Cerebral Venous Thrombosis

Department of Neurosciences
Canberra Hospital
March 1999
 Cerebral Venous Thrombosis

• Rare and severe disease characterised clinically by

headache, papilledema, seizures, focal deficits, coma and
death; pathologically by hemorrhagic infarction often
contraindicating anticoagulation.
 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
 FREQUENCY OF VENOUS SITES
(OFTEN MULTIPLE)
• Superior sagittal sinus 72%
• Lateral sinus 70%
• Right 26%
• Left 26%
• Both 18%
• Straight sinus 14.5%
• Cavernous sinus 2.7%
• Cerebral veins 38%
• Superficial 27%
• Deep 8%
• Cerebellar veins 3%
 FREQUENCY OF VENOUS SITES
(SINGLE SITE)

• One sinus only 23%

• Superior sagittal sinus 13%
• Lateral sinus 9%
• Straight sinus 1%
• Deep veins only 1%
• Isolated cortical veins 1%
 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
– Psychiatric disturbances, migraines, subarachnoid
hemorrhages.
 CLINICALLY BY SYMPTOMATOLOGY
• Headache 75%
• Papilledema 49%
• Motor or sensory deficit 34%
• Seizures 37%
• Drowsiness, mental changes, confusion, or coma 30%
• Dysphasia 12%
• Multiple cranial nerve palsies 12%
• Cerebellar incoordiantion 3%
• Nystagmus 2%
• Hearing loss 2%
• Bilateral or alternating cortical signs 3%
 INVESTIGATIONS – DIAGNOSTIC
• .CT
– Infarction in nonarterial distribution (often hemorrhagic)
– Empty delta sign
– Dense triangle sign
– Cord sign
• .DSA
• .MRI/V
– Early: absence of flow void & isointense on T1 for occluded
vessel; Hypointense on T2
– Late:hyperuintense thrombus on T1 & T2
• .CRANIOTOMY
• OTHERS: EEG, CSF, isotope brain scanning.
 INVESTIGATIONS – ETIOLOGIC

• FBE
• ANA, antiphospholipid antibodies
• APC resistance (Factor V Leiden)
• Antithrombin
• Protein C, S
• Homocysteine
• Prothrombin gene 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.
 SUMMARY

• Uncommon but life threatening disease.

• Mimic many benign conditions.
• Untreated carries 50% mortality.
• If treated, majority of patients have no long term disability.
• An underlying cause should always be sought.
THROMBOPHILIA & CEREBRAL VENOUS
THROMBOSIS
• 25% CVT have a detectable thrombophilia (APC resistance;
antithrombin, protein C or S deficeincy, antiphospholipid syn)
• 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

• OCP RR13
• Thrombophilia RR4
• OCP & Thrombophilia RR30

• De Bruijn et al. Case control study of risk of cerrebral

sinus thrombosis in oral contraceptive users who are
carriers of hereditary prothrombotic conditions. BMJ
1998: 316, 589-92.
 ISSUES
• APC resistance is not always caused by Factor V
Leiden.
• Different thrombogenicity of second vs third generation
OCP.
• Contribution of G20210A prothrombin gene mutation
to CVT.