DR. DIVAKAR REDDY MMBBS;( DNB GENERAL MEDICINE)

CEREBRAL VENOUS THROMBOSIS(CVT)

• Thrombosis of dural sinus and/or cerebral veins

• Uncommon form of STROKE

• 0.5% to 1% strokes

• CVT has diversity of causes and presenting scenarios

• YOUNG>>> OLD

• F:M- 3:1.. Pregnancy/peurepreum/ocp

ETIOPATHOGENESIS• Predisposing conditions of CVT are multiple• Risk factors for venous thrombosis In general are linked clasically to VIRCHOWS TRIAD

Among all risk factors imp.. Prothrombotic conditions

• Important mechanisms involved in development of c/f.. a) Thrombosis of cerebral/dural sinuses leading to cerebral parenchymal leisons/dysfunction b) Occlusion of dural sinus leading to decreased CSF absorption and raised ICT

RISK FACTORS• INHERITED THROMBOPHILIA Factor V leiden mutation Prothrombin gene mutation Protein S deficiency Protein C deficiency Anti thrombin deficiency Homocysteinemia Dysfibrogenemia

ACQUIRED DISORDERS Malignancy CHF APLA surgery IBD PNH Trauma Nephrotic syndrome Polycythemia vera Pregnancy HIV Multiple myeloma OCP’ s HRT Myeloproliferative d/s Hyperviscocity

Pathogenesis

CLINICAL FEATURES

• CVT has a highly variable clinical presentation• It can present as… Acute, Subacute, Chronic• Presentation as TIA also repoted• Signs and Symptoms can be grouped into 3 main syndromes

• 1) ISOLATED INTRACRANIAL HYPERTENSION SYNDROME( Headache +/- vomitings,papilledema,visual disturbances)• 2) FOCAL SYNDROME( focal deficits, seizures, both)• 3) ENCEPHALOPATHY( mutlifocal signs, mental status changes, stupor

or coma)

• Variables affecting presentation and course Age Presence of parenchymal leisons Site and number of occluded sinuses and veins Gender Interval from CVT onset and presentation

HEADACHE most frequent symptom common in young pts and women usually gradual in onset, often localised than diffuse but site of headache has no relation to site of occluded vessel/ parenchymal leison if due to ict.. Dull difuse and increases with valsalva and recumbency

SEIZURES Focal/generalised more common compared to other forms ENCEPHALOPATHY

FOCAL SYMPTOMS AND SIGNS

INVESTIGATIONS 1) NEURO IMAGING a) NON INVASIVE- CT,CTV, MRI ,MRV , ULTASOUND b) INVASIVE - CEREBRAL ANGIOGRAPHY, DIRECT CEREBRAL VENOGRAPHY

NON INVASIVE

CT,CTV CT is initial modality in new onset neurological symptoms, often normal only around 30% cases abnormal signs in CT associated with CVT Dense triangle sign/Empty delta sign/ Cord sign other fidings.. Hemorrhagic leisons/ non-hemorrhagic leisons like edema venous infarction

MRI, MRV MRI using gradient echo T2* susceptibility-weighted sequences in combination with MR venography is the most sensitive imaging

In the first five days, the thrombosed sinuses appear isointense on T1-weighted images and hypointense on T2-weighted images Beyond five days, venous thrombus becomes more apparent because signal is increased on both T1 and T2-weighted images After the first month, thrombosed sinuses exhibit a variable pattern of signal, which may appear isointense

MRV, usually performed using the time-of-flight (TOF) technique, is useful for demonstrating absence of flow in cerebral venous sinuses, though interpretation can be confounded by normal anatomic variants such as sinus hypoplasia and asymmetric flow

• CEREBRAL INTRA ARTERIAL ANGIOGRAPHY is recommended mainly when the diagnosis of CVT is uncertain, such as in the rare suspected cases of isolated cortical vein thrombosis, or when the clinical suspicion for CVT is high but CT venography or MR venography are inconclusive• Anatomic variations, such as variability of number and location of cortical veins, hypoplasia of the anterior part of the superior sagittal sinus, duplication of the superior sagittal sinus, and hypoplasia or aplasia of the transverse sinuses, may make the diagnosis of CVT by all types of angiography difficult 

2) LAB INVESTIGATIONS Role of D-DIMER- negative predictive value Role of LP- to r/o meningitis, to measure csf pressure ROUTINE LAB TESTS

TESTING FOR THROMBOPHILIA SHOULDNOT BE DONE IN ACUTE STATE

TREATMENT ANTICOAGULATION OTHER TREATMENTS FIBRINOLYTIC THERAPY DIRECT CATHETER ABLATION MECHANICAL THROMBECTOMY/ THROMBOLYSIS SURGERY

• STEROIDS.. Useful in vasogenic edema but not recommended routeinly. steroid medications are not recommended, even in the presence of parenchymal brain lesions on CT/MRI, unless needed for another underlying disease

ANTIBIOTICS Local infection can cause CVT so appropriate antibiotics should be adminsitered if needed surgical intervention should be done

ANTICOAGULATION Initial anticoagulation by.. LMWH/ UFH( dose adjusted with a goal of 2-3 times control APTT) Later.. oral Vit K Antagonists Target INR- 2.0-3.0

DURATION.. 1) Povoked CVT- 3-6 mon (associated with transient risk factor) 2) Unprovoked CVT- 6-12 mon 3) recurrent/ CVT with severe THROMBOPHILIA / VTE after CVT- indefinite anticoagulation

severe thrombophilia- deficeincy of protein c/ protein S/ antithrombin, APLA, homozygous factor V leiden, homozygous prothrombin G20210A

• Testing for protein C,protein S, and antithrombin deficiency is generally indicated 2 to 4 weeks after completion of anticoagulation.There is a very limited value of testing in the acute setting or in patients taking warfarin

• Special situation of CVT with cerebral hemorrhage on presentation

Anticoagulants appear to be safe to use in adult patients with CVT who have intracranial hemorrhages, either intracerebral or subarachnoid

• ENDOVASCULAR THROMBOLYSIS  Some patients with CVT worsen despite anticoagulant therapy. Direct endovascular thrombolysis has been used as an alternative treatment in such cases. Direct thrombolysis aims to dissolve the venous clot by delivering a thrombolytic substance (urokinase or rt-PA) within the occluded sinus through an intravenous catheter. In some cases, mechanical endovascular disruption of the thrombus has also been used  • DECOMPRESSIVE HEMICRANIECTOMY In patients with neurological deterioration due to severe mass effect or intracranial hemorrhage causing intractable intracranial hypertension, decompressive hemicraniectomy may be considered

COMPLICATIONS EARLY LATE

1)EARLY.. a)SEIZURES- 37% cases Rx.. Controversial.. To initiate or await initial seizures before treatment RECOMMENDATIONS Early initiation of AED in pts with CVT and single seizure with parenchymal leisons for definite period is recommended to prevent furthur seizure CVT with seizures withOUT parenchymal leison AED initiation is probably recommended Pts withOUT seizures routine use of AED not recommended

b) HYDROCEPHALUS Communicating/ Obstructive If obstructive- ventriculostomy/ VP shunt

c) INTRACRANIAL HYPERTENSION 40% Rx.. Anticoagulation LP Acetazolamide Decompressive craniotomy

LATE HEADACHE 50% Common complaint in follow up Persistent/severe headache- r/o recurrence or intracranial HTN In patients with a history of CVT who complain of new, persisting, or severe headache, evaluation for CVT recurrence and intracranial hypertension should be considered VISUAL LOSS SEIZURES DURAL ARTERIOVENOUS FISTULA

CVT and PREGNANCY one of risk factor because of hypercoaguable state• The greatest risk periods for CVT include the third trimester and the

first 4 postpartum weeks• Cesarean delivery appears to be associated with a higher risk of CVT• women with a history of VTE appear to have an increased risk of

thrombotic events • CVT is not a contraindication for future pregnancies

• Recommendations1. For women with CVT during pregnancy, LMWH in full

anticoagulant doses should be continued throughout pregnancy, and LMWH or vitamin K antagonist with a target INR of 2.0 to 3.0 should be continued for at least 6 weeks postpartum (for a total minimum duration of therapy of 6 months)

2. It is reasonable to advise women with a history of CVT that future pregnancy is not contraindicated. Further investigations regarding the underlying cause and a formal consultation with a hematologist and/or maternal fetal medicine specialist are reasonable.

3. It is reasonable to treat acute CVT during pregnancy with full-dose LMWH rather than UFH

4. For women with a history of CVT, prophylaxis with LMWH during future pregnancies and the postpartum period is probably recommended

PROGNOSIS

CVT is associated with a good outcome (complete recovery or minor residual symptoms or signs) in close to 80 % of patients. Nevertheless, approximately 5% of patients die in the acute phase of the disorder, and longer-term mortality is nearly 10%. The main cause of acute death with CVT is neurologic, most often from brain herniation. After the acute phase, most deaths are related to underlying disorders such as cancer Causes of death in acute phase… TranstentorialHerniation, Diffuse brain edema, Statusepilepticus, Medicalcomplications, Pulmonaryembolism cause of death in later phase is generally due to underlying cause like cancer• Neurological worsening may occur in 23% of patients,

even several days after diagnosis. Approximately one third of patients with neurological deterioration will have new parenchymal lesions when neuroimaging is repeated

Predictors of mortality at 30 days

• Depressed consciousness• Altered mental status• Thrombosis of the deep venous system• Right hemisphere hemorrhage• Posterior fossa lesions

Predictors of poor long-term prognosis • Central nervous system infection• Any malignancy• Thrombosis of the deep venous system• Hemorrhage on head CT or MRI• Glasgow coma scale score <9 on admission • Mental status abnormality• Age >37 years• Male gender

• Recanalization In a systematic review of 5 small studies, recanalization rates of CVT at 3 months and 1 year of follow-up were 84% and 85%,respectively. The highest rates of recanalization are observed in deep cerebral veins and cavernous sinus thrombosis and the lowest rates in lateral sinus thrombosis. In adults, recanalization of the occluded sinus is not related to outcome after CVT. A follow-up CTV or MRV at 3 to 6 months after diagnosis is reasonable to assess for recanalization of the occluded cortical vein/sinuses in stable patients

Recurrence The risk of recurrent CVT is approximately 2 -4 %, while the risk of recurrent venous thromboembolism in other locations ranges from 4 - 7 %

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