Protein C, S, or antithrombin III deficiency; factor V Leiden mutation; prothrombin gene mutation; antiphospholipid syndrome (lupus anticoagulant/anticardiolipin antibody); nephrotic syndrome; hyperhomocysteinemia
Encephalitis; cerebritis; meningitis; mastoiditis; otitis; sinusitis; mouth, face, and neck infections
Pregnancy and puerperium (about 12 cases per 100,000 deliveries)
Central nervous system tumors with invasion of the venous sinus, hematologic cancers, hypercoagulable state due to malignancy
Vasculitis, lupus, Wegener’s granulomatosis, inflammatory bowel disease (Crohn’s and ulcerative colitis), Behcet’s disease, thromboangiitis obliterans, sarcoidosis
Polycythemia, thrombocythemia, paroxysmal nocturnal hemoglobinuria
Oral contraceptives (especially third generation contraceptives), hormone replacement therapy, asparaginase, tamoxifen, steroids, androgens
Head injury, lumbar puncture, neurosurgical procedures, jugular catheter occlusion
Dehydration, congenital heart disease, thyroid disease
9.2 Case Presentation
A 36 year old female with no significant past medical history was brought to the emergency department after suffering a seizure in a movie theater. She had complained of onset of a diffuse and slowly worsening headache earlier in the day. She has no prior history of seizures and is a regular one pack per day cigarette smoker. She takes no over the counter medications and is on oral contraceptive therapy for prevention of pregnancy. She denies prior history of thrombotic events and denies a family history of clotting disorders. Upon initial assessment, she was post-ictal, with improving alertness. She exhibited some difficulty with language expression, but did not demonstrate any focal motor or sensory abnormalities. A non-contrast head CT (Fig. 9.1) showed a small amount of subarachnoid blood in the left parieto-occipital cortex. Further vascular imaging revealed thrombosis of her left transverse venous sinus and associated distal jugular vein (Fig. 9.2). She was diagnosed with a cerebral venous thrombosis and placed on intravenous unfractionated heparin. Her anticoagulation was transitioned to oral warfarin and she was eventually discharged on an anti-seizure medication. At 3 month follow up, the patient reported no further clinical events and repeat vascular imaging showed recanalization of her thrombosed transverse sinus. The patient was successful in smoking regimen cessation and was placed on an alternative contraception. Her anticoagulation and seizure prophylaxis were discontinued.
Non-contrast head CT of a 36 year old female presenting with headache, seizure, and language difficulty. Subarachnoid hemorrhage is present within the left parieto-occipital lobe (black arrow) and small amounts of edema are present in a non-arterial distribution in the temporo-partietal lobe (white arrow)
Magnetic resonance venogram (MRV) of 36 year old female presenting with headache, seizure, and language impairment. The MRV shows absence of the L transverse sinus consistant with occlusion (white arrow)
9.3 Initial Evaluation
When taking a history on patients with CVT, it is important to assess for hypercoagulability, pregnancy, recent infection, dehydration, drug use (especially oral contraceptives or hormone replacement therapy with a smoking history), malignancy, or an inflammatory disease. Patients can present acutely with an onset of less than 48 h (28%), subacutely (42%), or chronically at greater than 30 days (30%) with symptoms . An acute onset is often associated with pregnancy or infectious etiology. Although a patient may present with a variety of symptoms, 90% of the time they will have a chief complaint of a headache . A headache is the only complaint for patients 14% of the time . Other symptoms include vision loss, seizures (often associated with Todd’s paralysis), motor and/or sensory deficits, and encephalopathy. Some patient may experience fluctuating symptoms secondary to incomplete or ongoing thrombus.
9.3.2 Neurological Examination
A patient’s initial presentation can vary from decreased level of alertness to coma. Symptoms vary depending on the location of injury as well. A superior sagittal sinus thrombus occurs approximately 62% of the time can elicit motor symptoms primarily in the lower extremities and can be bilateral or alternating (Fig. 9.3). Patients can also experience seizures or psychiatric symptoms. Thromboses in the transverse sinus occurs most frequently (86%) and can be complicated with symptoms of intracranial hypertension. If the left transverse sinus is occluded, aphasia can be present as well. Motor and/or sensory deficits and focal seizures occur with cerebral cortical vein thromboses in 17% of patients. A thrombus of the deep venous system occurs approximately 11% of the time and can lead to coma and changes in mental status as well as bilateral motor deficits. Cavernous sinus thromboses are rare but cause cranial nerve III, IV, V1, V2, or VI palsies, orbital pain, chemosis, or proptosis . The cavernous sinus is the only anatomic location where a single lesion can produce all of these neuropathies. Therefore, this unique clinical presentation should strongly raise suspicious of a vascular or mass lesion in the cavernous sinus.
When performing a fundoscopic exam, papilledema may be present. The patient will experience an enlarging blind spot with ensuing color desaturation. A late sign includes vision loss. Other vision changes can include hemianopsia as seen with a thrombosis of the vein of Labbé.
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