Cerebral Venous Thrombosis

Occlusion of the cortical and subcortical veins causes focal neurologic symptoms and signs, including neuro-ophthalmic findings. Most commonly affected are the cavernous sinus, lateral (transverse) sinus, and superior sagittal sinus, and each produces a distinct clinical syndrome. In pregnancy, the lateral and superior sagittal sinuses are more commonly affected, and the thrombosis may present with headaches and papilledema and may simulate IIH. Thrombosis of the deep veins may cause infarction of the thalamus or basal ganglia, and death can occur in 3%–15% of cases.

Saposnik G, Barinagarrementeria F, Brown RD Jr, et al; American Heart Association Stroke Council and the Council on Epidemiology and Prevention. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011;42(4):1158–1192.

Cavernous sinus thrombosis

Cavernous sinus thrombosis (CST) in the septic form results from an infection of the face, sphenoid or ethmoidal sinuses, or oral cavity. Otitis media or orbital cellulitis is a rare cause. Patients experience headache, nausea, vomiting, and somnolence. There may also be fever, chills, tachycardia, evidence of meningitis, or generalized sepsis. Ocular signs from anterior infection (facial, dental, orbital) are initially unilateral but frequently become bilateral. They include orbital congestion, lacrimation, conjunctival edema, eyelid swelling, ptosis, proptosis, and ophthalmoplegia. Sixth nerve palsy is the most consistent early neurologic sign. Corneal anesthesia, facial numbness, Horner syndrome, and venous stasis retinopathy can occur. Septic CST is a medical emergency and carries a high mortality rate if not recognized promptly and treatment not initiated immediately. Treatment includes the administration of antibiotics, anticoagulants, or corticosteroids and surgery.

The signs and symptoms of aseptic CST resemble those of septic CST, but clinical or laboratory examination shows no evidence of infection. Pain around the eye is common, but orbital congestion is typically less severe than with septic CST. Anticoagulation or antiplatelet therapy is often used.

Lateral (transverse) sinus thrombosis

Lateral sinus thrombosis may be septic or spontaneous (see Chapter 2, Fig 2-13). With the widespread use of antibiotics, septic thrombosis has become rare, but it may result from otitis media.

Patients have features of systemic infection as well as neck pain, tenderness of the ipsilateral jugular vein, retroauricular edema, and sometimes, facial weakness. Severe facial pain also may occur and when accompanied by sixth nerve palsy is called Gradenigo syndrome. The IIH-like syndrome caused by lateral sinus thrombosis was originally called otitic hydrocephalus. Lateral sinus thrombosis is much more likely to be spontaneous and produce an IIH-like syndrome with increased intracranial pressure. The most common ophthalmic signs are papilledema and sixth nerve palsy.

Superior sagittal sinus thrombosis

The most commonly involved cerebral venous sinus is the superior sagittal sinus (SSS), and symptoms depend on the extent and location of the occlusion. With thrombosis of the anterior third of the sinus, symptoms are mild or absent. Posterior SSS thrombosis may produce a clinical picture similar to that of IIH, with headaches and papilledema. It is a diagnosis to consider in atypical IIH patients, such as slim women and men. Seizures and motor deficits may occur.