Consistent with the variations in the underlying pathophysiology, clinical presentations also vary significantly, depending of the type and extent of venous thrombosis. This variability makes diagnosis, at times, challenging. There are three large categories of clinical features [11]:

The most commonly utilized neuroimaging studies, non-contrast head CT and MRI, may be normal in CVT patients. Hyperdensity of the dural sinuses may be detected by the meticulous neuroimager but is only 25 % sensitive for this condition [13]. The famous “empty delta” sign is seen on contrast CT or MRI. An axial or coronal image through the torcular Herophili or superior sagittal sinus will show the triangular enhancement of the dural walls, but no contrast enhancement within the lumen due to clot. However, even this sign is only found in 30 % of CVT [14]. For this reason, venography is crucial to rule out CVT. MR venography and CT venography are both effective tools to image the dural sinuses, but are less sensitive for cortical vein and deep vein thrombosis. The sensitivity may be increased with catheter angiography, but must be accompanied by a high index of suspicion on the part of the angiographer. Catheter angiography also has the advantage of providing a dynamic assessment of venous flow/flow restriction [15] (Fig. 8.1).

Once CVT has been diagnosed, the first line of therapy should include aggressive hydration and anticoagulation. The evidence base for anticoagulation in the setting of CVT is not deep, but the two published, prospective, randomized trials point to its benefit [16, 17]. A meta-analysis showed over 50 % reduction in death and disability compared to placebo [18]. It is important to emphasize that anticoagulation appears effective even in the setting of intracerebral hemorrhage (ICH).

Both unfractionated (UFH) and low molecular weight heparin (LMWH) have been used to achieve anticoagulation in the acute setting [16, 17]. UFH has the advantage of rapid normalization of coagulation parameters once the intravenous drip is turned off LMWH, on the other hand, maintains a more consistent therapeutic effect. In a mildly affected patient (i.e., headache and papilledema), LMWH may be relied upon. However, in the unstable or deteriorating patient, UFH should be utilized. This is especially true in the setting of ICH where hematoma expansion would necessitate prompt cessation of the anticoagulant effect. Although clinical trial evidence is lacking, it is common practice to transition from UFH or LMWH to oral anticoagulation once clinical stabilization/improvement is noted. The duration of oral agent use can be tailored to the particular patient. Typically, it is maintained until clot resolution is visualized on follow-up imaging (3–6 months). In patients with ongoing risk (e.g., a hypercoagulable condition), lifetime treatment may be required [19].

Seizure prophylaxis in CVT patients is somewhat controversial, but should be considered when significant cortical edema or hemorrhage has occurred [20]. Once seizures have occurred, however, initiation of an antiepileptic agent (AED) is universally recommended [21]. Status epilepticus is a not uncommon and particularly deadly manifestation of CVT and requires the use of intravenous agents. Fosphenytoin and levetiracetam are effective agents with good side effect profiles. It is reasonable to continue oral AED treatment for up to 1 year to prevent delayed seizure activity.

Surgical therapies are also effective and at times life saving in CVT. Extraventricular drain (EVD) placement is particularly effective in patients with a predominant involvement of the dural sinuses and impaired CSF drainage due to venous hypertension and obstruction of the arachnoid granulations by clot. In patients with significant preexisting parenchymal injury and/or a herniation syndrome, however, EVD placement is generally not helpful [22]. However, decompressive surgery (i.e., hemicraniectomy) does appear to be effective in preventing death under these dire circumstances. Such surgeries may also reduce long-term disability [23, 24].