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Post gravidic superior sagittal sinus thrombosis with elevated levels of homocystinemia.

Cerebral ischemia in the young (less than 45 years) is an uncommon finding, its ethiology remaining undefined in up to half of cases even if all the common diagnostic paths have been followed. Pregnancy and puerperium are characterised by an increased risk of ischemic stroke and intracranial venous thrombosis. We present a case of a young woman with post gravidic thrombosis of the superior sagittal sinus, occurred 10 days after eutocic delivery. She presented elevated plasma levels of homocystinemia, a sulphur containing amino acid that induce complex changes within the blood vessel wall, acting with an oxidative stress and with a reduction of nitric oxide availability. This clinical case suggests that a complete screening for thrombophilic risk factors should be an integral part in the diagnostic path of central venous thrombosis in young patients also in the puerperium, because it allows to identify thrombophilic risk factors requiring specific treatment.

Adult↗

Recovery from primary deep cerebral venous sinus thrombosis with recanalisation.

A 50-year-old woman with idiopathic deep cerebral sinus and vein thrombosis (DCVT) had cerebellar disturbance prior to impaired consciousness. CT and MRI revealed haemorrhagic infarction in the cerebellum and signal changes suggesting infarction in the thalamus and basal ganglia bilaterally. The straight sinus and internal cerebral vein (ICV) were dense on CT. On angiography, the vein of Galen (VG) and straight sinus were not seen. Following clinical recovery, CT and MRI became normal, and angiography showed recanalization of the VG and ICV. The relationship between cerebellar infarction and DCVT, and signal changes on CT and MRI are discussed.

Basal Ganglia↗

Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome.

Neuroimaging and management advances require review of indications for excluding cerebral venous sinus (sinovenous) thrombosis (CSVT) in children. Our goals were to examine (i) clinical presentations of CSVT, (ii) prothrombotic risk factors and other predisposing events, (iii) clinical and radiological features of brain lesions in CSVT compared with arterial stroke, and (iv) predictors of outcome. We studied 42 children with CSVT from five European paediatric neurology stroke registries. Patients aged from 3 weeks to 13 (median 5.75) years (27 boys; 64%) presented with lethargy, anorexia, headache, vomiting, seizures, focal signs or coma and with CSVT on neuroimaging. Seventeen had prior chronic conditions; of the 25 previously well patients, 23 had recent infections, eight became dehydrated and six had both. Two children had a history compatible with prior CSVT. Anaemia and/or microcytosis (21 probable iron deficiency, five haemolytic, including two with sickle cell disease and one with beta-thalassaemia) was as common (62%) as prothrombotic disorder (13/21 screened). High factor VIII and homozygosity for the thermolabile methylene tetrahydrofolate reductase polymorphism were the commonest prothrombotic disorders. The superficial venous system was involved in 32 patients, the deep in six, and both in four. Data on the 13 children with bland infarction and the 12 with haemorrhage in the context of CSVT were compared with those from 88 children with ischaemic (AIS) and 24 with haemorrhagic (AHS) arterial stroke. In multiple logistic regression, iron deficiency, parietal infarction and lack of caudate involvement independently predicted CSVT rather than arterial disease. Five patients died, three acutely, one after recurrence and one after 6 months being quadriparetic and blind. Follow-up ranged from 0.5 to 10 (median 1) years. Twenty-six patients (62%) had sequelae: pseudotumour cerebri in 12 and cognitive and/or behavioural disabilities in 14, associated with epilepsy in three, hemiparesis in two and visual problems in two. Eighteen patients, including six with haemorrhage, were anticoagulated. Older age [odds ratio (OR) 1.54, 95% confidence limits (CI) 1.12, 2.13, P = 0.008], lack of parenchymal abnormality (OR 0.17, 95% CI 0.02, 1.56, P = 0.1), anticoagulation (OR 24.2, 95% CI 1.96, 299) and lateral and/or sigmoid sinus involvement (OR 16.2, 95% CI 1.62, 161, P = 0.02) were independent predictors of good cognitive outcome, although the last predicted pseudotumour cerebri. Death was associated with coma at presentation. Of 19 patients with follow-up magnetic resonance (MR) venography, three had persistent occlusion, associated with anaemia and longer prodrome. A low threshold for CT or MR venography in children with acute neurological symptoms is essential. Nutritional deficiencies may be modifiable risk factors. A paediatric anticoagulation trial may be required, after the natural history has been further established from registries of cases with and without treatment.

Adolescent↗