Cerebral Venous Thrombosis

 05 November 2004

Rare and severe disease characterised clinically by headache, papilledema, seizures, focal deficits, coma and death; pathologically by hemorrhagic infarction often contraindicating anticoagulation.

POWERPOINT PRESENTATION

HISTORICAL BACKGROUND

Ribes 1825

45 yo man

6 months severe headache, epilepsy and delirium.

Postmortem: superior sagittal sinus, left lateral and left parietal cortical vein thrombosis

Abercrombie 1828

Postpartum cerebral venous thrombosis

Incidence

Unknown incidence

Increased frequency of diagnosis since advent of DSA, CT & MRI/V.

Ehlers & Courville 1936

16 sagittal sinus thrombosis in 12500 autopsies

Kalbag & Woolf 

21.7 deaths per year in England & Wales from 1952 – 1961.

Male/female ratio = 1.29/1

Males uniform age distribution

Females 61% CVT in 20-35 age group

Venous Anatomy

SINUSES

Superior sagittal sinus

Lateral sinus

Cavernous sinus

Straight sinus

CEREBRAL VEINS

Cortical veins

Deep cerebral veins

Posterior fossa veins

FREQUENCY OF VENOUS SITES

(OFTEN MULTIPLE)

FREQUENCY OF VENOUS SITES

(SINGLE SITE)

ETIOLOGY

IDIOPATHIC

INFECTIVE

Local: direct septic trauma

          Intracranial infection

          Regional infection

General: Septicemia, measles, encephalitis, HIV, CMV, malaria

NONINFECTIVE

Local: head injury, neurosurgery, tumors, infusions into jugular vv

General: Postoperative, pregnancy/postpartum, dehydration, inflammatory bowel disease, connective tissue disease, malignancy, thrombophilia.

CLINICALLY BY SYNDROMIC DESCRIPTION

1.Isolated intracranial hypertension 40%

          mimic benign intracranial hypertension

2.Focal signs 50%

3.Cavernous sinus thrombosis

4.Unusual presentations

          Psychaitric disturbances, migraines, subarachnoid hemorrhages.

 CLINICALLY BY SYMPTOMATOLOGY

INVESTIGATIONS – DIAGNOSTIC

1.    CT

Infarction in nonarterial distribution (often hemorrhagic)

Empty delta sign

Dense triangle sign

Cord sign

2.    DSA

3.    MRI/V

Early: absence of flow void & isointense on T1 for occluded vessel

           Hypointense on T2         

      Late:hyperintense thrombus on T1 & T2

4.    CRANIOTOMY

OTHERS: EEG, CSF, isotope brain scanning.

INVESTIGATIONS – ETIOLOGIC

FBE

ANA, antiphospholipid antibodies

APC resistance (Factor V Leiden)

Antithrombin

Protein C, S

Homocysteine

Thrombin mutation

Repeat tests in 4-6 months.

TREATMENT

1.Infective cause

2.Increased intracranial pressure

3.Anticoagulation: initially heparin

                             warfarin (?duration)

                             direct urokinase infusion

PROGNOSIS

MORTALITY

          Untreated: 50%

          Treated: nonseptic cause 10%

                       septic cause 30%

OUTCOME

77% no sequelae

20% develop thrombosis intra or extracerebrally

Longest followup study is 8 yrs.

THROMBOPHILIA & CEREBRAL VENOUS THROMBOSIS

25% CVT have a detectable thrombophilia (APC resistance; antithrombin, protein C or S deficeincy)

20% CVT have APC resistance

                    95% APC resistance due to Factor V leiden.

In patients with CVT attributable to APC resistance,

72% had a second contributing factor (OCP, other thrombophilia)

Contribution of  G20210A prothrombin gene mutation unknown.

 ORAL CONTRACEPTIVE PILL AND CVT

Relative risk of developing CVT

De Bruijn et al. Case control study of risk of cerebral sinus thrombosis in oral contraceptive users who are carriers of  hereditary prothrombotic conditions. BMJ 1998: 316, 589-92.