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Cerebral venous sinus thrombosis in Behcet's disease: a case report.

Behcet's disease ( BD ) is a systemic inflammatory vascular disease with variable clinical manifestations and numerous clinical masquerades. The venous involvement may affect veins of different size with tendency for thrombosis. BD is the commonest cause of cerebral venous thrombosis in some Middle Eastern countries. However, in Thailand, this clinical entity has been rarely described as a cause of cerebral venous sinus thrombosis. A 38 year-old man presented with recurrent attacks of transient ischemic attack (TIA) for 9 months. Two months after the TIA, he developed right hemiparesis and persistent diffuse headache. He had a 10 year history of recurrent oral and genital ulcers. Physical examination revealed bilateral uveitis, right hemiparesis, bilateral sixth cranial nerve paresis and bilateral papilloedema. Pathergy skin test showed positive result. Neuroimaging demonstrated empty delta sign on CT-scan and thrombosis of the posterior part of superior saggital sinus, transverse sinus, straight sinus and internal jugular vein on magnetic resonance venography (MRV). Cerebrospinal fluid (CSF) analysis revealed increased intracranial pressure and aseptic CSF profiles. Prednisolone, chlorambucil and anticoagulant had been prescribed. The clinical course improved gradually.

Adult↗

[Superior sagittal sinus thrombosis following L-asparaginase therapy of acute lymphoblastic leukemia].

A 48-year-old female received serial combination chemotherapy including L-asparaginase (L-ASP) for acute lymphoblastic leukemia. After administration of L-ASP, the prothrombin time and activated partial thromboplastin time were prolonged, while fibrinogen and antithrombin III levels markedly decreased, so she was given fresh frozen plasma (FFP). But subsequently, she developed cerebral infarction in the left parietal region and further hemorrhagic infarction in the right parietal region, and died. Autopsy revealed superior sagittal sinus thrombosis and bilateral cerebral infarction, but no obvious thrombus in other organs. Coagulopathy following L-ASP therapy is well-known. In this case, the coagulation studies at the first attack showed that the plasma protein levels of coagulation and fibrinolysis factors decreased in spite of administration of FFP. Fibrin-fibrinogen degradation products (FDP) slightly increased. However there were no significant abnormalities in the platelet count, nor soluble fibrin monomer, which suggested no evidence of disseminated intravascular coagulation. Thus, these findings suggest that L-ASP might be associated with the pathogenesis of thrombosis in this case.

Antineoplastic Combined Chemotherapy Protocols↗

Lateral sinus thrombosis associated with zoster sine herpete.

Herpes zoster results from reactivation of the varicella zoster virus (VZV). Zoster sine herpete (ZSH) is an uncommon manifestation of VZV infection and presents with similar symptoms but without the vesicular rash. We describe an unusual case of lateral sinus thrombosis (LST) that developed during the clinical course of ZSH in the C2 distribution. A 55-year-old woman presented with a 3-day history of left temporal and postauricular pain, nausea, vomiting, and mild photophobia. She denied otalgia, otorrhea, and hearing loss. Examination revealed hyperesthesia in the left C2 nerve root distribution without evidence of herpetic rash. A computed tomography scan showed minimal fluid in the left mastoid cavity (not mastoiditis) and thrombus within the left lateral and sigmoid dural sinus. Magnetic resonance imaging and magnetic resonance angiogram confirmed these findings. Laboratory studies revealed elevated neurotrophic immunoglobulin G levels to VZV. Hypercoagulable studies were normal. She was subsequently treated with Neurontin, acyclovir, and anticoagulation. Her symptoms improved, and she was discharged 3 days later. LST is generally a complication of middle ear infection. Nonseptic LST, however, may result from dehydration, oral contraceptive use, coagulopathy, or thyroid disease. This unusual case raises the suspicion that thrombosis resulted from VZV associated thrombophlebitis in the ipsilateral cerebral venous sinuses along the second cervical nerve root distribution. A high index of suspicion is necessary in such cases so that a different treatment course can be identified and antiviral medication initiated promptly.

Clinical Laboratory Techniques↗

A novel thrombomodulin gene mutation in a patient suffering from sagittal sinus thrombosis.

Thrombomodulin is an endothelial cell membrane glycoprotein that promotes protein C activation. It has been clearly demonstrated that the anticoagulant functions of the protein C system are important in the prevention of thromboembolic disease. Patients with protein C or protein S deficiency and/or resistance to activated protein C (APC resistance) are at higher risk for developing thromboembolic disease. The first mutation in the thrombomodulin gene was discovered in an American patient suffering from pulmonary embolism at the age of 45 (Ohlin and Marlar 1995). Here we report a case of sagittal sinus thrombosis in a 42-year-old Swedish woman. She was found to carry a heterozygous point mutation changing G127 to A, predicting an Ala25 to a Thr change in the mature thrombomodulin protein. This mutation was also found in her 16-year-old daughter, who so far has not suffered from any thrombotic events. The patient had no other detectable prothrombotic genetic defects associated with the coagulation system. This case supports the hypothesis of an association between mutations in the thrombomodulin gene and venous thrombosis.

Adolescent↗

High resolution computed tomography of superior sagittal sinus-thrombosis and -abnormalities.

Ten patients with suspected abnormalities or thrombosis of the superior sagittal sinus were studied by high resolution computed tomography using a thin slice technique after bolus injection. In all patients good visualization of the sinus pathology could be achieved, which correlated exactly with the findings in the 7 angiographically controlled cases.

Diagnosis, Differential↗

Open thrombectomy combined with thrombolysis in massive intracranial sinus thrombosis.

A case of complete thrombosis of the superior sagittal sinus in a pregnant women with multiple haemorrhages and hydrocephalus is described. Due to acute onset and progressive neurological deterioration, the decision was taken to remove the clot surgically. A sinotomy was performed removing the thrombotic material followed by local infusion of tissue plasminogen activator (t-PA). The combination of surgical removal and thrombolysis was life-saving for this young patient.

Adolescent↗

Gyral abnormalities in the early stage of superior sagittal sinus thrombosis.

We report computed tomography (CT) features in a case of segmental thrombosis of the superior sagittal sinus. On the initial cranial CT scan, both frontal cortices showed focal areas of slightly increased attenuation. The lesions were isointense on magnetic resonance (MR) images no matter what pulse sequence was used, except on fluid-attenuated inversion recovery images, which showed curvilinear sulcal hyperintensity. On postcontrast T1-weighted images, curvilinearly enhancing structures were apparent in both frontal cortical sulci. No lesion appeared on follow-up CT or in MR images. We speculated that the areas of slightly increased attenuation in the cortices represented blood congestion in the cortical veins, venules and capillaries without serum leakage. Cranial CT images should be carefully interpreted to avoid overlooking subtle lesions.

Aged↗

Dural puncture and corticotherapy as risks factors for cerebral venous sinus thrombosis.

Dural puncture with corticosteroid could be a predisposing factor for cerebral venous thrombosis (CVT). A 35-year-old woman using oral contraception was treated with corticosteroid epidural infiltration for L5 radiculalgia. The following day a postural headache developed and accidental dural puncture was suspected. Four days later, she presented with fever and consciousness impairment requiring mechanical ventilation. Magnetic resonance angiography (MRA) confirmed thrombosis of the superior sagittal sinus. Recanalization was observed three weeks later and the patient fully recovered. Blood tests for thrombophilia showed a moderate decrease in the C protein level (chronometric activity 44%, N = 65-130). CVT has been reported after spinal anaesthesia or peridural anaesthesia with accidental puncture. After dural puncture the decrease of cerebrospinal fluid pressure induces a rostrocaudal sagging effect with traumatic damage to the fragile venous endothelial wall, and may trigger a venous vasodilatation with resultant stasis. CVT has also been described in patients after lumbar puncture and oral corticoid treatment for multiple sclerosis and after corticosteroid intrathecal infiltration. Therefore, corticosteroids can be considered as a potential additional procoagulant stimuli.

Adrenal Cortex Hormones↗

Ocular complications of sigmoid sinus thrombosis from L-asparaginase.

This article presents a 9-year-old boy with acute lymphocytic leukemia who developed ocular complications after beginning treatment with L-asparaginase. Ocular motor examination revealed esotropia with a right abduction deficit, consistent with a sixth nerve palsy, and funduscopy revealed bilateral optic disc edema. Thrombosis of the right sigmoid sinus was noted on magnetic resonance imaging and magnetic resonance venography. Symptoms gradually resolved after discontinuing L-asparaginase and treatment with heparin.

Abducens Nerve↗

Rapid thrombectomy of superior sagittal sinus and transverse sinus thrombosis with a rheolytic catheter device.

Thrombosis of the dural venous sinuses is a potentially lethal condition that remains a diagnostic dilemma. Clinical outcome is typically dependent on the timeliness of diagnosis and definitive treatment. We report a case of successful rapid thrombectomy of extensive thrombus within the superior sagittal and transverse sinuses using a rheolytic catheter device. This appears to be a promising treatment option, particularly in those patients who do not respond to other, more established, forms of therapy.

Acute Disease↗

Rheolytic catheter thrombectomy, balloon angioplasty, and direct recombinant tissue plasminogen activator thrombolysis of dural sinus thrombosis with preexisting hemorrhagic infarctions.

We describe the case of a 28-year-old obtunded woman who presented with bilateral anterior parietal lobe cortical hemorrhages associated with thrombosis of the superior sagittal sinus, both transverse and sigmoid sinuses, and multiple cortical veins draining into the sagittal sinus. Initial heparin therapy was not effective. A combination of AngioJet rheolytic catheter thrombectomy, balloon angioplasty, and continuous direct superior sagittal sinus recombinant tissue plasminogen activator infusion led to venous recanalization with a successful clinical outcome, without worsening of the preexisting intracranial hemorrhages.

Adult↗

[Benign intracranial hypertension in polycythemia which had caused a lateral sinus thrombosis].

Report of a case of so called "benign" intracranial hypertension whose main characteristics are: the etiology attributed to a thrombosis of the venous transverse sinus in the course of a polycythemia; the clinical signs with a reinforcement during the course of the illness of a previous epilepsy; the prognosis with specially heavy visual impairment questioning the prefix "benign" applied to this condition.

Adult↗

Osteomyelitis, lateral sinus thrombosis, and temporal lobe infarction caused by infection of a percutaneous cochlear implant.

OBJECTIVE: Cochlear implantation has become a routine operation in the last 10 years. The most common soft tissue complications with transcutaneous cochlear implants include infection or necrosis of the flap and extrusion of the implant and device failure. The most common complication reported with percutaneous devices include minor skin irritations at the pedestal site, retraction of skin from the pedestal site, and loosening of screws that retain the pedestal. We describe one case of lateral sinus thrombosis and secondary temporal lobe infarction caused by infection of a screw anchoring the percutaneous pedestal of an Ineraid implant. STUDY DESIGN: Case report. SETTING: Tertiary referral center. CONCLUSIONS: Intracranial complications of a percutaneous bone-anchored pedestal may occur with little prodrome. Computed tomography (CT) scan of the pedestal and bone anchoring screws may be indicated if local evidence of infection persists.

Anti-Bacterial Agents↗

Endovascular treatment of dural sinus thrombosis with rheolytic thrombectomy and intra-arterial thrombolysis.

BACKGROUND AND PURPOSE: Cerebral venous thrombosis is a rare entity that can be difficult to manage. Intrasinus thrombolysis is an increasingly applied intervention, but this modality carries an increased risk of hemorrhage. We describe for the first time an option with a potentially lower incidence of intracranial bleeding, the combination of the AngioJet rheolytic thrombectomy catheter with intra-arterial thrombolysis, in 2 patients with extensive dural sinus thromboses, preexisting intracranial hemorrhage, and severe progressive neurological deficits despite heparin therapy. METHODS: Four procedures were performed in 2 patients with thromboses in the superior sagittal and transverse sinuses (right in 1 patient and bilateral in 1 patient) and cortical veins. Rheolytic thrombectomy was performed in the sigmoid, transverse, straight, and superior sagittal sinuses; this technique involves the use of the Bernoulli effect to create a vacuum that fragments and aspirates thrombus. For associated persistent cortical vein thromboses, low-dose intra-arterial thrombolysis was used. RESULTS: Both patients had excellent angiographic results with sinus reopening after rheolytic thrombectomy and cortical vein reopening after intra-arterial thrombolysis. Follow-up CT showed no change in 1 patient and increased preexisting intracranial hemorrhage in the other. One patient had a negative hypercoagulable workup, and the other patient had probable anti-phospholipid antibody syndrome. At 6 months, both patients had excellent clinical outcome with no neurological deficits except mild short-term memory loss in 1 patient. CONCLUSIONS: The combination of rheolytic thrombectomy with intra-arterial thrombolysis is a treatment modality that allows accelerated recanalization of occluded dural sinuses and cerebral veins with lower doses of thrombolytic agents.

Adult↗

Thrombotic events are not exclusive to the remission induction period in patients with acute lymphoblastic leukemia: a report of two cases of cerebral sinus thrombosis.

It is well established that in acute lymphoblastic leukemia (ALL) patients L-asparaginase (L-Ase) provokes thrombotic events reducing coagulation inhibitors in both adults and children. A tight correlation between thrombotic and hemorrhagic complications and ALL has also been hypothesized because of the high incidence of disseminated intravascular coagulation (DIC) found during the early period of chemotherapeutic treatment apart from L-Ase. All the authors reporting on this subject, however, consider the remission induction phase of treatment the most risky, if not exclusive, for the development of a thrombotic event, in particular if it includes the administration of L-Ase. We report here two cases of ALL patients who experienced a cerebral sinus thrombosis in a later phase of treatment, demonstrating that the thrombotic risk is surely exacerbated by chemotherapy but is not exclusive to the remission induction period.

Adult↗