Thrombosis of the superior cerebral vein with hemorrhagic cerebral infarction--serial MRI and pathological study of a case

We reported an autopsy case of thrombotic occlusion of the superior cerebral vein with hemorrhagic laminar necrosis of the right parietal cortex. A 68-year-old woman was admitted to our hospital because of a severe headache and left hemiplegia of acute onset. There was a past history of hypertension, fever of unknown origin, leukocytopenia and nasal dermatitis. Magnetic resonance images (MRI) disclosed thrombosis of the superior sagittal sinus and of the right parietal cortical vein as well as right parieto-occipital cerebral infarction. Although she improved with mild sequelae, the subsequent MRI showed a recurrent thrombosis of the superior sagittal sinus. Ten months after the onset she died suddenly, presumably due to acute myocardial infarction. Pathologically, thrombotic occlusion of the right parietal cortical vein, recurrent thrombosis of the superior sagittal sinus and old hemorrhagic cortical laminar necrosis of the right parietal region were revealed. Moreover, intracranial arteritis and phlebitis were observed, as well as arteriolitis in the peripheral nerves. In our case, MRI was useful for the diagnosis and following the course of cerebral venous thrombosis. Cerebral noninfective vasculitis may well have caused the venous thrombosis.     

A Case of Cerebral Venous Thrombosis and Deep Venous Thrombosis Due to Hyperthyroidism with Increased Factor VIII Activity

A 48-year-old woman was admitted to our hospital because of headache and fever. She was diagnosed with aseptic meningitis. Five days later, she had a seizure and developed left hemiparesis. Magnetic resonance imaging showed hyperintensity in the right parietal area on fluid attenuated inversion recovery imaging. She was diagnosed as having cerebral venous thrombosis (CVT) because the suprasagittal sinus was invisible on the venographic studies. Moreover, deep venous thrombosis (DVT) was detected in her left lower extremity. Laboratory findings showed hyperthyroidism and markedly increased factor VIII activity. This is a rare case of concomitant CVT and DVT induced by high factor VIII activity due to hyperthyroidism under the presence of meningitis, an additional risk factor for thrombosis.     

Interventions in cerebrovascular emergencies among patients with Paroxysmal nocturnal haemoglobinuria - A word of caution

IntroductionParoxysmal nocturnal haemoglobinuria (PNH) is a clonal hematopoietic disorder, where there is deficiency of glycosylphosphatidylinositol (GPI) anchored proteins in the cell membrane, leading to increased complement sensitivity of red blood cells, intravascular hemolysis and vascular inflammation. Arterial and venous strokes in patients with PNH are a rarity posing significant diagnostic and therapeutic challenges. We report our experience with management of PNH patients with cerebrovascular emergencies.MethodsWe report 2 patients with PNH, one who was previously diagnosed with PNH and had arterial stroke, the other had an index presentation of cerebral venous sinus thrombosis (CVT) and was subsequently diagnosed with PNH. We also present the systematic review of literature reporting similar cases, highlighting the challenges in management.ResultsBoth patients presented to our centre with cerebrovascular emergency. The first patient was a diagnosed with PNH, and presented with left hemispheric infarction caused by thrombosis of middle cerebral artery. He was thrombolysed and underwent mechanical thrombectomy, which was unsuccessful in view of repeated re – thrombosis of the vessel. The patient survived with significant disability. The second patient had severe cerebral venous sinus thrombosis with large right hemispheric hemorrhagic venous infarction. She underwent emergency decompressive hemicraniectomy complicated by massive blood loss and disseminated intravascular coagulation. She subsequently had recurrent life threatening intracranial bleed secondary to platelet transfusions, thrombocytopenia, and use of contrast agents. She progressed to develop Budd Chiari syndrome and was initiated on Eculuzimab. She became transfusion independent, however remained in minimally conscious state and succumbed to sepsis.ConclusionsManagement of arterial and venous strokes is complex in patients with PNH. Invasive procedures and platelet transfusions are to be avoided in acute thrombosis, till robust evidence is available establishing the safety of the same in patients with PNH. Eculuzimab is a promising option, but far from reach for patients in developing countries.     

Overlooked early CT signs of cerebral venous thrombosis with lethal outcome

The aim of this report is to emphasize the consequences of overlooked initial CT signs of cerebral venous thrombosis. Brain CT was ordered in an afebrile patient with neck pain and occipital headache. Since no abnormalities were noted on non-contrast CT study, the patient was discharged with recommendation for routine laboratory tests and plain X-ray of the cervical spine. Right hemiparesis developed the next day with persistent headache and the patient was sent back to the Neurology Clinic where he developed myoclonic seizures compatible with focal motor status epilepticus. Neuroimaging, performed two days later, revealed a huge hemorrhagic venous infarcts in the left posterior cerebral hemisphere associated with typical signs of dural sinus thrombosis. Subtle curvilinear hyperdensities were detected within the left parietal cortico-subcortical border zone on reevaluation of the initial brain CT. A posteriori these were thought to be compatible with a developing venous infarct, associated with subtle signs most consistent with combined cortical vein and sinus thrombosis. No improvement was noted after administration of anticoagulant treatment and the patient died 11 days after the initial CT scan. Detection of early CT signs of cerebral venous thrombosis is extremely important, since delaying adequate treatment may have catastrophic consequences.     

Venous thrombosis during pregnancy: leg and trimester of presentation.

In order to determine the relative frequencies of left and right leg venous thrombosis during pregnancy and the frequencies of venous thrombosis during the three trimesters, a cohort study of 60 consecutive patients with a first episode of venous thrombosis during pregnancy was performed. Fifty-eight women had isolated left leg thrombosis, two patients had bilateral venous thrombosis and no patient had isolated right leg venous thrombosis. Thirteen patients had venous thrombosis during the first trimester (21.7%), 28 during the second trimester (46.7%) and 19 during the third trimester (31.7%). These findings indicate that patients with symptoms in the right leg rarely have venous thrombosis. Because leg pain and swelling occur most frequently during the third trimester but venous thrombosis is relatively equally distributed during all three trimesters, patients presenting earlier during pregnancy are more likely to have venous thrombosis than patients presenting later during pregnancy.     

Temporal Profile of CT and T2*-Weighted Gradient-Echo MRI in a Patient with Unilateral Thalamostriate Vein Thrombosis

Deep cerebral venous system thrombosis (DCVST) is an uncommon variety of thrombosis that accounts for 11% of cases of cerebral venous thrombosis. Thalamostriate vein (TSV) thrombosis is further rare among patients with DCVST. Although patients with cerebral venous thrombosis commonly have characteristic neurological deficits including headache, deterioration of consciousness, and seizures, patients with DCVST do not necessarily show such symptoms. Therefore, diagnose of DCVST is sometimes difficult. Here we report a case of TSV thrombosis with a unilateral basal ganglion lesion presenting with right-sided hemiparesis. A 61-year-old Japanese female was referred to our hospital. On neurological examination, she had no headache but presented with right facial paresis with dysarthria. Her right hemiparesis was present in the upper and lower extremities. We repeatedly performed brain computed tomography (CT) and T2*-weighted conventional gradient-echo (GRE) magnetic resonance imaging, and conclusively diagnosed as left TSV thrombosis. We firstly report a case of unilateral DCVST associated with TSV thrombosis in which a temporal profile of CT and T2*-weighted GRE images was obtained. Although DCVST is a rare clinical entity, physicians should be aware that repeated radiological observations can be useful for the diagnosis and early medical treatment for DCVST.     

Intracranial Extension of Spinal Subarachnoid Hematoma Causing Severe Cerebral Vasospasm

Spinal subarachnoid hemorrhages (SAH) can extend into the intracranial subarachnoid space, but, severe cerebral vasospasm is rare complication of the extension of intracranial SAH from a spinal subarachnoid hematoma. A 67-year-old woman started anticoagulant therapy for unstable angina. The next day, she developed severe back pain and paraplegia. MRI showed intradural and extramedullar low signal intensity at the T2-3, consistent with intradural hematoma. High signal intensity was also noted in the spinal cord from C5 to T4. We removed subarachnoid hematoma compressing the spinal cord. The following day, the patient complained of severe headache. Brain CT revealed SAH around both parietal lobes. Three days later, her consciousness decreased and left hemiplegia also developed. Brain MRI demonstrated multiple cerebral infarctions, mainly in the right posterior cerebral artery territory, left parietal lobe and right watershed area. Conventional cerebral angiography confirmed diffuse severe vasospasm of the cerebral arteries. After intensive care for a month, the patient was transferred to the rehabilitation department. After 6 months, neurologic deterioration improved partially. We speculate that surgeons should anticipate possible delayed neurological complications due to cerebral vasospasm if intracranial SAH is detected after spinal subarachnoid hematoma.     

Cerebral venous thrombosis mimicking acute unilateral vestibulopathy

We report a patient with cerebral venous thrombosis who presented with acute onset of severe prolonged vertigo, nausea, vomiting, unilateral decreased caloric response and occipital headache, simultaneously with acute venous cerebral infarcts on brain MRI. Although the patient had occipital headache, overall symptoms and signs closely mimicked those of acute unilateral vestibulopathy. Cerebral venous thrombosis should be considered in the differential diagnosis of acute unilateral vestibular syndrome.     

A case of young adult presenting with cerebral infarction caused by homocystinuria]

Homocystinuria is a congenital metabolic disorder, and has been known as life-threatening risk factor of vascular disease including ischemic stroke. We report a case of cerebral infarction due to homocystinuria. The patient was a 21-year-old woman exhibiting left hemiparesis and a previous history of ectopia lentis. Magnetic resonance imaging showed multiple fresh infarctions in the right frontal and temporal lobes, basal ganglia, corona radiata, and internal capsule. The right common carotid angiogram demonstrated complete occlusion at the origin of the right internal carotid artery. Further investigation clarified increased level of serum methionine and homocysteine and urinary homocystin due to cystathionine beta-synthase deficiency. Homocystinuria was diagnosed as the cause of cerebral infarction. The patient was treated by low methionine diet and administration of folic acid, cobalamin, and aspirin. It should be recognized that some patients with homocystinuria are missed in the neonatal screening for congenital metabolic disorders. Recent studies indicated that the homocysteinemia is one of risk factors of ischemic stroke in the general population as well as in the patients of homocystinuria. We recommend metabolic screening for homocystinuria, when treating a juvenile patient with ischemic stroke of unknown etiology.     

Cerebral venous infarction following thrombosis of the draining vein of a venous angioma (developmental abnormality)

We report two cases of cerebral venous angioma presenting as venous infarction, one in the left parietal lobe, the other in the left frontal lobe. Cerebral imaging demonstrated thrombotic occlusion of the draining vein of the venous angioma associated in the latter case with thrombosis of the anterior part of the superior longitudinal sinus. Both patients were free of coagulopathy. They were treated with anticoagulant therapy. One completely recovered, while the other was left with slight residual disability. Thrombosis of the draining vein has been reported in only 6 previous cases, of whom only 2 received anticoagulant therapy. Discovery of a venous angioma in the diagnostic workup of a patient with recent neurological disorders should raise the question of a possible occlusion of the draining vein and lead to an appropriate therapy.     

Prolonged direct catheter thrombolysis of cerebral venous sinus thrombosis in children: a case series

Cerebral venous sinus thrombosis is a rare condition with potentially devastating neurologic outcome--death and severe disability are common in advanced cases. In adults, protocols for mechanical clot disruption and direct thrombolysis are established; no guidance exists for children. We present our experience of 6 children with cerebral venous sinus thrombosis and ominous clinical progression. We found that effective thrombolysis required substantially longer infusion, more rounds of mechanical disruption, and higher doses of thrombolytics than are commonly practiced. Despite pervasive thrombosis, prethrombolysis hemorrhage, coma, and other predictors of death and disability, our patients survived and 4 of 6 had no functional deficits. One patient had moderate, and one had severe deficits. We report these cases to illustrate that hemorrhage may not be a contraindication to thrombolysis for cerebral venous sinus thrombosis, that prolonged infusion may be required to restore perfusion, and that good neurologic outcomes can be achieved despite dire clinical presentations and extensive sinus thrombosis.     

Superior sagittal sinus thrombosis as first manifestation of essential thrombocythemia]

A 52-year-old previously healthy woman was admitted to our hospital for status epilepticus in November 1999. She had not taken oral contraceptives. After treatment with intravenous diazepam and phenytoin, she did not develop seizures anymore. When she became alert, there was a mild left hemiparesis. Lumbar puncture showed an opening pressure of 145 mm H2O, and the cerebrospinal fluid was acellular. Cranial MR imaging demonstrated thrombosis of the superior sagittal sinus and fresh infarction in the right frontal lobe. Plasma fibrinogen, fibrin degradation product, and prothrombin fragment 1 + 2 levels were elevated. Proteins S and C activities and anti-thrombin III levels were within the normal range. Lupus anticoagulant and anti-cardiolipin antibody were negative. She was treated with continuous heparin infusion for ten days and with oral warfarin thereafter. Six months after the first admission, platelet count became more than 400 x 10(3)/microliter. In July 2002, she developed slowly progressive monoplegia of the left arm. Cranial MR imaging demonstrated patent superior sagittal sinus, fresh infarction in the right parietal lobe, and old small infarction in the right corona radiata. The patient was maintained on warfarin and 100 mg of aspirin thereafter. In September 2002, platelet count was 737 x 10(3)/microliter. Bone marrow examination showed increased megakaryopoiesis with normal erythroid and myeloid series and no chromosomal aberrations. Serum C-reactive protein and iron levels were in the normal range. An abdominal ultrasound demonstrated mild splenomegaly. Thus, we made a diagnosis of essential thrombocythemia (ET). ET causes thrombotic events in the course of the disease at a rate of 7% per year. Cerebral infarction is not uncommon, but occurrence of cerebral sinus thrombosis has been rarely reported. Recently, several cases have been reported in which cerebral infarction was the first manifestation of ET even with platelet counts lower than 600 x 10(3)/microliter. To our knowledge, there have been no reported cases of ET presenting with cerebral venous sinus thrombosis. Platelet count should be monitored in the patients with venous sinus thrombosis of undetermined etiology.     

Impaired washout--embolism and ischemic stroke: further examples and proof of concept

We previously posited that impaired washout of emboli was an important mechanism of brain infarction in patients with cerebral hypoperfusion. Hyperviscosity and hypoperfusion enhance thrombus formation promoting embolization of fresh thrombi. Hypoperfusion also reduces dissolution of emboli due to reduced flow velocity leading to classic hemodynamic patterns of stroke. To prove this concept further, we identified patients with severe hemodynamic compromise of either arterial or venous origin, or both, and natural or iatrogenic mechanisms of embolism. Three conditions were investigated in a stroke MRI protocol: 5 patients who had conventional cerebral angiography for the diagnosis of moyamoya disease, 1 patient with atrial fibrillation and thrombosis of the left transverse dural sinus and 1 patient with a patent foramen ovale with atrial septum aneurysm presenting with thrombosis of cortical cerebral veins. In all patients, subcortical arterial embolization within an atypical borderzone of hypoperfusion was observed.     

自发性低颅压并发静脉血栓形成和出血性梗死一例临床分析

目的自发性低颅压(SIH)多以与体位相关的剧烈头痛为主要症状,误诊率高。SIH并发静脉和(或)静脉窦血栓形成非常罕见。方法我们报道1例SIH并发静脉血栓形成和出血性梗死的患者,并复习相关文献。结果本例患者自发性低颅压在前,静脉血栓形成和出血性梗死在后,同时经过检查排除了其他可导致静脉血栓形成的危险因素。所以我们考虑SIH是静脉血栓形成的危险因素。假如SIH患者与体位相关的间断的头痛变成持续性头痛时,应考虑到有静脉血栓形成的可能。结论因为SIH的患者有形成硬膜下血肿的风险,所以对于静脉血栓形成的抗凝治疗应谨慎。     

Cerebral emboli of paradoxical origin

A diagnosis of paradoxical cerebral embolus (PCE) was made in five patients aged 31 to 62 years who sustained eight cerebral ischemic events. No patient had evidence of primary carotid system or left heart disease. A probe-patent foramen ovale was the presumed mechanism in four patients, and an unsuspected congenital atrial septal defect was found in the fifth patient. Clinically apparent pulmonary emboli or venous thrombosis preceded the cerebral event in only one instance. Review of the literature reveals a high mortality with PCE. However, careful clinical search for this lesion may be rewarding: four of our five patients survived. One should consider PCE in any patient with cerebral embolus in whom there is no demonstrable left-sided circulatory source. This principle applies particularly if there is concomitant venous thrombosis, pulmonary embolism, or enhanced potential for venous thrombosis due to, for example, morbid obesity, use of hormonal birth control pills, prolonged bed rest (especially postoperatively), or systemic carcinoma.     

Cerebral venous sinus thrombosis complicated with acute development of dural arteriovenous fistula: A case report

Development of dural arteriovenous fistula (dAVF) after cerebral venous sinus thrombosis (CVST) was very uncommon and for all these reported cases, the dAVF was a chronic complication. We present a case of acute development of dAVF after CVST. A 40-year-old female was admitted into our department with 2 day’s headache and vomiting for 9 h. Head computed tomography (CT) scan showed only scattered minor hematomas over the right frontal lobe. Blood test indicated an elevated D-dimer. The patient experienced transient paralysis (Todd’s paralysis) after intermittent focal epilepsy from day 3, which progressed into sustained epilepsy on day 6. Magnetic resonance imaging (MRI) on day 7 confirmed the thrombosis of the superior sagittal sinus and a large area of infarction and edema in the left frontal and parietal lobe. She was then treated with heparin and warfarin. Cerebral angiography on day 9 demonstrated a dAVF which was classified as Borden Type II and fed by the left occipital artery. Subsequently, endovascular occlusion of the fistula was conducted and the patient recovered well with only slight right limbs weakness at 1 year follow up.     

Diagnosis of acute phase of venous infarction by diffusion-weighted image: case report and review of the literature]

Diffusion-weighted imaging (DWI) can diagnose early stage not only of the arterial infarction but also of venous infarction. We successfully diagnosed a case as acute venous infarction by DWI. The patient, an infant of one year and ten months, presented disturbance of consciousness and left hemiparesis two weeks after dehydration and infections. Computed tomographic scan revealed a cerebral hemorrhage in the right parietal lobe. Cerebral angiography revealed no contrast filling of the posterior side of superior sagittal sinus, straight sinus and transverse sinus. DWI demonstrated a large hyperintensity lesion around the hematoma, suggesting venous infarction in the early stage. We thought that venous infarction was caused by secondary extension of thrombus to cerebral cortical veins and deep cerebral veins. External decompression and postoperative hypothermia therapy were performed because of rapidly deteriorating intracranial hypertension. Intracranial hypertension was, however, uncontrollable. The patient died four days after the onset. Diagnosis of the venous infarction by DWI was discussed along with other recent reports. Diffusion hyperintensity was displayed in almost all subjects with acute venous infarction. DWI pattern of venous infarction is more heterogeneous than that of arterial one because pathway from venous obstruction to infarction is complicated.     

Familial antithrombin III abnormality accompanied with progressing ischemic stroke

We report a case of familial antithrombin III (AT-III) abnormality accompanied with progressing ischemic stroke. The patient was a 31-year-old female who developed consciousness disturbance and left hemiparesis on December 1 in 1987. She had a history of two transient ischemic attacks and three episodes of thrombophlebitis of the extremities. Cerebral CT scan showed a low density area on the right temporal lobe that had extended to the right parietal and on the left frontal lobe as clinical symptoms worsened. Cerebral angiogram revealed branch occlusions of the right middle cerebral artery and showed no cerebral venous and sinus obstruction. When her symptoms had been progressing to show semi-comatose state, left hemiplegia and transient Cheyne-Stokes respiration, we found her decreased biological activity and normal immunological level of AT-III. The diagnosis of familial AT-III abnormality had been made by familial investigation. As the treatment of AT-III concentrates transfusion was started from the third day, her symptoms gradually recovered and the low density area stopped extending. Further examinations revealed that she was a homozygote of AT-III abnormality presenting no affinity for heparin and that her parents were heterozygotes. It was suggested that the homozygous AT-III abnormality was the main cause of her progressing ischemic stroke.