Teaching Case

A 60-year-old female patient with code stroke and headache.  No previous history. On neurological exam, she was alert, with left-sided facial droop and slurred speech. National Institutes of Health Stroke Scale (NIHSS) score was 7 points.  TPA was not given. Glasgow Coma Scale/Score (GCS) was ~10-11.

Non-contrasted computed tomography shows hyperdense behaviour of right-internal cerebral veins, right basal vein of Rosenthal, vein of Galen, inferior sagittal sinus and straight sinus. Hypoattenuation and oedema was recognisable in the right lenticulate nuclei, internal capsule and thalamus (Figs. 1a and 1b). CT post-contrast images outline venous filling without haemorrhagic conversion. (Fig. 1c). CT perfusion showed prolongation of perfusion times. (Fig. 1d).

Cerebral digital subtraction angiography showed occlusion of the right basal vein of Rosenthal and slow flow in the left vein of Rosenthal, vein of Galen, and internal cerebral veins. (Figs. 2a and 2b).  After TPA and mechanical thrombectomy (Fig. 3a), it was recanalisation of the vein of Galen and straight sinus. (3b and 3c).

Immediate follow-up CT showed intracranial parenchymal and intraventricular haemorrhage (Figs. 4a and 4b). CT four hours later showed diffuse cerebral oedema, multicompartment haemorrhages, subfalcine, transtentorial and uncal herniations. Brain death six hours after haemorrhage due to herniation (Figs. 4c and 4d).

Cerebral venous thrombosis (CVT) is an uncommon disorder, accounting for 0.5% to 1.0% of all strokes.[1,2]. The patterns of cerebral venous drainage stroke do not correspond to cerebral arterial territories. [2,7] The cerebral venous system is divided in superficial and deep. The superficial is composed of haemispheric cortical veins which drain into the dural sinuses. The deep system includes the white matter medullary veins and septal veins, internal cerebral veins, basal veins of Rosenthal, which drain into the great vein of Galen and then join the superficial venous system through the straight sinus into the torcular confluence of sinuses at the inner occipital protuberance. From the torcula, venous outflow from both superficial and deep venous systems flow together through the transverse and sigmoid dural sinuses into the corresponding internal jugular veins. [2,6]

The pathophysiology is complex and may result in thalamic and basal ganglia venous congestion from obstruction of the venous outflow leading to cerebral oedema with ischaemia and venous infarction. [4,7]

CVT is a multicausal disease, resulting from the interaction of several risk factors. The most common are genetic or acquired prothrombotic disorders. [2,7] The clinical presentation is highly variable. Intracranial hypertension syndrome is the most frequent, followed by seizures, focal neurologic deficits and/or encephalopathy. Headache is usually the initial and most frequent symptom. [3]

The wide variety of symptoms expressed at time of initial presentation confound and delay the diagnosis, with symptom onset to diagnosis delays of up to seven days. [3,6]

Diagnosis can be confirmed through the demonstration of thrombi by using MRI with MR venography, CT with CT venography (CTV), and digital subtraction angiography (DSA), three imaging techniques optimally suited for the diagnosis. CTV is the most often used, although MRI is still superior to CT for detecting isolated cortical venous thromboses and parenchymal damage. Cerebral DSA has higher spatial resolution but is reserved for cases with inconclusive or contradictory imaging findings, however, it is used selectively due to its invasive nature and risk of neurological complications. [3-5]

CVT prognosis is usually good when treated timely but can result in death or permanent disability. [2,5]

CVT treatment consists in parenteral heparin followed by oral anticoagulation. [2,7] Numerous observational studies and treatment trials to be soon reported in the near future should enlighten several uncertain issues, such as safety of heparin in the acute phase, duration of anticoagulation, use of endovascular thrombolysis/thrombectomy, and indication of decompressive surgery. [6,7]

Written patient consent for this case was waived by the Editorial Board. Patient data may have been modified to ensure patient anonymity.