Clinical Relevance Etiology and Imaging

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Bangladesh Journal of Medicine (BJM)

ISSN : 1023 - 1986


eISSN : 2408 - 8366

ORIGINAL ARTICLE
CLINICAL RELEVANCE, ETIOLOGY AND IMAGING
CHARACTERISTICS OF CEREBRAL VENOUS SINUS
THROMBOSIS AT A TERTIARY CARE HOSPITAL IN
BANGLADESH
MD. RASHEDUL ISLAM1, AMINUR RAHMAN2, TANBIN RAHMAN3, MOHAMMAD SAKHAWAT HOSSEN
KHAN4, DILRUBA ALAM5, RUMANA HABIB6

Abstract
Background: Cerebral sinus thrombus (CVST) is a rare form of stroke often affects young people
with diverse clinical, etiological and radiological presentation. The aim of the study was to evaluate
clinical relevance, etiologies, and imaging characteristics of CVST in Bangladesh. Methods: A
prospective, observational study was done at a tertiary care hospital with patients recruited in the
period of January 2021 to January 2023. 38 patients with clinical and radiological features suggestive
of cerebral venous sinus thrombosis (CVST) were studied with thorough clinical evaluation and
comprehensive work up. Results: The mean age of presentation was 28.42 years with female
predominance (n = 24). Headache was the most common presenting symptoms (92%, n = 35)
followed by vomiting (52%, n = 20). Hemi paresis (38%; n = 14) was the most common clinical sign
followed by cranial nerve palsy (26%, n = 10). 31% of the patients (n=12) had provoked CVST
among those the most common cause was found to be pregnancy/puerperium in 58% (n = 7
patients) followed by OCP which were 25% (n = 3). 69 % of the patients (n=26) had unprovoked
CVST among those the most common cause was found to be prothrombotic conditions in 85% (n =
22 patients) followed by idiopathic which were 15% (n = 4). In magnetic resonance imaging venography
(MRV), 74% of patients (n = 28) had thrombosis of transverse sinus, 53% of patients (n = 20) had
thrombosis of the sigmoid sinus and 42% patients (n = 16) of patients had saggital sinus thrombosis.
Conclusion: Clinical presentation is variable, etiology must be determined, and diagnostic method
of choice is MRV. Headache was most the common clinical presentation and the most common
etiological factor is puerperium. Provoked CVST is more common than unprovoked CVST
and transverse sinus thrombosis frequently involved.
Key words: Clinical relevance, Cerebral venous sinus thrombosis, Hemorrhagic infarct, imaging
characteristics
Received: 14.07.2023 Accepted: 16.08.2023
DOI: https://doi.org/10.3329/bjm.v34i3.68419

Citation: Islam MR, Rahman A, Rahman T, Khan MSH, Alam D, Habib R. Clinical Relevance,
Etiology and Imaging Characteristics of Cerebral Venous Sinus Thrombosis at a Tertiary Care
Hospital in Bangladesh.Bangladesh J Medicine 2023; 34: 186-191.

1. Associate Professor, Department of Neurology, BIRDEM General Hospital, Dhaka, Bangladesh.


2. Assistant Professor Dept. of Neurology, Sir Salimullah medical college , Mitford, Dhaka, Bangladesh.
3. Assistant Professor (Medicine), Anwer khan modern medical college hospital, Dhaka, Bangladesh.
4. Registrar, Department of Neurology, BIRDEM General Hospital, Dhaka, Bangladesh.
5. Assistant Professor, Department of Neurology, BIRDEM General Hospital, Dhaka, Bangladesh.
6. Associate professor, Department of Neurology, BIRDEM General Hospital, Dhaka, Bangladesh.
Correspondence: Md. Rashedul Islam, Associate Professor, Department of Neurology, Room-1420, 13th
Floor, BIRDEM General Hospital, Shahbag, Dhaka-1000, Dhaka, Bangladesh. Email: [email protected]
Copyright: © 2023 Association of Physicians of Bangladesh
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Clinical Relevance, Etiology and Imaging Characteristics of Cerebral Venous Sinus BJM Vol. 34 No. 3

Introduction: territories, and juxtacortical lesions. 9 Magnetic


Cerebral sinus thrombus (CVST) is a rare form of Resonance Imaging (MRI) brain with Magnetic
stroke that accounts for 0.5-1% of all strokes and Resonance Venography (MRV) is the current
often affects young people1. Overall CVST has an diagnostic modality of choice with very high sensitivity
incidence of 0.22– 1.32/100,000/year.4 It occurs more and specificity.110 Management of CVST is focused
frequently in young adults in contrast to arterial on timely diagnosis and treatment. The current
stroke and three times more common in females than therapeutic options for CVST includes antithrombotic
in males.2, 3 The basic pathology of CVST is thrombosis treatment with heparin overlapping with oral
of cerebral veins and the commonest site of origin is anticoagulants, thrombolysis (intravenous/local
thought to be the junction of cerebral veins and larger thrombolysis by selective sinus catheterization) and
sinuses.4 Once a thrombus is formed in the cerebral a combination of thrombolysis and anticoagulation.11
or cortical veins, it can propagate and occlude large Evaluation for an underlying pro-coagulant state may
draining venous sinuses. This causes physiological be rewarding for further prevention with long term
back pressure in the venous system, leading to anti coagulation. Outcome of CVST is a bit
cerebral oedema and occasionally cerebral infarction unpredictable, it is not unusual to see dramatic
and haemorrhage.5, 6 recovery in deeply comatose patient and, sudden
worsening in conscious patients due to extension of
The variability of clinical pictures complicates the
thrombosis. 12 Early diagnosis and appropriate
clinical diagnosis of CVST and sometimes leads to a
treatment of CVST is essential, as it may prevent
high rate of suspicion in the diagnosis. In addition,
morbidity and can be life saving.
there may be regional differences in the incidence of
risk factors and clinical manifestations.7 The clinical On the contrary to the disease burden, there is a
presentation of CVST is variable and categorized like scarcity of data on CVST in Bangladesh. We therefore
this: symptoms and signs of raised intracranial attempted to fill the gap to understand CVST in a
pressure (ICP), a focal brain lesion, or both a focal Bangladeshi setting, by conducting this study at our
lesion and raised ICP. Onset is also variable and up referral center. Our aim was to see the clinical profile,
to 40% of patients can present acutely with a stroke- etiological factors and imaging charecteristics CVST
like syndrome within 48 h of symptom onset. 5 patients who were admitted at a tertiary care hospital.
Headache is the most common symptom and is
present in <“90% of cases; in 25% of patients, it is Methods:
reported as the only symptom . 7 Given a lower We conducted a single-center, prospective,
incidence of headache in arterial stroke (25–30%), 8 descriptive study was carried out in the neurology
department of BIRDEM General Hospital, Dhaka,
the presence of severe headache in the context of
Bangladesh. Study protocol was approved by the
stroke-like symptoms can raise suspicion of CVST. institutional ethics committee and only patients/next
Seizures are also a presentation in CVST compared of kin who gave a written informed consent were
with arterial stroke (40% vs 6%). Focal neurological included in the study. The study design was a
symptoms and signs are common, such as motor prospective, observational study with patients
weakness (present in up to 40% of patients). 5 recruited in the period of January 2021 to January
Cerebral venous sinus thrombosis can be provoked 2023. A successive of 38 admitted patients in
or unprovoked and numerous risk factors are reported neurology department were included in the study
in individual patients. Up to 90% of patients with initially based on the clinical profile of raised
CVST have at least one risk factor for venous intracranial pressure and seizures with or without
thromboembolism (VTE), and thrombophilias (genetic neurological deficits.
or acquired) are detected in more than 30% of
The diagnosis of CVST was made using established
patients. 5 Female-specific risk factors are more
diagnostic criteria 13, which include (i) a clinical
important in younger age groups (ie oestrogen
hypothesis of CVST (headache, focal neurological
containing contraceptives, pregnancy and
deficit and cranial hypertension) (ii) supported by
puerperium). 6
neuroimaging showing a “delta sign” on cranial
Neuroimaging is important to the diagnosis of CVST computed tomography (CT) scan and magnetic
and working with radiologists to identify the most resonance imaging (MRI) or MR venography showing
appropriate imaging techniques is important. There cerebral sinus or venous occlusion. Cranial
are certain characteristics of parenchymal lesions hypertension was clinically diagnosed by
that are suggestive of CVST, including bilateral or neuroimaging. After diagnosis, all consecutive
parasagittal lesions, lesions crossing arterial patients (aged 20–60 years) with confirmed CVST were

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BJM Vol. 34 No. 3 Clinical Relevance, Etiology and Imaging Characteristics of Cerebral Venous Sinus

included in the study after obtaining written informed Table-I


consent. Patients with clinical symptoms suggestive Clinical profile of cerebral venous sinus thrombosis in
of arterial stroke or primary cerebral hemorrhage, aged study group.
18 to 60 years, with sinusitis, intracranial space Symptoms and signs No. of patients Percentage
occupying lesions, metabolic encephalopathy, visual
Headache 35 92
impairment due to optic nerve papilledema diagnosed
by fundoscopy, or unenhanced CT showing the arterial Vomiting 20 52
region, infarction or hemorrhage were excluded from Seizure 8 21
the study. Altered Sensorium 12 32
Neuroimaging was performed by experienced Hemiparesis 14 38
radiologists who were unaware of the patients’ clinical Fever 4 11
signs and symptoms to avoid influencing the
Papilledema 7 18
diagnosis. Magnetic resonance imaging (MRI) was
performed on a 1.5 T machine (Philips). The MR scan Cranial nerve involvement 10 26
protocol included an axial Fluid Attenuated Inversion Dysarthria/aphasia 3 8
Recovery (FLAIR), axial and coronal T2W, T1 3D
sagittal. The location, extent and nature of
In Table II an etiological point of view, 12/38 (31%)
abnormalities were recorded. Magnetic resonance had clear clinical triggers and were considered
venography (MRV) was used in both coronal and axial provoked CVT in this study. In this subset, pregnancy/
planes in the lower saturation band to eliminate puerperium 7/12(58%), OCP use 3/12 (25%) and para
signals from arterial structures. infectious 2/12 (17%), were identified as risk factors
All patients underwent complete blood count, routine for provoking CVT. The breakdown of para infectious
CVST included mastoiditis (1 patient), bacterial
blood biochemistry and coagulation profile, and
meningitis (1 patient). In the other subset of
routine laboratory tests for hypercoagulable
unprovoked CVST 26/38 (69%) where no clinical
conditions including protein C, protein S,
triggers were evident, a standard procoagulant workup
antithrombin III, factor V Leiden, homocysteine,
was done. In 22 (85%) of these cases a prothrombotic
phospholipid bodies (APLA). Other studies were state could be identified.
performed when necessary. Genetic tests were not
performed due to financial constraints.
Table-II
The data thus collected was entered into Microsoft
Etiologic profile of cerebral venous thrombosis in the
Excel work sheets. Frequencies for the clinical
study
features, etiology and radiological findings were
analyzed. Statistical analysis was done via SPSS Risk factors No. of patients Percentage
version 25 (SPSS Inc. Chicago, IL USA). Discrete Provoked (12/38)
variables were listed as counts or percentages. Parainfectious 2 17
Pregnanacy/puerperium 7 58
Results:
OCP use 3 25
A total of 38 patients were included in this study, 24
Unprovoked (26/38)
(63%) were females and 14 (37%) were males. The
Procoagulant conditions 22 8515
mean age of presentation was 28.42 years with female
predominance (n = 24). Headache was most the Idiopathic 4
common presenting symptoms (92%, n = 35) followed
by vomiting (52%, n = 20). Most common pattern of In Table: III This included Protein C deficiency in 9/
headache was diffuse throbbing type (51%) followed 22 (41%), Protein S deficiency in 7/22 (32%), APLA in
by dull aching headache (22%). Hemi paresis (38%; n 2/22 (9%) and Anti thrombin III deficiency 1/22 (5%).
= 14) was the most common clinical sign followed by Some patients had combined pro-thrombotic states
cranial nerve palsy (26%, n = 10). Among the cranial like combined Protein C & S deficiency in 3 cases. In
nerve palsy cases, upper Motor Neuron (UMN) seventh the remaining 4/26 (15%) no clear clinical or lab
nerve palsy was present in 6 cases and bilateral sixth abnormality could be identified and these were
nerve paresis in 4 patients. considered Idiopathic subset in this study. They may

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Clinical Relevance, Etiology and Imaging Characteristics of Cerebral Venous Sinus BJM Vol. 34 No. 3

have factor VIII elevation or genetic prothrombotic Discussion:


states like FVL (Factor V Leiden) or MTHFR Cerebral venous sinus thrombosis, unlike arterial
(methylenetetrahydrofolate reductase) which was not stroke, often occurs in young individuals.14 Due to
tested for in this study. diverse clinical presentation, it is often a diagnostic
challenge for the clinicians. Modern neuro-imaging
Table III techniques and diagnostic laboratory investigations
Pro-coagulant conditions identified in unprovoked CVST are providing precious information about risk factors
and clinical profile of CVST. In this study, we tried to
Procoagulant No. of Percentage highlight and compare its variable clinical
conditions (n = 22) patients presentations, etiological factors and neuro-imaging
Protein C deficiency 9 41 findings with other studies.
Protein S deficiency 7 32 Comparing the age group involved, 20–40 years was
APLA panel 2 9 the commonest age group involved in various studies
At III deficiency 1 5 with mean age of onset being 35 years. The present
study also showed similar finding with mean age of
Combined Prot C & S Def 3 14
onset being 28.42 years. Our study showed female
preponderance (F: M – 1.7:1), in contrast to some other
There were various MRI findings of CVST patients in studies. 15,16 Headache was the most common
which hemorrhagic infracts were 53%, and 18(47%) symptom in the present study accounting for 92% of
cases had dural and cortical CVST without patients. Most common pattern of headache was
parenchymal lesions as shown in Fig.-1. diffuse throbbing type (56%) followed by dull aching
headache (26%). Seizures are far more frequently
Findings of CVST on Neuroimaging in figure 1
seen in CVST than in arterial stroke. At times
seizures are heralding symptoms in CVST and should
arouse the suspicion of the diagnosis. In our study,
venous infarctions 21% of cases had seizures which are comparable with
Dural or cortical another study.12 The types of the seizures observed
CVST without in the patients were generalized tonic-clonic (68%),
47% 53%
parenchymal focal with or without dyscognitive features (22%) and
lesions focal with secondary generalization (10%). 32%
patients had altered level of consciousness at
presentation, which is comparable with Vidyasagar S
et al.17 and Dhadke VN et al.18 In this study, 11%
patients had fever at the onset which is similar to
Fig.-1: Findings of CVST on MRI of Brain.
findings of another study.18
In Table IV 74% of patients (n = 28) had thrombosis Prothrombotic conditions are the most common risk
of transverse sinus, 53% of patients (n = 20) had factor identified for unprovoked CVST in published
thrombosis of the saggital sinus thrombosis and 42% literature throughout the world. In the International
patients (n = 16) of patients had sigmoid sinus. 40%
study on Cerebral Venous Thrombosis (ISCVT)
of patients (n = 15) had multiple sinus involvement.
cohort5, 34% patients had prothrombotic conditions,
and 22% had underlying genetic prothrombotic states.
Table IV However, we didn’t find earlier published studies from
Sinuses involved on MRV in the study group. Bangladesh that have information regarding these
Sinus involved No. of patients Percentage prothrombotic conditions due to paucity of laboratory
data. Recently Pai et al.,12 and Zhou L.-X et al.,14
Transverse sinus (TS) 28 74 reported thrombophilia as a risk factor for CVST in
Superior sagittal sinus (SSS) 20 53 18% and 12.3% patients respectively. In our study
Sigmoid sinus (SS) 16 42 22/26 (85%) of the unprovoked CVST patients had
Straight sinus 2 5 predisposing thrombophilic conditions. Another study
Cortical veins 1 3 stated that, 29/36 (81%) of the unprovoked CVST of
their patients had underlying thrombophilic
Deep veins 1 3
condition.19 This included Protein C deficiency in 9/
Multiple sinus involvement 15 40 22 (41%), Protein S deficiency in 7/22 (32%), APLA in

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BJM Vol. 34 No. 3 Clinical Relevance, Etiology and Imaging Characteristics of Cerebral Venous Sinus

2/22 (9%) and Anti thrombin III deficiency 1/22 (5%). Data Availability:
Majority of cases of CVST as recorded in published The datasets analysed during the current study are
literature, have multifactorial etiology, which not publicly available due to the continuation of
suggests that pro-thrombotic workup should be analyses but are available from the corresponding
extensive. However, this is not always possible to do author on reasonable request.
due to financial issues. In the present study, 12/38
(31%) had clear clinical triggers and were considered Conflict of Interest:
provoked CVST. In this subset, pregnancy/ The authors stated that there is no conflict of interest
puerperium 7/12(58%) were identified as risk factors in this study
for provoking CVST. Another study stated that,
puerperal group consists of 4/15 (26%).18 Among the Funding:
cases of unprovoked CVT In 15% (4/26) of cases we This research received no external funding.
were unable to find any cause even after procoagulant
Ethical consideration:
workup, this constituted Idiopathic cases in our
The study was approved by the Ethical Review
study. They may be harboring some form of genetic
Committee of of BIRDEM General Hospital, Dhaka,
thrombophillia like FVL or MTHFR mutation or Factor
Bangladesh. Informed consent was obtained from
VIII elevation, but we could not test due to financial
each participant or caregivers of the patients.
constraints.
In the present study transverse sinus was most Author Contributions:
frequently involved (28/38) 74% followed by superior All authors made a significant contribution to the
sagittal sinus in (20/38) 53%, sigmoid sinus in (16/ work reported, whether that is in the conception,
38) 42%, Straight sinus in (2/38) 5%, cortical veins study design, execution, acquisition of data, analysis
alone in (1/38) 3% and deep veins in (1/38) 3% which and interpretation, or in all these areas; took part in
is comparable with another study.1 Another study drafting, revising or critically reviewing the article;
done in India showed that, transverse sinus was most gave final approval of the version to be published;
frequently involved (40/54) 74% followed by superior have agreed on the journal to which the article has
sagittal sinus in (29/54) 52%, sigmoid sinus in (27/ been submitted; and agree to be accountable for all
54) 50%, Straight sinus in (4/54) 7%, cortical veins aspects of the work.
alone in (2/54) 4% and deep veins in (1/50) 2% in
Acknowledgments:
their study. 19 In the present study, 20 cases (53%)
had venous infarctions on MRI brain, out of which 12 The authors were grateful to the staffs of the
cases (60%) were hemorrhagic infarctions and 8 cases Department neurology wards of BIRDEM General
(40%) were non-hemorrhagic infarctions in a venous Hospital, Dhaka, Bangladesh
distribution. The remaining 18 (47%) cases had only References:
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