Kikuchi-Fujimoto disease evolves into lupus encephalopathy characterized by venous sinus thrombosis: a case report.

Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting illness that can progress to systemic lupus erythematosus (SLE) in approximately 30% of cases. Neurological injuries can occur in both diseases, albeit with distinct presentations. Venous sinus thrombosis is a serious cerebrovascular complication in patients with neuropsychiatric SLE but is rarely observed in patients with KFD. The involvement of various antibodies, particularly antiphospholipid antibodies, can cause vascular endothelial cell injury, resulting in focal cerebral ischemia and intracranial vascular embolism in SLE. However, there are cases in which thrombotic pathology occurs without antiphospholipid antibody positivity, attributed to vascular lesions. In this report, we present a case of KFD and lupus encephalopathy featuring cerebral venous sinus thrombosis, despite the patient being negative for antiphospholipid antibody. We also conducted a comparative analysis of C3 and C4 levels in cerebrospinal fluid (CSF) and peripheral blood, along with the protein ratio in CSF and serum, to elucidate the pathological changes and characteristics of lupus encephalopathy.

[The clinical characteristics of neutrophil extracellular trap in patients with severe cerebral venous sinus thrombosis and its prognostic value in acute and subacute patients].

Objective: To explore the clinical characteristics of neutrophil extracellular trap (NET) in patients with severe cerebral venous sinus thrombosis (CVST) and to study their prognostic value in the acute and subacute phases. Methods: This study is a retrospective case series analysis. Clinical and pathological data of 52 patients with severe cerebral venous sinus thrombosis who underwent endovascular treatment in the Department of Neurosurgery, Tianjin Huanhu Hospital from June 2019 to June 2022 were retrospectively analyzed. There were 20 males and 32 females, with an age of (40.1±13.6) years(range:18 to 66 years). Forty-five healthy physical examinees were included in the control group. High-resolution MRI was used to stage the thrombus, with 11 cases in the acute group, 28 cases in the subacute group, and 13 cases in the chronic group. Thrombus specimens were obtained through endovascular treatment, and the fluorescence intensity of NET in peripheral blood at different time points was analyzed by immunofluorescence contrast,including the double-stranded DNA structure and adhesion protein components (citrolinated histone H3 (CitH3), myeloperoxidase-DNA complex(MPO-DNA), neutrophil elastase (NE)). The NET markers were determined by ELISA. Spearman rank correlation analysis was used to analyze the correlation between the NET markers in peripheral blood of patients with severe cerebral venous sinus thrombosis in the acute and subacute phases and the volume of venous sinus thrombus, the degree of venous sinus recanalization after treatment, and the discharge modified Rankin scale(mRS)score. The accuracy of NET markers in predicting the prognosis of patients with severe cerebral venous sinus thrombosis was analyzed by drawing receiver operating characteristic (ROC) curve and calculating the area under the curve (AUC). Results: The results of immunofluorescence staining and ELISA showed that no NET structure was formed in the peripheral blood of the control group, while CitH3, MPO-DNA and NE levels in the peripheral blood of CVST patients were increased, among which the acute stage group was the highest, followed by the subacute group, and the chronic group was the lowest. Spearman correlation analysis showed that CitH3, MPO-DNA and NE levels in peripheral blood of patients in acute group and subacute group were positively correlated with thrombus volume and mRS score at discharge (P<0.05). The levels of CitH3 and MPO-DNA in peripheral blood of patients with complete venous sinus recanalization were lower than those of patients with partial venous sinus recanalization (P<0.01). ROC curve analysis results showed that MPO-DNA and NE had no predictive ability for the prognosis of CVST patients (P values were 0.614 and 0.324, respectively), and the AUC of CitH3 was 0.800 (95%CI: 0.638~0.962, P=0.032), the best cut-off value was 13.5 µg/L, the sensitivity was 100%, and the specificity was 58.8%. Conclusions: A large number of NET are formed in patients with severe cerebral venous sinus thrombosis in acute stage. Patients with severe cerebral venous sinus thrombosis in acute stage and subacute stage with high peripheral blood NET content has a low rate of complete sinus revascularization and poor neurological function recovery after treatment.

Full sample analysis of cerebral venous sinus thrombosis in ophthalmology inpatients.

PURPOSE: We hope that by analyzing the clinical features of cerebral venous sinus thrombosis (CVST), we can help ophthalmologists reduce misdiagnosis or delayed diagnosis. DESIGN: We evaluated 47 patients with CVST in terms of clinical manifestations. METHODS: All cases were analyzed in terms of risk factors, clinical symptoms, ophthalmic examination, imaging examination and lumbar puncture. RESULTS: The body mass indices (BMIs) of 41 patients (87.2%; 95% CI, 77.7-96.8%) were≥24, which is overweight by Chinese standards. There were 22 patients (46.8%; 95% CI, 32.5-61.1%) with BMIs≥28, who were considered obese. Thirteen were hypertensive (27.7%; 95% CI, 14.9-40.5%). The initial symptoms included blurred vision (23, 48.9%; 95% CI, 34.6-63.2%), amaurosis fugax (13, 27.7%; 95% CI, 14.9-40.5%), headache (11 patients, 23.4%; 95% CI, 11.3-35.5%), dizziness (3, 6.4%; 95% CI, -0.6-13.4%), and bilateral diplopia (3, 6.4%; 95% CI, -0.6-13.4%). There were 9 patients (9, 19.2%; 95% CI, 7.9-30.4%) with blindness, 23 patients (48.9%; 95% CI, 34.6-63.2%) with pupillary abnormalities, and 40 patients (85.1%; 95% CI, 74.9-95.2%) with papilledema. Forty-three of the 45 patients who successfully underwent a routine lumbar puncture showed high intracranial pressure (91.7%; 95.6% CI, 89.6-101.6%). Finally, two cases are reported in greater detail for illustrative purposes. CONCLUSION: The main reasons interfering with the diagnosis of CVST might be its nonspecific ocular symptoms and the physicians' clinical thought process being limited to the scope of common ophthalmological diseases.

A Rare Intersection of Septic Cavernous Sinus Thrombosis and Subarachnoid Hemorrhage: Insights from the Case of a 70-Year-Old Patient.

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.

Derivation of a clinical decision rule to exclude cerebral venous sinus thrombosis in emergency department patients: A retrospective cohort study.

OBJECTIVE: To derive a clinical decision rule to exclude cerebral venous sinus thrombosis (CVST) in the ED. A secondary aim was to derive a rule that incorporated clinical parameters and the non-contrast CT brain. METHODS: Single-centre, retrospective cohort study. Patients suspected of CVST were identified from the radiology database for CT/MR venograms. Clinical features included in the rule were determined by literature review. The presence of these features in participants was determined by chart review. Variables were tested for univariate association with CVST using logistic regression. Variable selection was accomplished using a forward-stepwise process, calculating the sensitivity/specificity of a rule containing the variable of most significance, then repeating the process after adding the next most significant variable. RESULTS: Forty-five out of 912 participants had confirmed CVST. The primary clinical rule was answering 'no' to all the following: any prothrombotic risk factor, age ≥54 years, confusion: sensitivity 95.6% (95% confidence interval [CI] 84.9-99.5%), specificity 40.9% (95% CI 37.6-44.2%), negative predictive value 99.4% (95% CI 97.9-99.9%) and positive predictive value 7.7% (95% CI 7.1-8.3%). The rule classified 39.5% of participants as CVST ruled out. The rule incorporating the non-contrast CT brain was answering 'no' to all the following: abnormal non-contrast CT brain, any prothrombotic risk-factor, age ≥54 years, confusion: sensitivity 100.0% (95% CI 91.6-100.0%), specificity 42.0% (95% CI 38.7-45.4%), negative predictive value 100.0% (95% CI not calculated) and positive predictive value 7.8% (95% CI 7.4-8.2%). The rule classified 40.0% of participants as CVST ruled out. CONCLUSIONS: A clinical decision rule was derived to rule out CVST. These results require validation before adoption into clinical practice.

[Multifocal cerebral venous thrombosis]. / Cas clinique. Thrombose veineuse cérébrale multifocale.

Cerebral venous thrombosis is a rare condition and represents a neurological emergency. It is a particular subtype of stroke, characterized by a huge diversity of neurological symptoms. Due to the diversity of its potential clinical presentations, medical imaging plays an important role in its early detection, even on non-dedicated examinations often performed in search of another acute neurological pathology. The aim of this case report is to illustrate the different radiological signs of cerebral venous thrombosis and to discuss the difficulties in diagnosing it by imaging at the acute stage.

La thrombose veineuse cérébrale (TVC) est une pathologie rare et constitue une urgence neurologique. Il s'agit d'un sous-type d'accident vasculaire cérébral (AVC) particulier, aux manifestations symptomatiques neurologiques très variées. De par la diversité de ses potentielles présentations cliniques, l'imagerie médicale joue un rôle important dans sa détection précoce et ce, y compris sur des examens non dédiés, réalisés à la recherche d'une autre pathologie neurologique aiguë. L'objectif de ce cas clinique est d'illustrer les différents signes radiologiques de la TVC et d'insister sur les difficultés, au stade aigu, de poser son diagnostic par imagerie.

Multi-inflammatory index as a new predictive and prognostic marker of acute symptomatic seizures in patients with cerebral venous sinus thrombosis.

BACKGROUND: The multi-inflammatory index (MII) is a novel marker that indicates the diagnosis, prognosis, and clinical severeness of inflammation-associated diseases. It is indicated that inflammatory biomarkers may help predict the occurrence of acute symptomatic seizures(ASS) after ischemic stroke. OBJECTIVE: Our goal was to search the predictive and prognostic capability of the MII-1 and MII-2 for ASS in cerebral venous sinus thrombosis (CVST) patients. METHOD: 91 patients with CVST were retrospectively analyzed from the medical records of patients between 2010 and 2023 and divided into the ASS group and non-ASS group. The MII-1 and MII-2 levels on admission, demographic and clinic features, predisposing risk factors, radiological characteristics involving thrombotic cerebral veins, and the type of parenchymal lesions were documented. Modified Rankin Scale (mRS) on admission and at the 3rd month were evaluated to determine the disability. RESULTS: MII-1 and MII-2 were statistically significantly higher in the ASS group and predictors for the occurrence of seizures with CVST in multivariate analysis. The area under the curve (AUC) of the receiver operating characteristics (ROC) curve for MII-1 was 0.791 (95 %CI = 0.691-0.891, p < 0.001), and AUC for MII-2 was 0.761 (95%CI = 0.660-0.861, p < 0.001). When the clinical variables that were included in the multivariate analysis and MII-1 and MII-2 were combined, the predictive power was greater with the AUC of 0.959. A significant positive correlation was found between mRS at the 3rd month and MII-1 and MII-2 in the ASS group. CONCLUSION: MII-1 and MII-2 can be used as new predictive and prognostic markers of ASS in patients with CVST.

Profile and Outcome of Prospective Non-Septic Lateral Sinus Thrombosis Patients from a Stroke Unit of a Developing Country.

Background: Due to effective treatment of middle ear infections there is a change in etiologies causing lateral sinus thrombosis (LST) and outcome. There is a paucity of literature describing homogenous group of patients with nonseptic LST (NS-LST). Objective: To describe the clinical profile, risk factors, outcome of patients of NS-LST seen at a single center from South India. Methods and Materials: Prospective, observational study of 100 patients of NS-LST patients, diagnosed by magnetic resonance imaging (MRI) seen at the stroke unit. Results: During 2 years, 100 patients of NS-LST (isolated: combined: 27:73) (male: female: 44:56), mean age: 31.45 ± 11.13 years, were seen. Subacute presentation (74%) with headache, seizures, focal deficits, and features of raised intracranial pressure were presenting features. Hyperhomocysteinemia (61%), anemia (57%), postpartum state (41%), OCP use (37%), and low VitB12 (32%) were commonly seen risk factors. Imaging with MRI compared withcomputerized tomography (CT) had better diagnostic sensitivity (100% vs. 67%), detection of parenchymal (81% vs. 67%)/hemorrhagic (79% vs. 74%) lesions, and cortical vein thrombosis (31% vs. 15.46%). Treatment with anticoagulation and supportive therapy resulted in good outcome (mRS (0-2)) at 3 months in 81%.There were four deaths, all during admission (one - isolated, three - combined) and 11 patients underwent decompressive surgery. Patients with low GCS level of sensoriumat admission, hemiparesis, combined LST, cerebellar involvement, and decompressive craniectomy had a poor outcome. Conclusion: This single-center large cohort study of NS-LST patients brings out the clinical features, risk factors (peculiar to developing countries), and the superiority of MRI in the diagnosis. Majority of patients have good outcome, with low mortality with 10% requiring decompressive surgery.

Clinical Values of Coagulation Factors X, XI, and XII in Cerebral Venous Sinus Thrombosis During Perinatal State.

Cerebral venous sinus thrombosis (CVST) has become a rare but potentially life-threatening condition in perinatal women. Early and rapid identification of CVST in pregnant women is a challenge for frontline clinical workers. In this study, 40 perinatal patients with CVST in our hospital were included in the five-year period, and 120 normal perinatal pregnant women in the obstetrics and gynecology department of our hospital were randomly enrolled in the five-year period as the control group, including 60 cases in pregnancy and puerperium. 5 mL of fasting venous blood was collected from puerperal CVST patients in the acute phase of onset (within 72 h of onset) and the recovery phase (fourth week of treatment). In the control group, 5 mL of fasting venous blood was collected. Coagulation factors X, XI, and XII, plasma D-Dimer were analyzed and compared. Coagulation factors X, XI, and XII in plasma of CVST patients were significantly increased compared with controls. Plasma coagulation factors X, XI, and XII and their combined detection (Union Model = 0.056 * FX: C + 0.046 * FXI: C + 0.081 * FXII: C) have diagnostic values for perinatal CVST. Plasma coagulation factors X, XI, and XII were significantly positively correlated with plasma D-dimer levels in perinatal CVST patients. Plasma coagulation factors X, XI, and XII have diagnostic values for perinatal CVST.

Dynamic Evolution of Catatonia in the Context of Cerebral Venous Sinus Thrombosis.

The Psychiatric Consultation Service at Massachusetts General Hospital sees medical and surgical inpatients with comorbid psychiatric symptoms and conditions. During their twice-weekly rounds, Dr Stern and other members of the Consultation Service discuss diagnosis and management of hospitalized patients with complex medical or surgical problems who also demonstrate psychiatric symptoms or conditions. These discussions have given rise to rounds reports that will prove useful for clinicians practicing at the interface of medicine and psychiatry.Prim Care Companion CNS Disord 2023;25(4):22f03471. Author affiliations are listed at the end of this article.

Post-operative segmental cerebral venous sinus thrombosis: risk factors, clinical implications, and therapeutic considerations.

Cerebral venous sinus thromboses (CVSTs) are rare complications of neurosurgical interventions and their management remains controversial as most of cases appear clinically silent. Here, we analyzed our institutional series of patients with CVSTs evaluating clinical and neuroradiological characteristics, risk factors, and outcome. From the analysis of our institutional PACS, we collected a total of 59 patients showing postoperative CVSTs after supratentorial or infratentorial craniotomies. For every patient, we collected demographics and relevant clinical and laboratory data. Details on thrombosis trend were retrieved and compared along the serial radiological assessment. A supratentorial craniotomy was performed in 57.6% of cases, an infratentorial in 37.3%, while the remaining were a single cases of trans-sphenoidal and neck surgery (1.7%, respectively). A sinus infiltration was present in almost a quarter of patients, and in 52.5% of cases the thrombosed sinus was exposed during the craniotomy. Radiological signs of CVST were evident in 32.2% of patients, but only 8.5% of them developed a hemorrhagic infarct. CVST-related symptoms were complained by 13 patients (22%), but these were minor symptoms in about 90%, and only 10% experienced hemiparesis or impaired consciousness. The majority of patients (78%) remained completely asymptomatic along the follow-up. Risk factors for symptoms occurrence were interruption of preoperative anticoagulants, infratentorial sinuses involvement and evidence of vasogenic edema and venous infarction. Overall, a good outcome defined mRS 0-2 was observed in about 88% of patients at follow-up. CVST is a complication of surgical approaches in proximity of dural venous sinuses. CVST usually does not show progression and courses uneventfully in the vast majority of cases. The systematic use of post-operative anticoagulants seems to not significantly influence its clinical and radiological outcome.

A case of suspected canine multifocal cervical venous sinus thrombosis causing cervical myelopathy.

A 10-year-old spayed female standard poodle was admitted for marked cervical pain. Magnetic resonance imaging (MRI) suggested cervical venous sinus thrombi. An excellent clinical response to clopidogrel and warfarin combination was achieved. Repeat MRI showed partial resolution of the thrombosis. The etiology of the thrombosis is suspected to be due to the previous long-term, chronic use of corticosteroids. To the authors' knowledge, this is the first case report with follow-up imaging of canine venous sinus thrombosis. This clinical case report may help practitioners recognize this condition as a possible differential diagnosis for cervical pain, and it provides important guidelines in the prognosis. This case report highlights the use of anticoagulant therapy for 14 wk leading to complete resolution of clinical signs and to partial resolution of the thrombus 5 wk into the treatment, based on comparative MRI. Key clinical message: To the authors' knowledge, this is the first report of suspected multiple cervical venous sinus thrombi that includes comparative imaging into the course of therapy. The aim of this report is to provide additional information for future guidance on the duration of venous sinus thrombosis therapy.

Un cas de thrombose multifocale du sinus veineux cervical causant une myélopathie cervicale. Une femelle caniche standard stérilisée âgée de 10 ans a été présentée pour une douleur aigüe marquée au cou. L'imagerie par résonance magnétique (IRM) était évocatrice de thrombose du sinus veineux cervical. Une excellente réponse au traitement combinant Warfarine et Clopidogrel a été notée. L'étiologie suspectée d'être à l'origine de la thrombose est l'administration prolongée de corticostéroïdes. Jusqu'à maintenant, il s'agit du premier rapport de cas avec imagerie de suivi de thrombose du sinus veineux cervical. Il pourrait aider à reconnaître cette condition comme l'un des diagnostics différentiels lors de douleur cervicale et il fournit des indications importantes reliées au pronostic. Ce rapport met en évidence l'utilisation d'un anticoagulant pendant quatorze semaines conduisant à la disparition complète des signes cliniques reliés à la thrombose et à une résolution partielle de la thrombose notée sur l'IRM comparative 5 semaines suivant l'introduction du traitement.Message clinique clé :À la connaissance des auteurs, il s'agit du premier rapport de cas de multiples thromboses du sinus veineux cervical suspectées incluant une imagerie comparative au cours du traitement. L'objectif de ce rapport est de fournir de l'information supplémentaire à la formation de lignes directrices futures sur la durée de traitement de la thrombose veineuse cervicale.(Traduit par les auteurs).

Utility of 3D T1-weighted turbo spin echo black blood sequence for the diagnosis of cerebral venous thrombosis.

PURPOSE: Accurate assessment of dural sinus, deep and cortical venous thrombosis on MR imaging is challenging. The aim of this study is to evaluate the accuracy of 3D-T1 turbo spin echo (T1S), sequences in detecting venous thrombosis and comparing it with susceptibility-weighted imaging (SWI), magnetic resonance venography (MRV) and post contrast T1 magnetization-prepared rapid acquisition gradient echo (T1C). METHODS: A blinded retrospective observational analysis of 71 consecutive patients evaluated for cerebral venous thrombosis (CVT) and 30 control patients was performed. Multimodality reference standard adopted included T1C, SWI with MRV. Sub-analyses in superficial, deep and cortical venous segments were performed in addition to correlation of signal intensity of thrombus with the clinical stage. RESULTS: A total of 2222 segments in 101 complete MRI examinations were evaluated. Sensitivity/specificity/positive predictive value/negative predictive value/accuracy and precision of T1S for detection of cortical vein thrombosis was 0.994/1/1/0.967/0.995/1, 1/0.874/0.949/1/0.963/0.950 for detection of superficial venous sinus thrombosis and 1/1/1/1/1/1 for deep venous thrombosis. The AUC yield for T1S was 0.997 for cortical, 1 for deep and 0.988 for superficial venous segments. CONCLUSION: T1S paralleled the accuracy of conventional sequences in the overall detection of CVT but showed superior accuracy in the detection of cortical venous thrombosis. It makes a fitting addition to the CVT MRI protocol in scenarios demanding negation of gadolinium administration.

Fatal cerebral sinus thrombosis associated with diabetic ketoacidosis in a child.

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.

The Novel Biomarkers-Based HALP (Hemoglobin, Albumin, Lymphocyte and Platelet)-Prognostic Model for Acute and Subacute Patients with Cerebral Venous Sinus Thrombosis: A Retrospective Cohort Study.

AIM: Increasing evidences suggest that HALP is an independent predictor of prognosis in patients with inflammation. However, the relationship between HALP and prognosis in patients with cerebral venous sinus thrombosis (CVST) has not been studied. In this study, we aimed to evaluate the prognosis values of HALP in acute or subacute CVST and explore the new prognostic model for CVST. METHODS: Consecutive patients who were diagnosed as having acute and subacute CVST were retrospectively investigated. We determined the patients' functional outcomes by modified Rankin Scale (mRS). Multivariate logistic regression analysis was used to assess the relationship between factors and poor functional outcomes. The area under the ROC curve (AUC) was estimated to evaluate the ability of markers and models in predicting clinical prognosis. The prognostic model was presented as nomogram. In addition, the decision curve analysis (DCA) was used to analyze the benefit of this model. Furthermore, survival curves were described by the Kaplan-Meier analysis. RESULTS: A total of 270 patients were included of which 31 had poor outcome. Multivariable logistic regression analysis demonstrated HALP (OR=0.978, 95%CI: 0.958-0.999, P=0.039) was a protective predictor of outcome. The AUC of HALP was 0.749 (95% CI: 0.633-0.865, P=0.044). DCA demonstrated that this model significantly improved risk prediction at threshold probabilities of CVST at 0 to 85% compared to ISCVT-RS scores. Patients with higher HALP (P=0.006) presented higher overall survival rates. CONCLUSION: HALP may be a potential protective marker in acute and subacute CVST patients. The new prognostic model with HALP had potentially better value for acute and subacute CVST patients.

Newborn with a Sudden Increase in Head Circumference, Wide Sutures, and Open Fontanels.

We describe an uncommon presentation of a rare disease in a 15-day-old term infant with a sudden increase in head circumference, wide sutures, and open fontanels. Magnetic resonance imaging/magnetic resonance venography showed cerebral venous sinus thrombosis (CVST). The infant had multiple maternal-fetal risk factors for CSVT, with screening coagulation studies within normal limits. Unfractionated heparin was the treatment of choice with a suitable response and no further thrombosis or hemorrhages. Neurologic examination findings on discharge and at 2 months were within normal limits.