Cerebral Sinus Thrombosis

7 Cerebral Sinus Thrombosis


Isabel Fragata and Aman Patel


Thrombosis of cerebral veins and dural sinuses is an uncommon clinical entity, representing less than 1% of all stroke cases in adults. Cerebral sinus venous thrombosis (CVT) is more common in neonates and children than in adults; among adults, it is more common in women.1 The mean age of onset is 39 years, and 75% of cases involve multiple veins and/or sinuses.1 Obstruction of cerebral venous drainage can lead to increased venous and capillary pressure with subsequent blood-brain barrier breakdown and vasogenic cerebral edema, venous hemorrhage, and/or ischemia or cytotoxic edema. Cerebrospinal fluid (CSF) absorption is also impaired, further contributing to elevated intracranial pressure (ICP). Elevated ICP is most common with superior sagittal sinus obstruction, but it can occur with jugular or transverse sinus thrombosis. A high index of clinical suspicion and neuroimaging techniques are the key to diagnosis.2



Case Example


A 35-year-old woman on oral contraceptives has complained of headache for the last few days. She had been admitted for a focal seizure and is now stuporous, with a right-sided hemiparesis. On funduscopic examination, papilledema is noted.


Questions



  • Is the patient protecting her airway?
  • Is the patient pregnant?
  • Is there any history of malignancy?
  • Is the patient still seizing?
  • Is there any sign of brain herniation?

Urgent Orders



History and Examination


History



  • Assess for a history of hypercoagulable state, pregnancy/puerperium, malignancy, recent infection, inflammatory disease, dehydration (common with Crohn’s disease), or drug use (particularly oral contraceptives + smoking and hormone replacement therapy).
  • Variability in the clinical presentation of CVT is common. Headache is the most common complaint (90% of patients) and can sometimes resemble migraine with aura. Persistent postlumbar puncture headache should raise concern for CVT, as lumbar puncture can rarely precipitate CVT.
  • Onset can be acute (>48 hours; typically with infectious etiology or during pregnancy/puerperium), subacute, or chronic (<30 days).
  • Most frequent symptoms are headache, seizures (frequently associated with Todd’s paresis), vision loss, encephalopathy, and motor/sensory deficits.
  • Symptoms may fluctuate, which may reflect ongoing thrombosis and endogenous fibrinolysis.

Physical and Neurologic Examination


































Table 7.1 Common Cerebral Sinus Thrombosis Syndromes
Location of CVT Frequency% Presenting Symptoms
Superior sagittal sinus 62 Motor deficits (predominantly affecting lower limbs); possible bilateral deficits Seizures Psychiatric symptoms
Transverse sinus 86 Intracranial hypertension, aphasia when left transverse sinus occluded
Cerebral cortical vein 17 Motor/sensory deficits according to territory Focal seizures
Deep venous system 11 Coma, alteration of mental status Bilateral motor deficits
Cavernous sinus Rare III, IV, V1, V2, or VI nerve palsy Orbital pain, chemosis, proptosis

Frequency column data from: Ferro JM, Canhão P, Stam J, et al. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004;35(3):664–670.


Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005;352(17):1791–1798.


Differential Diagnosis



  1. Cerebral sinus thrombosis. The most common causes are pregnancy and puerperium, oral contraceptives, local infections, and thrombophilia. Most often CVT is multifactorial: more than one cause can be found in 44% of patients. In 15% of patients, no inciting cause is found1 (Table 7.2).




































    Table 7.2 Common Causes of Cerebral Sinus Thrombosis
    Category Causes
    Hypercoagulable state (most common etiology found in 34% of patients) Protein C, S, or antithrombin III deficiency; factor V Leiden mutation; prothrombin gene mutation; antiphospholipid syndrome (lupus anticoagulant/anticardiolipin antibody); nephrotic syndrome; hyperhomocysteinemia
    Infectious (found in 10% of patients) Encephalitis; cerebritis; meningitis; mastoiditis; otitis; sinusitis; mouth, face, and neck infections
    Obstetric Pregnancy and puerperium
    Malignancy CNS tumors with invasion of the venous sinus, hematologic cancers, hypercoagulable state due to malignancy
    Inflammatory diseases Vasculitis, lupus, Wegener’s granulomatosis, inflammatory bowel disease (Crohn’s and ulcerative colitis), Behçet’s disease, thromboangiitis obliterans, sarcoidosis
    Hematologic diseases Polycythemia, thrombocythemia, paroxysmal nocturnal hemoglobinuria
    Drugs Oral contraceptives (particularly when combined with tobacco use or prothrombotic disease), hormone replacement therapy, asparaginase, tamoxifen, steroids, androgens
    Trauma (including iatrogenic) Head injury, lumbar puncture, neurosurgical procedures, jugular catheter occlusion
    Other Dehydration, congenital heart disease, thyroid disease

    Abbreviation: CNS, central nervous system.


  2. Dural arteriovenous fistula (DAVF). DAVFs are characterized by a direct connection between meningeal arteries and dural venous sinuses or meningeal veins. DAVFs can be associated with sinus thrombosis or trauma. They are classified as type I—dural arterial supply drains anterograde into venous sinus; type II—dural arterial supply drains into the venous sinus, but high pressure in the sinus results in both anterograde drainage and retrograde drainage into subarachnoid veins; and type III—dural arterial supply drains retrograde into subarachnoid veins. Subarachnoid veins can form varices and aneurysms and are prone to rupture. Both type II and III DAVF should be treated endovascularly or surgically to prevent hemorrhage.4
  3. Stroke (ischemic or hemorrhagic) can present with acute deficits and seizures. Headache and mental status changes are more common with hemorrhagic stroke (ICH, SAH). Venous infarct and ICH can both be caused by sinus thrombosis.
  4. Brain tumor. A brain tumor can occasionally have strokelike sudden presentation or present with seizure, headache, and/or evidence of elevated ICP.
  5. Encephalitis/cerebritis/abscess. Look for signs of infection, including fever, elevated white blood cell count (WBC), and lumbar puncture results. Sinus thrombosis may accompany these infections.
  6. Benign intracranial hypertension (pseudotumor cerebri). This can present with headache and VI nerve palsy, but encephalopathy, focal deficits, and seizure are atypical and should prompt a more thorough evaluation. Patients with suspected pseudotumor cerebri should undergo MR or CT venography (MRV or CTV) imaging to rule out sinus thrombosis.

Life-Threatening Diagnoses Not to Miss



  • Cerebral sinus thrombosis. Expedient treatment can prevent intracranial hemorrhage.
  • Encephalitis/cerebritis/abscess requires urgent antibiotic administration.
  • Any process that causes dangerous elevations in ICP should be diagnosed and managed immediately.

Diagnostic Evaluation


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Aug 30, 2016 | Posted by in NEUROSURGERY | Comments Off on Cerebral Sinus Thrombosis

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