Cerebral Venous Thrombosis

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Cerebral Venous Thrombosis

• Department of Neurosciences
• Canberra Hospital
• March 1999

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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.

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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

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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

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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

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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

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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.

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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.

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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

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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
• Latehyperuintense thrombus on T1 T2
• .CRANIOTOMY
• OTHERS EEG, CSF, isotope brain scanning.

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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.

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TREATMENT

• 1.Infective cause
• 2.Increased intracranial pressure
• 3.Anticoagulation
• initially heparin
• warfarin (?duration)
• direct urokinase infusion

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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.

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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.

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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.

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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.

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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.