysis indicated CVST-Score as an independent predictor of ICP ≥250mmH2O (odds ratio, 2.15; 95% confidence interval, 1.49-3.10; p<0.001). CONCLUSIONS: A simple and noninvasive CVST-Score can rapidly estimate the thrombus burden and predict the severity of intracranial hypertension in patients with CVST. The CVST-Score can aid in evaluating therapeutic responses and avoiding unnecessary invasive procedures at long-term follow-up.
Asunto(s)
Hipertensión Intracraneal , Trombosis de los Senos Intracraneales , Trombosis , Humanos , Estudios Retrospectivos , Trombosis de los Senos Intracraneales/complicaciones , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Imagen por Resonancia Magnética , Hipertensión Intracraneal/complicaciones , Hipertensión Intracraneal/diagnóstico por imagenRESUMEN
Cerebral sinus thrombosis (CST) is an uncommon condition in children with a variable clinical presentation which has rarely been described in the setting of diabetic ketoacidosis. We present the case of 14-year-old child in whom lateral sinus thrombosis was caused by dehydration complicating ketoacidosis in a previously undiagnosed type 1 diabetes. The diagnosis of the CST was established during the autopsy due to the rapidity of the neurological deterioration. The cause of death was tonsillar herniation due to diffuse cerebral edema secondary to CST. This is the first published report of a CST in association with new onset type 1 diabetes in a child diagnosed at the postmortem examination.