Superior sagittal sinus thrombosis and myeloproliferative disorder
VIKELIS M., XIFARAS M., SALI D., BASTA A., KOLOVOU A., TSAKIRIS A., GEKAS G.

Cerebral venous sinus thrombosis (CVST) is an uncommon condition which over the past few years has been diagnosed more frequently due to greater awareness and the availability of better diagnostic techniques. CVST presents with a wide spectrum of symptoms and signs and recognition remains a challenge for the clinician. Headache is the presenting symptom in most cases. Focal deficits such as hemiparesis and hemisensory disturbance, seizures, impairment of level of consciousness and papilledema may also be part of the clinical picture. All ages can be affected and the situation is slightly more common in women due to pregnancy and oral contraceptive use. The onset may be acute, subacute or insidious, most patients presenting with symptoms which have evolved over days or weeks. Numerous conditions can cause or predispose to CVST. A principal distinction can be made between infective and non-infective causes. Infective causes include penetrating head injury, intracranial infection, regional infection, sepsis and systemic infection. Amongst the non-infective causes, systemic conditions such as connective tissue diseases, other granulomatous or inflammatory disorders and malignancies are most common. Other non-infective causes include head injury, neurosurgery, stroke and haemorrhage, hormonal and endocrine causes, red blood cell disorders, thrombocythaemia, coagulation disorders (acquired or hereditary), surgery with immobilisation and others. Treatment includes antibiotics in patients with septic thrombosis, acetazolamid for lowering of intracranial pressure and heparin or thrombolytic therapy. The prognosis is guarded in patients with septic thrombosis but less grave in patients with non septic thrombosis. Because of the variable clinical signs, many cases remain clinically undetected. The occurrence of cerebral sinus thrombosis and myeloproliferative disorder has been rarely reported.

We report the case of a 29-year-old man with a six month history of headache and a two-week history of blurred vision. He had free past personal and family medical history. The headache had subacute onset and progressive course, resulting in a frequency of 15-20 headache attacks per month two months after onset. Attacks usually lasted a few hours, pain was located in the occipital area and was aggravated by bending forward. There were no accompanying symptoms as photophobia, phonophobia, nausea or vomiting at any time. Attacks did not awake the patient during the night. Two weeks before his admission the patient noticed visual disturbances in both his eyes. The symptoms had a progressive course and a few days later the patient visited an ophthalmologist who diagnosed a bilateral papilledema and referred the patient to our department. At admission, his physical and neurological examination was normal except for marked bilateral papilledema. A MRI brain scan was without abnormal findings. A lumbar puncture showed significantly raised opening pressure (>45 cm H2O column). A digital subtraction angiography followed revealing slow and delayed venous drainage to the superior sagittal sinus, which presented multiple filling defects throughout its length. These findings suggested superior sagittal sinous thrombosis with partial recanalization. The patient was started treatment with continuous heparin infusion for ten days and with oral warfarin thereafter and the investigation was directed towards demonstrating the underlying cause of the thrombosis. Laboratory findings suggested an underlying haematological disorder with increased hematocrit (49-50%), white blood cell count (10-16 Κ/uL) and platelets count (400-500 K/uL). Proteins S and C activities and anti-thrombin III levels were within the normal range. Lupus anticoagulant and anti-cardiolipin antibody were negative. A bone marrow biopsy confirmed the diagnosis of a myeloproliferative disorder. The patient was referred to a haematology department for further treatment.

Key words: Cerebral sinus thrombosis, myeloproliferative disorder, raised intracranial pressure.