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[Superior sagittal sinus thrombosis is the newborn--diagnosis by computerized tomography].

The case of a newborn with a superior sagittal sinus thrombosis is reported. The clinical symptoms are difficult to differentiate from other cerebral conditions in the neonatal period. Sudden appearance of signs of raised intracranial pressure, however, is characteristic. Main predisposing factors are infection, dehydration and polycythemia. Before computed tomography (CT) became available, the diagnosis could be made only by means of invasive examination techniques. Today CT, in many cases, allows a rapid diagnosis of this condition.

Cerebrospinal Fluid Shunts↗

Systemic lupus erythematosus complicated with cerebral venous sinus thrombosis : a report of two cases.

A rare manifestation of systemic lupus erythematosus (SLE) is cerebral venous sinus thrombosis (CVST), in which early diagnosis and aggressive therapy are of prime importance for favorable outcome. The pathogenesis of CVST is largely unknown, but it is thought to be caused by cerebral vasculitis, antiphospholipid antibodies or other conditions associated with enhanced coagulability. We describe two cases of SLE with CVST which were not associated with antiphospholipid antibodies. Both cases were treated with immunosuppressants (intravenous methylprednisolone and cyclophosphamide pulse therapy) and anticoagulant drugs (heparin and subsequent maintenance therapy with warfarin). There was a marked improvement of neurologic symptoms with the disappearance of thrombus in a follow-up MRI. The possibility of CVST should be considered in any patients with SLE who show neuropsychiatric manifestations.

Adult↗

POEMS syndrome with venous sinus thrombosis and visual failure: a case report.

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome is a multisystem disorder associated with plasma cell dyscrasia. Other clinical signs include clubbing of the fingers, edema, papilledema etc. Although papilledema and increased intracranial pressure are common features, their causes or pathophysiology have been uncertain. The authors report here a 16-year-old Thai patient with these features who also suffered from venous sinus thrombosis and visual failure which have never been reported before. The former is considered to be one of the possible causes of the intracranial hypertension and visual failure. MRI of the brain and optic nerve revealed enhancement and swelling of the optic nerve sheaths and optic discs. MRV findings were compatible with chronic veno-occlusive disease. Bone marrow aspiration and biopsy demonstrated an increase of aggregates of intermediate and mature plasma cells. The CSF pressure was markedly elevated. His clinical condition continued to deteriorate and he expired 3 years and 5 months from the onset of his illness. Although, overproduction of vascular endothelial growth factor has been reported and is being considered to be the possible cause of vascular hyperpermeability, the chronic venous sinus thrombosis may play an important role in the pathogenesis of intracranial hypertension and visual failure.

Adolescent↗

Treatment of dural sinus thrombosis by urokinase infusion.

PURPOSE: To gain a preliminary understanding of the role of thrombolytic therapy for the thrombosed dural sinus, we retrospectively reviewed our initial experience. METHODS: Seven patients, ages 25 to 71, who presented with symptomatic dural sinus thrombosis and who failed a trial of medical therapy were treated with direct infusion of urokinase into the thrombosed sinus. Patients received urokinase doses ranging from 20,000 to 150,000 U/h with a mean infusion time of 163 hours (range 88 to 244 hours). RESULTS: Patency of the affected dural sinus was achieved with antegrade flow in all patients. Six patients either improved neurologically over their prethrombolysis state or were healthy after thrombolysis; one of them required angioplasty. The other patient improved after surgical repair of a residual dural arteriovenous fistula. The only complications were an infected femoral access site which resolved after treatment with antibiotics and hematuria which cleared after discontinuation of anticoagulation. CONCLUSIONS: Thrombolysis of the thrombosed dural sinus shows promise as a safe and efficacious treatment. The results of this study should provide the impetus for further research.

Adult↗

Rhinosino-orbital mucormycosis causing cavernous sinus thrombosis and internal carotid artery occlusion: radiological findings in a patient with treatment failure.

The authors describe a case of rhinosino-orbital mucormycosis with cavernous sinus thrombosis in association with internal carotid artery occlusion diagnosed by use of computerized tomography (CT) and magnetic resonance imaging (MRI). Cranial CT is a useful imaging tool in the diagnosis of rhinosinal invasive fungal disease and MRI offers excellent aid in the detection of intracranial extension. Early diagnosis and rapid institution of surgical debridement and antifungal therapy is the rule of thumb in treating this disorder. In our patient, surgically inaccessible bone lesion and involvement of the central nervous system are taken as major causes for his grave outcome. In addition, failure to advance appropriate amphotericin B dosage may also make the infectious process uncontrollable in this patient.

Carotid Artery Thrombosis↗

Moyamoya disease and sagittal sinus thrombosis in a child with Down's syndrome.

A girl with Down's syndrome, moyamoya disease and sagittal sinus thrombosis is described. She was diagnosed after acute neurological deterioration by MRI and angiography. Recombinant tissue plasminogen activator (r-TPA) was injected locally to recanalise the thrombus. The patient's condition significantly improved and she was discharged. After 2 years of follow-up the child remains asymptomatic. Moyamoya syndrome and cerebral venous thrombosis should not be overlooked as a cause of acute neurological deterioration in a child with Down's syndrome. MRA appears to be a safe and accurate alternative to traditional angiography for the diagnosis of moyamoya disease. Local fibrinolysis with r-TPA is the treatment of choice for cerebral venous thrombosis due to its safety and efficacy.

Child, Preschool↗

Risk and predictors of early epileptic seizures in acute cerebral venous and sinus thrombosis.

We assessed the risk and determined predictors of early epileptic seizures (ES) in patients with acute cerebral venous and sinus thrombosis (CVST). A prospective series of 194 consecutive patients with acute CVST admitted to neurological wards in two German university hospitals was analysed for frequency of ES and in-hospital mortality. Demographic, clinical and radiological characteristics during the acute stage were retrospectively analysed for significant association with ES in univariate and multivariate analyses. During the acute stage, 19 patients (9.8%) died. Early symptomatic seizures were found in 86 patients (44.3%). Status epilepticus occurred in 11 patients (12.8%) of whom four died. Amongst patients with epileptic seizures, mortality was three times higher in those with status than in those without (36.4% and 12%, respectively). In multivariate logistic regression analysis, motor deficit [odds ratio (OR) 5.8; 95% CI 2.98-11.42; P < 0.001], intracranial haemorrhage (OR 2.8; 95% CI 1.46-5.56; P = 0.002) and cortical vein thrombosis (OR 2.9; 95% CI 1.43-5.96; P = 0.003) were independent predictors of early epileptic seizures. Status epilepticus was an important source of morbidity and early mortality in patients with CVST in this study. Patients with focal motor deficits, cortical vein thrombosis and intracranial haemorrhage carried the highest risk for ES. Prophylactic antiepileptic treatment may be an option for these patients.

Adolescent↗

Delay in hospital admission of patients with cerebral vein and dural sinus thrombosis.

Factors influencing early hospital admission have been described for several stroke types but not for cerebral vein and dural sinus thrombosis (CVT). CVT is more difficult to diagnose than arterial stroke; delay in hospital admission may postpone CVT treatment. The purposes of this study were: (1) to describe the delay between the onset of symptoms and hospital admission of patients with CVT, and (2) to identify the variables that influence that delay. We registered the interval (days) between the onset of symptoms and hospital admission in 91 consecutive patients admitted to 20 Portuguese hospitals between June 1995 and June 1998. We also studied the impact of admission delay on treatments (prescription of anticoagulants and the number of days elapsed between the onset of symptoms and start of anticoagulation and admission). Median admission delay was 4 days. Twenty-two (25%) patients were admitted within 24 h. Two thirds of the patients were admitted within 7 days and 75% within 13 days. In multiple logistic regression analysis, admission within 24 h was positively associated with mental status disorder (delirium or abulia; OR = 4.59; 95% CI = 1.41-14.89) and negatively associated with headache (OR = 0.03; 95% CI = 0.00-0.32). Presentation as isolated intracranial hypertension was associated with admission delay of more than 4 days (OR = 2.63; 95% CI = 0.97-7.14). Papilloedema was associated with an admission delay of more than 13 days (OR = 4.69; 95% CI = 1.61-13.61). There was no association between admission delay and the proportion of anticoagulated patients. The interval between onset of symptoms and start of anticoagulation was shorter in patients admitted earlier (p = 0.0001, for either admission within 24 h, 4 or 13 days). There is a considerable delay until the clinical picture associated with CVT is recognised as justifying hospital admission, especially when patients present with symptoms identical to isolated intracranial hypertension syndrome.

Anticoagulants↗

Intracranial dural arteriovenous fistulas with or without cerebral sinus thrombosis: analysis of 69 patients.

OBJECTIVES: To compare the characteristics of dural arteriovenous fistulas (AVFs) with or without cerebral sinus thrombosis (CST), and to analyse the determinants of aggressive manifestations in patients with dural AVF. METHODS: We investigated 69 patients aged 51.4 (SD 15) years who were diagnosed as having dural AVF. According to the location of the lesion and venous drainage pattern, dural AVF was classified into three sites (cavernous sinus, large sinus, and other) and five types (by Cognard's method). Aggressive manifestations of dural AVF were defined as intracranial haemorrhage, venous infarction, seizure, altered mental status, and intracranial hypertension. The diagnosis of CST was based on cerebral angiography. Logistic regression methods were used to analyse the determinants of aggressive manifestation in patients with dural AVF. RESULTS: CST was found in 39% of the patients with dural AVF. It was located at almost either the sinus around the dural AVF or the downstream venous flow pathways of the dural AVF. There was no significant difference with regard to sex, location, or type of dural AVF between patients with dural AVF with and without CST. The location "other sinuses" and the type of dural AVF "IIb/IIa+b/III/IV/V" were significantly related to aggressive manifestations of dural AVF (odds ratio 19 (p = 0.001) and 5.63 (p = 0.033), respectively). Presence of CST in patients with dural AVF had an odds ratio of 4.25 (p = 0.12) for development of aggressive manifestations. CONCLUSIONS: CST affects two fifths of patients with dural AVF. The location and type of dural AVF are major determinants of aggressive manifestations in patients with dural AVF.

Adult↗

Cerebral venous sinus thrombosis in a neonate due to factor V Leiden deficiency.

UNLABELLED: A case is described of cerebral venous sinus thrombosis due to factor V Leiden deficiency that occurred in the neonatal period, presenting with seizures. Diagnosis was based on ultrasonography, Doppler ultrasound (US) and magnetic resonance imaging (MRI). The aetiology was only recognized after blood-clotting tests, antithrombin III, C and S protein levels and factor V Leiden were studied. This situation was treated with intravenous heparin controlled by activated partial thromboplastin time (APTT). MRI angiography and Doppler US were important in the follow-up. It is important to assess periodically the neurological development of the baby and to prevent situations such as dehydration or severe infection, which can precipitate further thrombotic events. Molecular genetics allowed the identification of this trait in other family members. CONCLUSION: In every newborn with a thrombotic episode, coagulation studies must be performed, including testing for activated protein C (APC) resistance. Doppler US flow measurement and the MRI studies provide the best tools for diagnosis and follow-up. Heparin should be given to double the initial individual APTT. It is important to prevent any risk situation such as dehydration or severe infection, which could lead to a recurrence of a thrombotic episode. Regular assessment of the child's neurodevelopment is an important aspect of further care.

Activated Protein C Resistance↗

[Cerebral venous and sinus thrombosis (author's transl)].

In a 38-year-old patient who had taken oral contraceptives for several years partial thrombosis of the superior sagittal sinus and cortical veins occurred. Progress could be observed with the computer assisted tomography (CAT). CAT showed multiple localised intracerebral haemorrhages fronto-occipitally and penetrating deep into the white matter. In view of the severity of morphological changes the ability for regression of such haemorrhages is surprising. This equally applies to subsequent restitution without substantial visible defects in cerebral parenchyma following treatment with heparin. Among the presentations of sinus and central venous thromboses in CAT, the empty triangle or delta sign and the cord sign should be mentioned.

Adult↗

Sagittal sinus thrombosis associated with thrombocytopenia: a report of two patients.

Reported are two patients presenting with both thrombocytopenia and sagittal sinus thrombosis. The first patient is a 42-month-old male with no identified thrombophilic risk factors who developed acute neurologic symptoms after an acute infection. The second patient is a 22-month-old female with no history of preceding infection but had a positive lupus anticoagulant test. She also developed deep venous thrombosis and was treated with intravenous heparin. Both patients are currently doing well without neurologic deficits. To the authors' knowledge the second patient is the youngest reported patient with cerebral vein thrombosis associated with thrombocytopenia and lupus anticoagulant. These observations call attention to the need for a thorough investigation of thrombophilic risk factors in pediatric patients with thrombotic complications.

Brain↗

Orbital cellulitis and cavernous sinus thrombosis after cataract extraction and lens implantation.

Orbital cellulitis as a complication of ophthalmic surgery is uncommon. We treated a patient who had orbital cellulitis and cavernous sinus thrombosis three weeks after uncomplicated cataract extraction and lens implantation. Sinus x-rays showed sphenoid sinus opacification. Computed tomographic scan confirmed the sphenoid sinus disease, and no abscess was found. The patient recovered completely after treatment with intravenous antibiotics. Most orbital cellulitis is secondary to sinus disease. The trauma of surgery and the retrobulbar block must be considered possible causative factors in this patient, but sinus disease is still the most likely cause. Intraocular inflammation did not increase during the illness although the intraocular pressure rose from 14 to 23mmHg.

Cataract Extraction↗

Seizures in cerebral vein and dural sinus thrombosis.

To describe early symptomatic and late seizures in a cohort of patients with acute cerebral vein and dural sinus thrombosis (CVDST) and to identify their determinants, we performed a prospective registry and follow-up study of CVDST patients admitted to 20 Portuguese hospitals, from June 1995 to June 1998. Of 91 registered patients, 31 (34%) had early symptomatic seizures; 29 (31.9%) as a presenting feature and 2 (2.1%) after admission. Early symptomatic seizures were more frequent in patients with motor and sensory deficits and in those with focal oedema/ischaemic infarcts or haemorrhages on admission CT/MR. On multivariate logistic regression analysis, sensory defects (OR = 7.8; 95% CI = 0.8-74.8) and a parenchymal lesion on admission CT/MR (OR = 3.7, 95% CI = 1.4-9.4) were found to be significant predictors of early symptomatic seizures. Seizures were directly related to acute death in 2 patients. Eight (9.5%) patients had late seizures, which were multiple in 4 (4.8%). Late seizures were more frequent in patients with early symptomatic seizures and with haemorrhage on admission CT/MR. Neither early symptomatic seizures nor late seizures were related to functional prognosis at the last follow-up (median = 1 year). There is a moderate risk of seizure recurrence early in the course and during the first year after CVDST. Seizures can be a cause of acute death, but might not have an independent influence on functional outcome. Pharmacological prevention of seizures after CVDST should probably be limited to patients with early symptomatic seizures and cerebral lesions on admission CT/MR.

Adult↗