Thrombosis of the cerebral veins and sinuses is an unusual but important cause of increased intracranial pressure and stroke, especially in the young and middle aged. Pregnant women, especially during the puerperium, and individuals with thrombophilia are a special risk group. What makes the diagnosis difficult is the vast range of symptoms including: headache, nausea, vomiting, blurry vision, reduction of consciousness, aphasia and motor and sensory disturbances. We present four cases which reflect the diverse clinical presentation of the disease.

Sveinsson OA¹, Kjartansson O², Valdimarsson EM³

¹Department of Neurology Karolinska Hospital, Stockholm, Sweden, ²Department of radiology, Landspítali, National University Hospital of Iceland, Reykjavík, Iceland, ³Department of neurology, Landspítali, National University Hospital of Iceland, Reykjavík, Iceland.

 

Table I. Overview of the brains most important veins and sinuses.

Table II. Known causes and risk factors for cerebral sinus venous thrombosis

Genetic thrombophilia

Factor V Leiden mutation

Antithrombin mutation

Protein C and protein S deficiency

Aquired thrombophilia

Hormonal contraception

Pregnancy and puerperium

Elevated homocystein in blood

Nephrotic syndrome

Anti-fospholipid syndrome

Inflammatory diseases

Sarcoidosis

Systemic lupus erythromatosis

Wegeners disease

Bechet syndrome Inflammatory bowel disease

Infections

Infections of the ear, nose and throat area (sinusitis, mastoiditis and middle ear)

Meningitis

Drugs

Hormone replacement therapy

Corticosteroids

Hormonal contraception

Asparginase

Miscellaneous

Anemia

Dehydration

Cancer

Head trauma

Medical procedures in the head and neck area

 

Picture 1a). MRI (FLAIR). Images in the upper row show thrombi in the transverse and superior sagittal sinus (arrows). Images in the lower row show that the thrombi have resolved, six months later.

Picture 1b). Magnetic resonance venography. Images in the upper row show lack of flow in the superior sagittal and transverse sinus on the right side. Images  in the lower row show normal flow, six months later.

Picture 2. Sagittal CT image in midline shows good contrast enhancement in v. cerebri interna, v. galeni, sinus sagittalis inferior and sinus rectus but no enhanchment in sinus sagittalis superior.

Picture 3. a) An unenhanched sagittal CT image in midline shows cerebral vein thrombosis as a hyperdense v. cerebri magna, sinus sagittalis inferior and sinus rectus. b) An enhanched sagittal CT image in midline shows no cerebral vein thrombosis as a contrast filling defect in the v. cerebri magna, sinus sagittalis inferior and sinus rectus.

Picture 4. Shows the largests sinuses of the brain and the frequency of sinus venous thrombosis in each sinus, respectively. Published with permission from The New England Journal of Medicine.