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Cerebral venous sinus thrombosis is a disorder with a unique pathophysiology which needs to be described. A Medline search of all articles detailing pathophysiology of CVST was done, using keywords: cerebral venous thrombosis and pathophysiology. In addition, major texts were reviewed for additional references. The pathophysiology of CVST depends on two interconnected events, local signs due to venous infarct, e.g., hemiparesis and global signs due to raised ICP from an obstructed venous system--papilloedema and isolated intracranial hypertension being one of them. Pathophysiology of CVST is diverse and makes it easier to understand the diversity of clinical presentations.
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PMID:Pathophysiology of cerebral venous thrombosis--an overview. 1718 77

Cerebral venous sinus thrombosis (CVT) can affect all age groups, particularly women of childbearing age. Overall prognosis for survival and functional independence is better than it was believed. Mortality usually ranges from 6-15% and transtentorial herniation is the major cause of death. Approximately 80% of patients are functionally independent in the long term. Altered mental status and cerebral haemorrhage at presentation are the strongest predictors of death and disability. Patients with CVT related to pregnancy and puerperium generally do better than patients with other causes. Septic CVT carries a worse prognosis than aseptic CVT and of the latter, patients with syndrome of isolated intracranial hypertension have a better prognosis than those with focal deficits or encephalopathy. Anticoagulation is believed to improve outcome in CVT although robust data are lacking. Epilepsy, headaches, visual loss, pyramidal deficits and cognitive impairment are some of the long term sequelae. The risk of recurrence of CVT is low, particularly after the first 12 months of the first episode.
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PMID:Prognostic indicators in cerebral venous sinus thrombosis. 1718 90

Cerebral venous sinus thrombosis (CVST) is a rare clinicopathological entity. The incidence of CVST in children and neonates has been reported to be as high as 7 cases per million people, whereas in adults the incidence is 3-4 cases per million. The predisposing factors to this condition are mainly genetic and acquired prothrombotic states and infection. The clinical picture of CVST is nonspecific, highly variable, and can mimic several other clinical conditions. Diagnosis of CVST is established with the implementation of neuroimaging studies, especially MR imaging and venography. Identification and elimination of the underlying cause, anticoagulation, proper management of intracranial hypertension, and anticonvulsant prophylaxis constitute cornerstones of CVST treatment. Newer treatment strategies such as endovascular thrombolysis and decompressive craniectomy have been recently used in the treatment of patients with CVST with variable success rates. Further clinical research must be performed to delineate the exact role of these newer treatments in the management of severe cases of CVST. The recent advances in the diagnosis and treatment of patients with CVST have significantly lowered the associated mortality and morbidity and have improved the outcome of these patients.
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PMID:Cerebral venous sinus thrombosis: review of the demographics, pathophysiology, current diagnosis, and treatment. 1987 94

Cerebral venous sinus thrombosis in children is increasingly recognized as diagnostic tools and clinical awareness has improved. It is a multifactorial disease where prothrombotic risk factors and predisposing clinical conditions usually in combination constitute the underlying etiology. Clinical features range from headache, seizures to comatose state. Although symptomatic treatment involving control of infections, seizures and intracranial hypertension is uniform, use of anticoagulation and local thrombolytic therapy is still controversial. Morbidity and mortality can be significant and long-term neurological sequelae include developmental delay, sensorimotor and visual deficits and epilepsy.
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PMID:Caring for cerebral venous sinus thrombosis in children. 2188 32

Cerebral venous sinus thrombosis (CVST) and idiopathic intracranial hypertension (IIH) are common considerations in young patients presenting with isolated intracranial hypertension. We report two patients with progressive visual failure and polyradiculopathy with areflexic quadriparesis, secondary to raised intracranial pressure (ICP). Both underwent cerebrospinal fluid diversion with complete recovery. Such a fulminant presentation of raised ICP with an excellent outcome has rarely been reported in the literature.
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PMID:Intracranial hypertension with polyradiculopathy--early CSF diversion to optimize neurological recovery. 2244 58

Spontaneous intracranial hypotension typically results from spontaneous cerebrospinal fluid (CSF) leak, often at spine level and only rarely from skull base. Once considered rare, it is now diagnosed far more commonly than before and is recognized as an important cause of headaches. CSF leak leads to loss of CSF volume. Considering that the skull is a rigid noncollapsible container, loss of CSF volume is typically compensated by subdural fluid collections and by increase in intracranial venous blood which, in turn, causes pachymeningeal thickening, enlarged pituitary, and engorgement of cerebral venous sinuses on magnetic resonance imaging (MRI). Another consequence of CSF hypovolemia is sinking of the brain, with descent of the cerebellar tonsils and brainstem as well as crowding of the posterior fossa noted on head MRI. The clinical consequences of these changes include headaches that are often but not always orthostatic, nausea, occasional emesis, neck and interscapular pain, cochleovestibular manifestations, cranial nerve palsies, and several other manifestations attributed to pressure upon or stretching of the cranial nerves or brain or brainstem structures. CSF lymphocytic pleocytosis or increase in CSF protein concentration is not uncommon. CSF opening pressure is often low but can be within normal limits. Stigmata of disorders of connective tissue matrix are seen in some of the patients. An epidural blood patch, once or more, targeted or distant, at one site or bilevel, has emerged as the treatment of choice for those who have failed the conservative measures. Epidural injection of fibrin glue of both blood and fibrin glue can be considered in selected cases. Surgery to stop the leak is considered when the exact site of the leak has been determined by neurodiagnostic studies and when less invasive measures have failed. Subdural hematomas sometimes complicate the CSF leaks; a rebound intracranial hypertension after successful treatment of a leak is not rare. Cerebral venous sinus thrombosis as a complication is fortunately less common, and superficial siderosis and bibrachial amyotrophy are rare. Short-term recurrences are not uncommon, and long-term recurrences are not rare.
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PMID:Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. 2380 30

Cerebral venous sinus thrombosis (CVST) may occur following head trauma and contribute to intracranial hypertension that mandates immediate action. Anticoagulant therapy is the first line of treatment in CVST but may not be applicable in patients with head trauma. Here, we report on the treatment of 2 patients with CVST. In 1 patient, there was an attempt to perform thrombectomy and thrombolysis, and eventually a decompressive craniectomy was performed. In this patient, there was an excellent outcome. In the other patient, an immediate decompressive craniectomy was performed that did not improve the outcome.
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PMID:Intracranial Hypertension due to Cerebral Venous Sinus Thrombosis following Head Trauma: A Report of Two Cases. 2416 73

Cerebral venous sinus thrombosis (CVST) is characterized by formation of widespread thrombus within the cerebral venous sinus system. CVST can cause venous hypertension, venous infarcts, hemorrhage and seizures. It is managed in most cases with systemic anticoagulation through the use of heparin to resolve the thrombus. Patients that demonstrate clinical deterioration while on heparin are often treated with endovascular strategies to recanalize the sinuses. We present the case of a patient with widespread CVST, involving his superior sagittal sinuses and bilateral transverse sigmoid sinuses, who was treated with a combination of endovascular therapies. The video can be found here: http://youtu.be/w3wAGlT7h8c .
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PMID:Endovascular management of cerebral venous sinus thrombosis. 2498 20

Cerebral venous sinus thrombosis after mild head trauma without skull fracture or intracranial hematoma is exceptionally rare. We describe an unusual case of progressive intracranial hypertension due to superior sagittal sinus thrombosis following mild head trauma. A 17-year-old boy presented with nape pain a day after a head blow during a gymnastics competition (backward double somersault). On admission, he showed no neurological deficit. CT scans revealed no skull fractures, and there were no abnormalities in the brain parenchyma. However, his headache worsened day-by-day and he had begun to vomit. Lumbar puncture was performed on Day 6, and the opening pressure was 40 cm of water. After tapping 20 mL, he felt better and the headache diminished for a few hours. MR venography performed on Day 8 revealed severe flow disturbance in the posterior third of the superior sagittal sinus with multiple venous collaterals. Because of the beneficial effects of lumbar puncture, we decided to manage his symptoms of intracranial hypertension conservatively with repeated lumbar puncture and administration of glycerol. After 7 days of conservative treatment, his symptoms resolved completely, and he was discharged from the hospital. Follow-up MR venography performed on Day 55 showed complete recanalization of the superior sagittal sinus. The exact mechanism of sinus thrombosis in this case is not clear, but we speculate that endothelial damage caused by shearing stress because of strong rotational acceleration or direct impact to the superior sagittal sinus wall may have initiated thrombus formation.
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PMID:[Progressive Intracranial Hypertension due to Superior Sagittal Sinus Thrombosis Following Mild Head Trauma: A Case Report]. 2613 27

Intraventricular hemorrhage (IVH) may occur as an isolated event from primary ventricular bleeding or as a complication of brain hemorrhage from another etiology. It is associated with high mortality and morbidity. The underlying risk factors include hypertension and aneurysms, among others. However, not all the exact etiologies are known. In this study, a case of a 24-year-old man who suffered from a headache and a decline in memory has been reported. A brain computed tomography scan suggested the diagnosis of spontaneous intraventricular hemorrhage. However, brain magnetic resonance imaging, magnetic resonance venography, and other tests eventually confirmed cerebral venous sinus thrombosis.Cerebral venous sinus thrombosis may be one of the causes of intraventricular hemorrhage and should be considered for unexplained intraventricular hemorrhage.
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PMID:Intraventricular hemorrhage caused by intracranial venous sinus thrombosis: Case report. 2742 84


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