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Bashir, Meherali, and Chandrakar: Clinical profile and treatment outcome of patients with cerebral venous sinus thrombosis


Introduction

It is noteworthy that cerebral venous sinus thrombosis (CVST) is a distinct subgroup of cerebrovascular diseases with a variety of deceptive and fluctuating clinical manifestations resulting in complex diagnostic and therapeutic challenges. It has been established that cerebral venous sinus thrombosis is responsible for 10% to 20% of strokes in young individuals. Prior to this discovery, this condition was thought to be rare. In the literature, more than 100 potential causes of CVST have been reported.1 Many risk factors are associated with CVST, such as pregnancy, puerperium, prothrombotic states, and malignancy.2, 3 However, in 20%-25% of patients, the cause remains unknown, despite extensive investigation.

The presenting symptoms include headache, hemiparesis, seizures, altered sensorium, and papilledema.4 Magnetic resonance imaging (MRI) with venogram is the preferred diagnostic method. Patients are treated with either anticoagulation or thrombolysis. It is primarily done using heparin in conjunction with oral anticoagulants. The thrombolysis can be administered via selective sinus catheterization or a systemic route in patients who don’t respond to anticoagulation.5, 6 To mitigate the risk of recurrent epiosdes , long-term anticoagulation should be implemented in patients with prothrombotic disorders.7

CVST patients can have variable outcome depending on their underlying etiology, but their prognosis is generally good.   The main causes of mortality in these patients are infections, metabolic abnormalities, brain herniation, hemorrhagic venous infarctions, and aspiration pneumonias.8 Timely diagnosis and prompt treatment is crucial for favorable outcome. The purpose of this research was to evaluate the clinical characteristics, risk factors and treatment options in patients diagnosed with CVST.

Materials and Methods

An observational prospective study was conducted at the Department of General Medicine, Dr. Dr. DY Patil Hospital and Research Centre, Nerul, Navi Mumbai, for a period of one year. Patients were selected  sequentially, using a non-probability method. The Institutional Ethical Committee approved the enrollment of patients in this study. A written consent was obtained before enrolling the subjects.

A total of 30 patients over the age of 12 years with clnical features and history suggestive of CVST were included. Patients with arterial stroke, space-occupying lesions, metabolic encephalopathy, and those unwilling to participate were excluded. MRI with MRV were performed on all patients with the 1.5 Tesla magnetic resonance machine. Anticoagulants and supportive measures were administered.  Patients were monitored for symptomatic improvement and residual neurological deficits. The statistical data was analyzed using SPSS version 25 (developed by SPSS Inc. in Chicago, USA).

Results

The mean age of our cohort was 31.53 ±12.40 years . Table 1 shows that 40% of the patients were males (n = 12), and 60% were females (n = 18).  In Table 2, headache was the most prevalent symptom (36.67 %), followed by seizures (33.33%; n = 10), stroke (20%; n = 6), headaches and seizures (6.7 percent; n = 2), and unconsciousness (3.3%; n = 1). Table 3 shows that 11 (36.67%) had thrombosis of the transverse sinus, cerebral veins, and superior sagittal sinus, whereas 26.67% had thrombosis of the superior sagittal sinus. A total of 13.3% of patients suffered both sigmoid and transverse sinus thrombosis. It is estimated that 66.7% of brain damage occurred in the temporal lobe and 13.3% in the frontoparietal lobe. 

Cosidering the risk factors, 16.6% (n = 24) had hyperhomocysteinemia and 13.4% were in the postpartum period. In addition, 6.7% were affected during peripartum, and an equal percentage had predisposing factors such as chronic suppurative otitis media (CSOM), tuberculous meningitis, trauma, and sinusitis.  Among 23.3% of patients, no cause was identified (Table 4). Twenty-five patients (83.3%) received LMWH and warfarin, and five (16.7%) received unfractionated heparin and warfarin. 14 patients were treated with anticonvulsants. A total of 24 patients (80%) had regular follow-ups, and six (20%) had irregular follow-ups. 22 patients (73.3%) recovered fully, and 8 patients (26.7%) recovered partially  (Table 6).

Table 1

Baseline characteristics of the study patients

Variable

Mean±SD

Range

Age in years

31.53±12.40

19-67

Gender

Male: Female

2:3

-

Table 2

Clinical profile

Variable

Frequency

%

Headache

11

36.7

Seizure

10

33.3

Stroke

6

20

Headache and seizure

2

6.7

Unconscious

1

3.3

Table 3

Sinus involvement

Variable

Frequency

%

SSS,TS,CV

11

36.7

SSS

8

26.7

SS,TS

4

13.3

SS,SSS

2

6.7

SS

1

3.3

SSS,CV

1

3.3

SSS,TS

1

3.3

TS

1

3.3

TS,CV

1

3.3

Table 4

Profile ofrisk factors

Variable

Frequency

%

Idiopathic

7

23.3

Hyperhomocysteininemia

5

16.7

Postpartum

4

13.4

Csom

2

6.7

Peripartum

2

6.7

TB Meningitis

2

6.7

Trauma

2

6.7

Sinusitis

2

6.7

Bacterial Meningitis

1

3.3

OC pills

1

3.3

Protein C deficiency

1

3.3

Puerperial sepsis

1

3.3

Table 5

Profile of treatment

Treatment

Frequency

%

LMWH and warfarin

25

83.3

Unfractionated Heparin with warfarin

5

16.7

Table 6

Treatment outcome

Variable

Frequency

%

Recovered

22

73.3

Partially recovered

8

26.7

Discussion

Young individuals are more vulnerable to cerebral venous sinus thrombosis1. Diagnosis can be challenging due to the wide range of presenting symptoms. Neuroimaging advances, however, have made early detection easier due to improved diagnostic accuracy. The purpose of this study was to elucidate the various clinical manifestations of CVST. We compared our results with the existing literature on the topic to identify the potential risk factors and assess treatment outcomes.

In most comprehensive studies on CVST, the age range is between 37 and 39 years. However, it can affect all age groups. The mean age of study population was 31.53 years±12.40 years and males to females ratio was 2 : 3. According to a Sudanese research, the mean age was 33.9 ± 11.8, with females comprising 80% of the sample. 8 CVST primarily affects young females, as evidenced by research conducted in nearby countries like Iran, Oman, and Saudi Arabia. 9, 10, 11, 12, 13 According to Narayan et al., males have higher prevalence of CVST as a result of increased alcohol consumption, advances in obstetric care, and clinical awareness that leads to early detection of CVST. 2

The predominant clinical feature was headache in 36.7% patients, in accordance with a study by Idris et al. 8 This incidence was slightly lower when compared to other regional and international studies on CVST.14, 15 A Turkish case report has documented that CVST was misdiagnosed as subarachnoid hemorrhage as headache being the only presenting symptom. 16 This highlights the importance of comprehensive clinical evaluation and investigation, especially in high -risk individuals. . Deep vein thrombosis (DVT) and stroke have been associated with elevated levels of homocysteine in the bloodstream, known as hyperhomocysteinemia. 17 In our cohort, hyperhomocystenemia was observed in 16.7%, with females showing a higher prevalence. Cantu et al. found an increased risk of CVST was associated with hyperhomocysteinemia and decreased plasma folate in Mexicans. 18 These findings were attributed to the prevailing low socioeconomic conditions and inadequate nutritional status experienced by the population. The study by Narayan et al., 2 showed 18.2% of patients had raised homocysteine levels which was in line with the present study . Compared to arterial strokes, cerebral venous sinus thrombosis (CVST) is more likely to cause seizures. The present study revealed that a significant proportion of patients (33.3%) had seizures resembling those described by Narayan et al.2 and the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT).19

The most commonly affected sinuses are the Superior Sagittal Sinus, the Transverse Sinus, and the Cerebral Vein, which account for 36.7% of all cases. Additionally, 26.7% of patients had thrombosis in the Sagittal Venous Sinus (SSS).   In comparison to Saudi Arabia and the International Study on Cerebral Veins and Dural Sinus (ISCVT), these findings are closely aligned with those of similar studies conducted in Oman, Iran, and India.9, 10, 12, 17, 18, 19

In terms of treatment options, 83.3 percent used LMWH and warfarin, while 16.7% used UFH and warfarin. In total, 73.3% of patients were fully recovered. According to the ISCVT cohort and an Oman study, these proportions are consistent.9, 19 The primary treatment approach focuses on detecting the condition early and initiating anticoagulation as soon as possible. The underlying etiological factors must also be investigated and managed in conjunction with anticoagulation. According to current recommendations, heparin should be administered immediately followed by long-term treatment with warfarin; the duration of anticoagulation is governed by the underlying cause of the bleeding.19 Typically, anticoagulation should be administered for 3–6 months for CVST that has been provoked and for 6–12 months for CVST that has not been provoked; lifelong if CVST has been recurrent or if CVST has been associated with venous thromboembolism or severe thrombophilia.17   Due to the lack of definitive evidence, direct acting oral anticoagulants are not recommended first-line therapy for patients ineligible for warfarin. In light of the fact that recanalization occurs primarily within the first few months following CVST, determining the duration of anticoagulation based on it seems inappropriate since it does not appear to be related to clinical outcomes.20, 21

Conclusion

Cerebral Venous Sinus Thrombosis (CVST) can present with a variety of clinical manifestations.  The clinical and radiology departments must work closely together to make an accurate diagnosis. The most common symptom on presentation was headaches, followed by seizures, with a subset of patients experiencing both. In most cases, the superior sagittal sinus and transverse sinus were affected. With a good prognosis, heparin and warfarin were the main treatment options.

Conflicts of Interests

The authors have no financial interests or conflicts of interest.

Source of Funding

None.

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