BACKGROUND: We present a rare case of NeuroBehcet\'s-related intracranial hypertension without cerebral venous thrombosis (NBrIHwCVT), occurring as the first presentation of NeuroBehcet\'s. In addition, we describe the novel use of subcutaneous tocilizumab for this indication. This is followed by a review of the literature on this topic.
METHODS: The patient was a 28-year-old lady of Southern Chinese origin with a known history of Behcet\'s disease with oral ulcers and ocular findings for which she was on mycophenolate mofetil and adalimumab. She presented with a headache and bilateral disc swelling associated with an intracranial pressure (ICP) of > 40cmH20. There were no structural lesions or cerebral venous thrombosis (CVT) on imaging. Initial lumbar puncture had raised leucocytes and protein. We discuss diagnostic challenges given persistently elevated ICP despite subsequent non-inflammatory cerebrospinal fluid (CSF) profiles and non-response to acetazolamide. She eventually showed a response to immunosuppressant therapy in the form of pulsed methylprednisolone, cyclophosphamide and subsequently subcutaneous tocilizumab, supporting the diagnosis of NBrIHwCVT. Complete normalization of ICP remains challenging. Her disease course was severe, unusual for her ethnicity.
METHODS: We identified 34 patients (including ours) from 14 publications. We found that the majority of NBrIHwCVT patients were young (average age of 34 years), with a slight female preponderance. Of the 17 cases in the literature with available data on CSF profile, none had raised leucocytes whilst one patient had elevated protein. Patients were generally treated with steroids and occasionally azathioprine, in line with the suspected autoimmune pathophysiology. Of 22 patients with data on outcome, six (27%) were noted to have recurrence of symptoms generally occurring a few months later.
CONCLUSIONS: As demonstrated by this case, NBrIHwCVT can present with BD with raised ICP even if there is no prior history of NB, central Asian ethnicity, cerebral venous thrombosis or features of inflammation on the CSF. We demonstrated how novel use of Tocilizumab may have a role in the management of NBrIHwCVT. Based on our literature review, patients were more likely to be young, female, display a non-inflammatory CSF picture, be treated with steroids and harbour a possibility of recurrence.

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UNASSIGNED: Dural arteriovenous fistulas (DAVFs) involving superior petrosal sinus (SPS) and superior petrosal vein (SPV) are extremely rare. The pathogenesis of these fistulas remains unclear. We are illustrating 2 cases of DAVFs involving the superior petrosal sinus and veins associated with venous sinus thrombosis with a literature review.
UNASSIGNED: We reviewed the literature using the PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines focusing on DAVFs involving the SPS and/or SPV. Additionally, we searched for additional articles through the reference lists of the included studies.
UNASSIGNED: Our review yielded 20 articles from 1997 until 2022 involving 33 patients with 34 fistulas, including our 2 patients. The mean age was 55.1 ± 12.9 years (range 25-85), 54.5% were males (n = 18). The patients presented with hemorrhage in 36.4% (n = 12), and progressive myelopathy in 30.3% (n = 10). Most fistulas often had arterial supply from MMA, MHT, and/or OA. The fistulas had infratentorial drainage in 64.71% (n = 22), supratentorial drainage in 23.53% (n = 8), and both supra and infratentorial drainage in 11.76% (n = 4). In 27.3% (n = 9), cerebral venous thrombosis was mentioned or identified. Endovascular treatment was performed in 47.1% of cases (n = 16), surgery in 29.4% (n = 10), and combination of treatments in 23.5% (n = 8). A total of 30.3% (n = 10) of cases had incomplete recovery or poor result.
UNASSIGNED: DAVFs involving the SPS and/or SPV are associated with aggressive natural history, requiring early diagnosis and prompt treatment, leading to good prognosis. These fistulas may be acquired in origin, probably secondary to cerebral venous thrombosis.

In managing cerebral venous sinus thrombosis (CVT), the standard approach has been administering parenteral anticoagulation for at least five days, despite limited supporting evidence. This study aimed to determine the optimal duration of parenteral anticoagulation for CVT patients and its potential impact on their functional outcomes upon discharge. This retrospective observational cohort study was conducted across multiple healthcare centers and included adult CVT patients who received varying durations of parenteral anticoagulation: less than 5 days (n = 25) or 5 days or more (n = 16). The primary focus was on the duration of acute anticoagulation treatment, with secondary endpoints including hospital stay length and functional outcomes. The study found that a shorter duration of anticoagulation treatment (< 5 days) was linked to more favorable outcomes, as measured by the modified Rankin Scale (mRS) (68% vs. 25%, RR = 0.37, CI 0.15-0.90, p = 0.007). However, regression analysis showed non statistically significant associations for all variables except gender. Female patients were significantly more likely to receive a shorter duration of anticoagulation (Odds Ratio: 2.6, 95% CI: 2.2-3.1, P-Value: <0.001). These findings suggest a potential connection between shorter anticoagulation duration (< 5 days) and improved CVT patient outcomes, as indicated by their mRS scores at discharge. The observed relationship between female gender and shorter anticoagulation duration warrants further exploration. Nevertheless, caution is necessary when interpreting these findings due to the small sample size and specific patient characteristics. Further research in a larger and more diverse cohort is essential to validate these results and understand their implications fully.

Traumatic brain injury (TBI) is a common diagnosis requiring acute hospitalization. Long-term, TBI is a significant source of health and socioeconomic impact in the United States and globally. The goal of clinicians who manage TBI is to prevent secondary brain injury. In this population, post-traumatic cerebral infarction (PTCI) acutely after TBI is an important but under-recognized complication that is associated with negative functional outcomes. In this comprehensive review, we describe the incidence and pathophysiology of PTCI. We then discuss the diagnostic and treatment approaches for the most common etiologies of isolated PTCI, including brain herniation syndromes, cervical artery dissection, venous thrombosis, and post-traumatic vasospasm. In addition to these mechanisms, hypercoagulability and microcirculatory failure can also exacerbate ischemia. We aim to highlight the importance of this condition and future clinical research needs with the goal of improving patient outcomes after TBI.

Several vaccine-induced thrombotic thrombocytopenia syndrome (VITTS) cases have been reported after the ChAdOx1 nCov-19 vaccination. The current study systematically reviewed the reported post-ChAdOx1 nCoV-19 vaccination thrombotic thrombocytopenia cases. Their laboratory and clinical features, as well as the diagnostic and therapeutic measures, were investigated. Online databases were searched until 25 August 2021. Studies reporting post-ChAdOx1 nCov-19 vaccination thrombotic thrombocytopenia syndrome (TTS) were included. Overall, 167 cases (21-77 years old) from 53 publications were included showing a female dominance of 1.75 times. About 85% of the cases exhibited the primary symptoms within the first two weeks post-vaccination. Headache was the most common initial symptom (>44.2%), and hemorrhage/thrombotic problems (22.46%), as well as discoordination/weakness/numbness/ hemiparesis/cyanotic toes (19.6%), were the most prevalent uncommon initial symptoms. Prothrombin time (PT), D-dimers, and C-reactive protein were the most remarkable increased laboratory parameters in 50.6%, 99.1%, and 55.6% of cases, respectively. In comparison, platelet and fibrinogen were the most remarkable decreased laboratory parameters in 92.7% and 50.5% of cases, respectively. Most VITT cases presented with cerebral venous thrombosis/cerebral venous sinus thrombosis, supraventricular tachycardia, transverse sinus/cerebral thrombosis, pulmonary embolism, and cerebral hemorrhage. Anti-PF4 antibody measurement through immunoassays and functional assays were positive in 86.2% and 73% of cases, respectively. About 31% of the cases died. Early diagnosis and proper therapeutic measures are important in ChAdOx1 nCov-19 vaccine-induced VITTS patients. Therefore, experts are recommended to know the corresponding clinical and laboratory features, as well as diagnostic methods. Elucidation of the pathophysiologic mechanism of ChAdOx1 nCov-19 vaccine-induced TTS deserves further investigation.

The evolution of the Coronavirus Disease-2019 pandemic and its vaccination raised more attention to cerebral venous thrombosis (CVT). Although CVT is less prevalent than arterial stroke, it results in larger years of life lost. CVT is more common in women and young patients. Predisposing factors are categorized as transient factors such as pregnancy, puerperium, oral contraceptive pills, trauma, and dehydration; and permanent factors such as neoplastic, vasculitic, thrombophilic, hematologic conditions, infectious causes such as severe acute respiratory syndrome coronavirus-2 infection and HIV. The most common manifestations are headache, seizures, focal neurologic deficits, altered level of consciousness, and cranial nerve palsies. The most common syndromes are stroke-like, raised-intracranial-pressure (ICP), isolated-headache, and encephalopathy, which may have overlaps. Diagnosis is mostly based on computed tomography, magnetic resonance imaging, and their respective venous sequences, supported by blood results abnormalities such as D-dimer elevation. Treatment includes the prevention of propagation of current thrombus with anticoagulation (heparin, or low molecular weight heparinoids and then warfarin, or direct oral anticoagulants), decreasing ICP (even by decompressive craniotomy), and treatment of specific underlying diseases.

Background: Systemic lupus erythematosus (SLE) is a common autoimmune disease with various symptoms involving multiple organs. Neuropsychological manifestations are various and generally serious. Leukoencephalopathy is particularly rare but life-threatening in patients with SLE. Results: Here, we describe the case of a young woman who developed a subacute onset intracranial hypertension, papillar edema on fundus examination, diffuse cerebral edema on brain CT scan, and diffuse leukoencephalopathy on brain magnetic resonance imaging (MRI). The immunological workup was positive for antinuclear antibodies, anti-DNA and anti-extractable nuclear antigens (ENA) antibodies. She was ultimately diagnosed with SLE and experienced significant improvement after treatment with high dose of corticosteroids, acetazolamide, and immunosuppressant. We additionally review the previously reported cases of SLE with diffuse cerebral edema and leukoencephalopathy with a focus on the possible pathophysiological mechanisms of such association. Conclusions: We highlight, through this case report and the literature review, the importance of considering SLE in patients with cerebral edema and diffuse leukoencephalopathy and treating it aggressively.

Kharel, Sanjeev, Suraj Shrestha, Samriddha Raj Pant, Suman Acharya, Amit Sharma, Santosh Baniya, and Sanjeeb S. Bhandari. High-altitude exposure and cerebral venous thrombosis: an updated systematic review. High Alt Med Biol. 24:167-174, 2023. Background: High altitude (HA) may increase the risk of cerebral venous thrombosis (CVT). Differentiating it from other HA illnesses is crucial for prompt treatment and better outcomes. We aimed to summarize the clinical data, etiology, and risk factors of this poorly understood entity at an HA. Materials and Methods: A systematic literature search of various databases, including PubMed, Embase, and Google Scholar, was done using relevant keywords; cerebral venous thrombosis; HA, up to May 1, 2022. Results: A total of nine studies, including 75 cases of CVT at HA (3,000-8,848 m), with 66 males and 9 females, were included in this review. Headache and seizure were the most common clinical presentations. Smoking, drinking habits, and the use of oral contraceptive pills (OCP) were the most common risk factors for the development of CVT. Similarly, various underlying hypercoagulable states were also present among cases of CVT associated with HA exposure. Conclusion: Our review concludes that HA exposure can predispose individuals with risk factors such as preexisting hypercoagulable states, smoking, drinking habits, and use of OCP to an increased risk of CVT.

Cerebral venous thrombosis can result from hypercoagulation, either genetic or acquired. Hyperhomocysteninemia was previously thought to be linked with thrombophilia, although this is still controversial to this present day. In recent years, there has been a notable surge in the recreational use of nitrous oxide, which could potentially lead to hyperhomocysteinemia. We present a case of a 19-year-old female who was diagnosed with cerebral venous thrombosis with intracerebral hemorrhage. She had a history of nitrous oxide abuse, which is known to cause dysfunction of vitamin B12. Additionally, we conducted a literature review of cerebral venous thrombosis following nitrous oxide usage. Investigation showed that her serum vitamin B12 level was <100 pg/mL (reference range 197-771 pg/mL), and homocysteine level was 100.6 µmol/L (reference range 5.0-15.0 µmol/L). After receiving a vitamin B12 supplement, both serum vitamin B12 and homocysteine levels returned to normal. No other risk factors for thrombophilia were detected. Previously reported cases predominantly demonstrated hyperhomocysteinemia. The most likely mechanism of her cerebral venous thrombosis was hyperhomocysteinemia due to vitamin B12 deficiency caused by nitrous oxide abuse. This finding supports the hypothesis that hyperhomocysteinemia can induce cerebral venous thrombosis.