Cerebral venous sinus thrombosis (CVST) is a rare form of stroke and refers to thrombosis of the cerebral sinuses and/or cerebral veins (1). CVST generally occurs in young patients (typically <50 years old) (1, 2) and affects women more than men (2). CVST accounts for 0.5–1% of all strokes (1), with an incidence of about 5–16 cases per million people annually (1, 2). The presentation is highly variable, and symptoms can take a few weeks to develop (3, 4). The common risk factors for CVST include high-risk thrombophilia (e.g., antithrombin III, protein C and protein S deficiency, homozygosity for either factor V Leiden, or prothrombin G20210A mutations), use of oral contraceptives, pregnancy, malignancy, infection, trauma, and populations in developing countries (1, 2, 5). The complications of CVST include intracranial hemorrhage, intracranial hypertension, and seizures (1). Mortality is 4–5% in the acute phase (2).

The risk factors for 30-day mortality include depressed consciousness, altered mental status, thrombosis of the deep venous system, right hemisphere hemorrhage, and posterior fossa lesions (1, 6, 7). The factors associated with poor short-and long-term outcomes include demographic factors (age > 33–37 years and male sex), clinical factors (coma, neurologic deficit on National Institutes of Health Stroke Scale (NIHSS), encephalopathy, decreased level of consciousness, hemiparesis, and seizures), neuroimaging factors (intracerebral hemorrhage, involvement of straight sinus, thrombosis of deep vein system, and venous infarction), and other risk factors (cancer, central nervous system infection, and underlying coagulopathy hereditary thrombophilia) (1, 8).

Nevertheless, the prognostic factors of CVST remain poorly understood, mainly because of the small number of cases. Furthermore, a study revealed that the epidemiology of CVST significantly changed from 1942 to 2012 because of improvements in treatments, changes in risk factors, and improvements in diagnostic modalities leading to mild cases being diagnosed (9). The exact natural history and prognosis of patients with mild CVST are poorly known since they were not included in early studies. A study tried to develop a risk score to predict the outcomes of CVST, but its predictive value was low (10). Therefore, recent data on the prognosis of CVST are necessary. Evidence regarding the etiology, treatment response, and prognosis of CVST is still lacking.

Therefore, this study aimed to examine the etiology, treatment response, and prognosis of patients with CVST at a single institution. The results could help improve the management of patients with CVST.

CVST is a rare condition. Its epidemiology is poorly known, especially with the improvements in diagnostic modalities and the diagnosis of mild cases. Therefore, this retrospective study examined the etiology, treatment response, and prognosis of patients with CVST at a single institution. The median age was 39 years. The majority were female (58%), 13% had thrombotic disorders, and 19% were or were recently pregnant. Only an NIHSS of >0 at follow-up was associated with a 3-month mRS >0.

This case series was small, but the results were consistent with those previously reported. Indeed, Khan et al. (6) reported 33 patients from Pakistan; 88% of their patients were female, and the mean age at presentation was 31 years. Ibrahim et al. (15) reported a median age of presentation of 35 (range, 23–75) years. Similar results were reported by Bousser et al. (16) and in a systematic review by Coutinho et al. (9). In China, Qiu et al. (17) reported that females were predominant and that the age at presentation was 20–40 years, also supporting the present study. A multicenter study of 11,400 patients with CVST reported a mean age at presentation of 38.1 years (18).

In the present study, 19% of the patients had an mRS >0 at presentation, while previous studies reported 32–39% of patients with altered consciousness (6, 19). The discrepancies could be due to differences in sample size, patient populations, and diagnostic methods. Headaches were the most common symptom. Although headaches are non-specific, they are the most common symptoms reported in the CVST literature, while other neurological symptoms were reported in 30–50% of the patients (6, 15, 19–22).

An international study revealed that the most common sinus involvement was the superior sagittal sinus (62%), followed by the transverse sinus (42%), straight sinus (18%), cortical veins (17%), and deep veins (11%) (23). Khan et al. (6) showed that the transverse sinus was commonly involved (55%), followed by the sigmoid sinus (52%), superior sagittal sinus (46%), and straight sinus (15%), similar to Bousser et al. (16) and Banakar et al. (24). In the present study, the transverse sinus (71.0%), superior sagittal sinus (48.4%), sigmoid sinus (45.2%), straight sinus (32.3%), internal jugular vein (6.45%), and cortical veins (3.23%) were involved. Discrepancies among studies could be due to differences in scanners, imaging modalities and parameters, and radiologist experience. Of note, magnetic resonance has a sensitivity of 72–84% and a specificity of 89.9–95% for CVST (25, 26).

The most important feature of CVST management is early recognition and anticoagulation (2, 21). Concomitant conditions like infections and seizures should also be managed appropriately. Other conditions should also be managed (e.g., edema, dehydration, and malnutrition).

In the present study, 86.7% of the patients had an mRS of 0 at 3 months, indicating excellent outcomes. No deaths were observed. Previous studies reported good outcomes in 79% of the patients, in-hospital mortality of 2.0%, mortality of 9–15%, and 1-year recurrence of 6.5% (18, 20, 21). A previous study showed that headaches were a protective manifestation of CVST, while consciousness disturbances, intracranial injury, and hematologic diseases indicated poor clinical prognosis; no significant associations were found between the number and location of lesions and clinical prognosis (22). Factors of poor outcomes also include age > 33–37 years, male sex, coma, neurologic deficit on NIHSS, encephalopathy, decreased level of consciousness, hemiparesis, seizures, intracerebral hemorrhage, involvement of straight sinus, thrombosis of deep vein system, venous infarction, cancer, central nervous system infection, and underlying coagulopathy (1, 8). Khan et al. (6) reported that the factors of poor prognosis in women with CVST were spontaneous vaginal delivery, primigravida, and anemia, but most of their patients were postpartum. Additional studies are still necessary to determine poor prognosis factors in patients with CVST. In the present study, an NIHSS of >0 at follow-up was associated with a 3-month mRS >0. The NIHSS was designed to quantify the impairments after a stroke (27, 28). The mRS measures the degree of disability or dependence on daily activities after a stroke or other neurological disabilities (29). Therefore, although there are differences between the two scales, they examine a similar outcome, and their association is not surprising.

This study had limitations. It was a single-center study spanning a relatively short period, resulting in a small sample size. The study was retrospective, limiting the data to those available in the patient charts. The 3-month outcomes were available for 30 patients. Long-term outcomes were unavailable for most patients since their mRS at 3 months was good, and many patients were returned to their local hospital. A risk score model could not be developed because of the small sample size. A multicenter study would increase the sample size and probably allow the development of a risk score model. The treatment groups were too small to allow statistical analyses and investigate the differences in patient characteristics and outcomes among the treatment groups. Therefore, only a description of the factors was possible. A multicenter study would allow the investigation of the effect of different treatments on patient outcomes and the clinical factors that could lead to the selection of a treatment pattern.

In conclusion, this case series reported the features of patients with CVST treated at a single institution in recent years. Although all patients with CVST diagnosed during the study period were included, most had mild CVST. In addition, the majority of patients were female. The median age was 39 years, 13% had thrombotic disorders, and 19% had a pregnancy history. Only an NIHSS of >0 at follow-up was associated with a 3-month mRS >0. Multicenter studies should be performed to examine the prognostic factors of CVST in a larger patient population.