OBJECTIVE: We aimed to identify conditions mimicking nervous system involvement among patients with Behçet\'s syndrome (BS) and to determine clinical, laboratory and imaging findings that may help in the differential diagnosis.
METHODS: We screened the charts of 500 consecutive BS patients to identify those who were referred to neurology at any time during their follow-up. The final diagnoses, presenting signs and symptoms, laboratory and imaging results were retrieved from patient charts. Patients who did not have a follow-up visit during the last 3 months were invited to the clinic.
RESULTS: Among the 500 BS patients, 116 (23%) had been referred to neurology. Among these, 29 (5.8%) were diagnosed with typical central nervous system involvement of BS (NeuroBS). The type of NeuroBS was parenchymal involvement in 21 patients, cerebral venous sinus thrombosis in 7 patients, and both in 1 patient. 30 patients (6%) had other conditions related to the nervous system, 46 (9.2%) did not have a nervous system disorder, and their symptoms recovered spontaneously, and 11 (2.2%) were lost to follow-up without a definite diagnosis. Of the 30 BS patients who were diagnosed with another nervous system condition, 14 (46%) had primary headache syndromes, 6 (20%) had psychiatric disorders, 2 had entrapment neuropathy, and 1 each had epilepsy, glial tumor, multiple sclerosis, Meniere\'s disease, optic neuritis, neuroretinitis, steroid myopathy and polyneuropathy.
CONCLUSIONS: Nervous system conditions other than NeuroBS are frequent among BS patients referred to neurology. Caution is required to avoid misdiagnosis of these patients as NeuroBS.

The proportion of cerebral venous sinus thrombosis involving the straight sinus (StS) is low, and the prognosis is poor. We report a case of multiple sinus thrombosis involving StS in which the patient underwent mechanical thrombectomy (MT) using a stent retriever and an aspiration catheter (combined MT) with a good postoperative course. A 15-year-old girl was admitted to our hospital with rapid loss of consciousness. Magnetic resonance imaging (MRI) revealed thrombosis of the bilateral internal cerebral veins, vein of Galen, StS, torcular herophili (TH), and right transverse sinus (TS), as well as edema mainly in the left thalamus, basal ganglia, and corpus callosum. Systemic heparinization was initiated, and combined MT was performed. Although complete recanalization of the TH and right TS via the left internal jugular vein was achieved, the microwire could not be advanced to the StS. Hence, the approach route was changed to remove the thrombus from the superior sagittal sinus and successfully reach the StS via the right TS. Partial recanalization of the StS was achieved, and venous congestion was improved. Two months after MT, the patient returned to school without neurological deficits. MRI performed 3 months after MT revealed disappearance of the edema and complete recanalization of the StS. In this case, StS catheterization via the left TS was not possible. However, we could reach the right TS, which were recanalized first. Partial recanalization of the StS can be expected a good prognosis under the patency of the TH and TS.

A 21-year-old gentleman presented with low responsiveness and an unwitnessed tonic-clonic seizure. A 3-day history of fevers, headaches, and poor sleep was reported. He was initially treated for meningoencephalitis. Subsequently, he developed an erythematous rash over the face and chest. He had three generalised tonic-clonic seizures and his Glasgow Coma Score (GCS) deteriorated to 8 out of 15 requiring intubation and ventilation, and antiepileptics. Lumbar puncture (LP) results were unremarkable; however, the computed tomography (CT) head concluded bilateral haemorrhages and commented on the possibility of cerebral venous sinus thrombosis (CVST). Computed tomography venogram (CTV) confirmed CVST in the superior sagittal sinus, cortical vein and left transverse sinus. Repeat CT head revealed no new changes. Clinically, he exhibited residual left-sided weakness following stroke secondary to CVST. The patient was discharged with lifelong warfarin due to unprovoked CVST. He re-presented ten months later with persistent headaches. Clinical review noted bilateral papilloedema and he required LP to relieve raised intracranial pressure (ICP). In a 5-year follow-up, he continues to have raised ICP and associated headaches requiring further LPs. He continues to take warfarin, levetiracetam and topiramate, for headaches. This is an atypical case of CVST presenting initially with meningoencephalitis-like symptoms, demonstrating diverse clinical presentation. Ergo, this encourages an early multidisciplinary approach in presentations of headaches and seizures as clinical suspicion for CVST is high. Ultimately, this will appropriately identify patients for neuroimaging with computed tomography/magnetic resonance venogram. Furthermore, 5-year follow-up is presented in this case highlighting the importance of long-term follow-up in view of variable long-term complications that remain difficult to predict.

Juvenile neurolupus presents primarily with neuropsychiatric manifestations which may also be the initial presentation. Such primary neuropsychiatric SLE (NPSLE) events are a consequence either of microvasculopathy and thrombosis, or of autoantibodies and inflammatory mediators. Diagnosis of NPSLE requires the exclusion of other causes, and clinical assessment directs the selection of appropriate investigations. These investigations include measurement of autoantibodies, analysis of cerebrospinal fluid, electrophysiological studies, neuropsychological assessment and neuroimaging to evaluate brain structure and function. In our patient, the disease presented with chronic headache initially diagnosed as migraine, followed by fever and paraparesis. Fundoscopy showed retinal haemorrhages. Investigations revealed anaemia, neutrophilic leucocytosis, thrombocytopenia and raised inflammatory markers (ESR 119 mm/h CRP 58 mg/L) and high globulin. MRI brain showed diffuse meningeal enhancement resembling meningitis but CSF analysis was normal. ANA and dsDNA were positive with low C3, C4. All diffuse meningeal enhancements may not be meningitis and one needs to corroborate all the clinical, biochemical and imaging analyses to come to a diagnosis.

OBJECTIVE: We aimed to describe clinical and laboratory characteristics and determine the predictors of outcome in patients with cerebral venous sinus thrombosis.
METHODS: This prospective study was conducted over 2 years among hospitalized patients with cerebral venous sinus thrombosis. Patient outcome was assessed using the Modified Rankin Scale (mRS) score at 3 months. Outcome predictors were identified using logistic regression analysis.
RESULTS: Eighty-one patients were included in this study. The median mRS outcome at 3 months was 1 (interquartile range 1-3). Poor outcomes were observed in 27.2% of patients, and the mortality rate was 9.8%. Factors associated with poor outcomes were age >60 years (relative risk [RR] 5.1), hemiparesis (RR 5.4), altered level of consciousness (RR 7.1), and transverse sinus involvement (RR 1.1). In general, mRS scores were not associated with D-dimer levels (RR 2.4). However, older patients with elevated D-dimer levels showed a significant association with poor outcomes (1.6) according to mRS scores.
CONCLUSIONS: Older age, hemiparesis, and altered consciousness levels were independent predictors of poor outcomes in patients with cerebral venous sinus thrombosis. High D-dimer level showed no association with functional disability, except in older patients.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke. Acquired and inherited prothrombotic conditions are the most common risk factors for CVST. Sometimes, an etiology is not found. Wide utilization of next generation sequencing technologies in clinical practice may lead to identification of risk factors other than those classically associated with CVST.
RESULTS: This retrospective clinical-laboratory observational study has a reference patient who presented with CVST as an adolescent. Work up for prothrombotic conditions showed high homocysteine level secondary to homozygosity for a common polymorphism, c.677 C > T in the methylenetetrahydrofolate reductase (MTHFR) gene. His older unaffected brother has a similar MTHFR genotype and high homocysteine. The whole exome sequencing revealed a likely pathogenic variant in the sodium voltage gated channel, alpha subunit 1(SCN1A) gene.
CONCLUSIONS: CVST is a multifactorial disease. Prothrombotic conditions are the most common risk factors for CVST. High homocysteine due to the common MTHFR polymorphisms was previously attributed to various thrombotic conditions including CVST. Although high homocysteine due to MTHFR polymorphism may be a contributing factor, additional risk factors such as blood flow abnormalities during SCN1A related seizures may be needed for thrombosis.

UNASSIGNED: We report a case of cerebral venous sinus thrombosis (CVST) that presented with bilateral optic disc swelling and diplopia in the absence of headaches.
UNASSIGNED: A 54-year-old woman with no relevant medical history presented with a 2-week history of diplopia and no loss of visual acuity in each eye. Eye movements revealed bilateral abduction deficits, and fundoscopic examination revealed bilateral optic disc swelling. Non-contrast computed tomography of the head showed no abnormalities. Magnetic resonance venography revealed the absence of flow in the superior sagittal and left transverse sinuses as a consequence of thrombosis. The patient was diagnosed with intracranial hypertension associated with abducens nerve palsies secondary to CVST and was initiated on anticoagulant therapy. CVST can lead to stroke even in younger individuals.
UNASSIGNED: CVST should be considered in differential diagnosis when bilateral papilledema and abducens nerve palsies are present, even in the absence of headache or other neurological findings.

A substantial number of patients with intracranial dural arteriovenous fistula (dAVF) suffer from coexistent cerebral venous sinus thrombosis (CVST), yet this clinical relation is poorly studied. We aim to study the clinical and radiological outcome of patients with coexistent dAVF and CVST based on different treatment modalities and to examine various other parameters in such patients. A search strategy was performed on the following search engines: PubMed, Wiley, Microsoft Academia, and Google Scholar. Analysis was performed using R software. Thirty-five articles met the inclusion criteria. Most studies (n = 30) used digital subtraction angiography alone to make the diagnosis. Treatment modality ranged from no treatment (n = 7) to a combination of anticoagulation, endovascular therapy, and surgery (n = 2). Radiological obliteration was achieved in the majority of patients (78%). However, there was no statistical significance between treatment modality and radiological outcome (P=0.651); 72% of patients achieved clinical improvement, with no statistical significance between clinical improvement and treatment modality (P=0.072). We did find a significant relationship between treatment modality and follow-up findings (P=0.023). When analyzing factors such as age, gender, grade, arterial supply, and time between dAVF and CVST development, we found no statistical significance. Additionally, there was no significant association between the grade of fistula and the treatment modality. The clinical relationship between dAVF and CVST is poorly studied. This is the first systematic review that aimed to study this relationship. Results from subsequent large-scale studies comparing to our findings are warranted.

Cerebral venous sinus thrombosis (CVST) and hyperlipidemia are severe complications of L-Asparaginase (L-Asp) during the treatment of B-cell acute lymphoblastic leukemia (B-ALL). Herein, we reported a 9-year-old B-ALL boy who underwent abnormal hypertriglyceridemia and CVST presenting as seizures and disturbance of consciousness twice during the induction therapy. Fortunately, he survived treatment with anticoagulant and lipid-lowering therapy. No thrombophilia-related gene mutation was detected, but a heterozygous mutation in lipoprotein lipase (LPL) gene was identified. His neurological symptoms were managed with short-term anticoagulant therapy and long-term lipid-lowering therapy. This case illustrated the manifestation and potential pathogenesis of CVST and highlighted the essentiality of screening baseline lipid profile and dyslipidemia- and thrombophilia-related gene mutation.

UNASSIGNED: The high incidence of wasp stings have been causing a variety of injuries in China, but systemic complications are rarely reported.
UNASSIGNED: A 59-year-old man was severely attacked by wasps. He developed an acute onset of right hemiplegia and chest distress and was admitted to our emergency department 13 hours after being attacked. Various abnormal signals were found by biochemical tests. Magnetic resonance venography of head demonstrated that the superior sagittal sinus was not visible, indicating cerebral venous sinus thrombosis. Magnetic resonance imaging showed abnormal signals in the left frontal lobe, parietal lobe, and thalamus, indicating venous cerebral infarction and hemorrhage, coupled with subarachnoid hemorrhage. The patient was diagnosed with a rare combination of cerebral venous sinus thrombosis, cerebral venous infarction, and multi-organ dysfunction following hornet stings. After undergoing systematic treatment including blood perfusion, blood dialysis, anti-inflammatory hormone therapy, antiallergic medication, antibiotic use, and anticoagulation treatment, the patient showed significant improvement in limb muscle strength and dizziness symptoms. However, the patient developed irreversible kidney damage and is currently dependent on renal replacement therapy.
UNASSIGNED: This case highlights the serious systemic consequences that can occur following multiple wasp stings, including rare complications such as venous sinus thrombosis leading to cerebral infarction and renal failure. Early intervention with blood perfusion, hemodialysis, and plasmapheresis, in addition to general treatment, may help prevent permanent organ damage in patients with a large number of wasp stings.