Cerebral venous thrombosis and demyelinating diseases: report of a case in a clinically isolated syndrome suggestive of multiple sclerosis onset and review of the literature

Cerebral venous thrombosis (CVT) has been described in several cases of clinically definite multiple sclerosis (MS). In the majority of these, lumbar puncture followed by intravenous corticosteroid treatment was suspected as the cause. We report what is, to our knowledge, the first case of a patient with a multifocal clinically isolated syndrome suggestive of MS onset, who developed multiple CVT after lumbar puncture and during high-dose i.v. corticosteroid treatment We conclude that the sequence 'lumbar puncture followed by corticosteroid treatment' may be a contributory risk factor for the development of CVT when associated with other risk factors.     

Lumbar puncture and dural sinus thrombosis--a causal or casual association?

BACKGROUND: A few cases of cerebral venous thrombosis (CVT) were reported after a lumbar puncture (LP), suggesting a causal association. The purpose of our study was to document that LP might predispose to CVT by decreasing blood flow velocities (BFV) in veins or dural sinus. METHODS: We performed a transcranial Doppler ultrasound study to register the mean BFV of the straight sinus (SS) before, during and after LP. RESULTS: Thirteen patients were studied. LP induced a decrease of 47% of mean BFV in the SS. The mean decrease of BFV was significant immediately at the end (p = 0.003), 30 min after (p = 0.015) and more than 6 h after LP (p = 0.008). CONCLUSIONS: LP induced a sustained decrease of mean BFV in the SS. The decrease of venous blood flow is a possible mechanism contributing to the occurrence of CVT.     

Cerebral venous thrombosis after lumbar puncture and intravenous high dose corticosteroids: A case report of a childhood multiple sclerosis

The association between cerebral venous thrombosis (CVT) and multiple sclerosis (MS) has already been reported in several adult patients with clinically definite MS, in a suspected relation to i.v. corticosteroids or previously performed lumbar puncture (LP). We are reporting a case, which is, to our knowledge, the first one concerning a child patient with a MS, who developed multiple CVT after LP and during high-dose i.v. corticosteroid. Our conclusions are that the sequence LP followed by high dose corticosteroids may be a contributory factor for the development of CVT when associated with other risk factors.     

Risk of thromboembolism after cerebral venous thrombosis

The outcome of cerebral venous thrombosis (CVT) has been studied infrequently. We assessed the frequency of recurrence of cerebral or systemic thromboembolism and factors influencing recurrence. We performed a retrospective study of consecutive patients with CVT in the period 1985-2002 who were admitted to the University Hospital Gasthuisberg. We performed a chart review and a semi-standardized telephone interview that focused on recurrent CVT or systemic thromboembolism. Fifty-four CVT patients with a mean age of 42 years were followed up for a mean of 3.5 years. Eighty percent were women. Coagulation disorders were found in 17 patients (31%). One patient (1.9%) had recurrent CVT and seven patients (12.9%) suffered systemic thromboembolism after a median of 2.5 months. Patients with recurrent thromboembolism more often had coagulopathies (P = 0.04) or a history of deep venous thrombosis (P = 0.007). Patients with early recurrent venous thromboembolism often were not treated with oral anticoagulants (P < 0.001). It was evident from the above study that a substantial number of patients suffer recurrent thromboembolism after CVT.     

Cerebral venous thrombosis in four patients with multiple sclerosis

We report four new cases of cerebral venous thrombosis (CVT) occurring in patients with multiple sclerosis (MS). Each patient had undergone lumbar puncture at varying times prior to clinical presentation (4 days to over 1 year). Only two of the patients had received intravenous (i.v.) methylprednisolone 48 h prior to CVT and were under oral contraception, a risk factor for cerebral thrombophlebitis. The other two patients had not undergone recent lumbar puncture, were not taking corticosteroids and did not present vascular risk factors. The patients all had normal routine blood work-ups and none had thrombophilia. All patients dramatically improved with full systemic heparinization. Minor sequelae were noticed in two patients. The pathogenesis underlying the occurrence of CVT in MS patients remains unclear and we discuss the relationship between lumbar puncture, steroid treatment and CVT.     

Safety and efficacy of Direct Oral Anticoagulants in cerebral venous thrombosis: A meta-analysis

Cerebral venous thrombosis (CVT) is caused by partial or complete occlusion of the major cerebral venous sinuses or the smaller feeding cortical veins which predispose to the risk of venous infarction and hemorrhage. Current guidelines recommend treating CVT with either low-molecular-weight heparin (LMWH) or unfractionated heparin (UFH) followed by an oral vitamin K antagonist (VKA) for 3–12 months. Direct oral anticoagulants (DOACs) have already established benefit over warfarin as a long-term treatment of symptomatic venous thromboembolic disorder like deep vein thrombosis (DVT), and pulmonary embolism (PE) given its equal efficacy and better safety profile. The benefit of DOACs over warfarin as a long-term anticoagulation for CVT has likewise been extensively studied, yet it has not been approved as first-line therapy in the current practice. We therefore performed a systematic review and meta-analysis of relevant studies to generate robust evidence regarding the safety and efficacy of DOACs in CVT. This meta-analysis demonstrates that the use of DOACs in CVT has similar efficacy and safety compared to VKAs with better recanalization rate.     

Deep cerebral vein thrombosis associated with iron deficiency anaemia in adults.

Cerebral venous thrombosis (CVT) is rare and has a wide spectrum of symptoms, therefore it is difficult to diagnose. Thrombosis of the deep cerebral veins occurs very rarely: it has been reported that approximately 6% of patients with CVT have deep CVT, and the prognosis for patients with this condition is poor. CVT has been reported in association with dehydration, a hypercoagulable state, mastoiditis, tumour invasion of a venous sinus, use of oral contraceptives, pregnancy, puerperium, head trauma, vasculitis, and intracranial and systemic infections. However, in the literature, there are few reported cases of CVT in association with iron deficiency anaemia, especially in adults. We present here two patients with bilateral thalamic and basal ganglionic lesions due to thrombosis of the deep cerebral veins. Both of our patients had severe hypochromic microcytic anaemia due to iron deficiency, and both had a good prognosis after 2 months.     

Isolated intracranial hypertension as the only sign of cerebral venous thrombosis.

BACKGROUND: Cerebral venous thrombosis (CVT) is often overlooked when intracranial hypertension (ICH) is isolated, hence mimicking idiopathic intracranial hypertension (IIH). OBJECTIVE: To describe the characteristics of patients with CVT and ICH. METHODS: We examined 160 consecutive patients with CVT between 1975 and 1998. They were separated into two groups according to their clinical presentation--isolated ICH and other neurologic symptoms and signs. RESULTS: Fifty-nine patients with CVT (37%) presented with isolated ICH. Neuroimaging showed involvement of more than one sinus in 35 patients (59%). Brain CT was normal in 27 of 50 patients (54%). Lumbar puncture was performed in 44 patients and showed elevated opening pressure in 25 of 32 (78%) and abnormal CSF content in 11 (25%). Etiologies and risk factors included local causes in 7 of 59 (12%), surgery in 1, inflammatory diseases in 18 (30.5%), infection in 2, cancer in 1, postpartum state in 1, coagulopathies in 11 (19%), oral contraception in 7 (12%), and remained unknown in 11 (19%). Anticoagulants were used in 41 of 59 patients (69.5%), steroids or acetazolamide in 26 (44%), therapeutic lumbar puncture in 44 (75%), and surgical shunt in 1. Three patients had optic atrophy with severe visual loss, 1 died from carcinomatous meningitis, and 55 (93%) had complete recovery. CONCLUSIONS: Central venous thrombosis (CVT) can present with all the classical criteria for idiopathic intracranial hypertension (IIH), including normal brain CT with normal CSF content. Because the recognition of CVT has crucial prognostic and therapeutic implications, MRI, with magnetic resonance venography when necessary, should be performed in patients with isolated intracranial hypertension. The outcome of CVT is unpredictable, and management of patients with CVT should not differ whether they present with isolated raised intracranial pressure or with other neurologic symptoms and signs. Therefore, isolated raised intracranial pressure from CVT differs in management from IIH and should be classified neither as "IIH" nor "pseudotumor cerebri."     

Dural puncture and activated protein C resistance: risk factors for cerebral venous sinus thrombosis

OBJECTIVES—Dural puncture is regarded asafe procedure when contraindications are carefully excluded and has sofar not been recognised as a risk factor for cerebral venous sinusthrombosis (CVST). Five patients are described with CVST after duralpuncture in the presence of additional risk factors.
METHODS—In four out of five patients completeinvestigations for thrombophilia were performed at least one monthafter withdrawal of oral anticoagulation.
RESULTS—In three out of four patients tested,activated protein C (APC) resistance due to heterozygous coagulationfactor V R506Q mutation (factor V Leiden) was found. One patient wasusing oral contraceptives as a circumstantial risk factor and three hadhad spinal anaesthesia for surgical procedures. Family history of venous thromboembolism was negative in all patients. Retrospective evaluation of 66 patients with CVST disclosed that dural puncture wasthe fourth most common risk factor (8%) possibly contributing to thrombosis.
CONCLUSION—Dural puncture may constitute anadditional risk factor for CVST especially in patients with APCresistance or surgery. In such patients a thrombophilia screen is indicated.

     

自发性低颅压并发颅内静脉窦血栓形成临床分析

目的 自发性低颅压（spontaneous intracranial hypotension,SIH）及颅内静脉窦血栓形成（cerebralvenous thrombosis,CVT）均为神经科少见疾病,而SIH合并CVT更为少见,本研究旨在探寻二者之间有无内在联系。方法 报道2例SIH并发CVT的临床资料,并复习相关文献。结果 2例患者SIH在前,CVT发生在后,同时排除了其他导致静脉窦血栓的危险因素,因此推测SIH也是CVT的危险因素之一。经补液纠正低颅压后,静脉窦血栓也随之好转。结论 SIH可能导致CVT,这一观点也被相关文献所证实。     

Neuropsychological manifestations in a case of bilateral thalamic infarction.

Neuropsychological manifestation has been reported with lesions of the anterior and non-specific thalamic nuclei and mammilothalamic tract (MMT). These have been reported in the setting of arterial infarction and/or haemorrhage. Cerebral venous sinus thrombosis (CVT) is a rare cause of brain infarction. It occurs in the setting of oral contraceptive administration or pregnancy. Inherited thrombophilias are documented risk factors. The most frequent being heterozygous factor V Leiden mutation. We report a single case of bilateral thalamic infarction due to cerebral vein and sinus thrombosis. Clinically the case manifested with memory impairment and dysexecutive symptoms. Predisposing factor for venous thrombosis was a homozygous factor V Leiden mutation. The patient was treated with anticoagulation and made a good recovery.     

Isolated Lateral Sinus Thrombosis Presenting as Cerebellar Infarction in a Patient with Iron Deficiency Anemia

As a rare cerebrovascular disease, cerebral venous thrombosis (CVT) is caused by various conditions including trauma, infection, oral contraceptive, cancer and hematologic disorders. However, iron deficiency anemia is not a common cause for CVT in adult. Posterior fossa infarction following CVT is not well demonstrated because posterior fossa has abundant collateral vessels. Here, we report a case of a 55-year-old man who was admitted with complaints of headache, nausea, and mild dizziness. The patient was diagnosed with isolated lateral sinus thrombosis presenting as cerebellar infarction. Laboratory findings revealed normocytic normochromic anemia due to iron deficiency, and the patient''s symptoms were improved after iron supplementation.     

Interobserver agreement in the magnetic resonance location of cerebral vein and dural sinus thrombosis

The interobserver variation in the magnetic resonance (MR) location of cerebral vein and dural sinus thrombosis (CVT) has not been previously reported. Four independent observers rated a convenience sample of 40 MR/MR angiographies to assess whether or not each dural sinus and major cerebral veins were occluded. Interobserver reliability was measured using κ statistics. Interobserver agreement was comparable between the six pairs of raters. Agreement was excellent for thrombosis of the deep cerebral venous system ( κ  = 1.00), cerebellar veins ( κ  = 1.00), superior saggital sinus ( κ range: 0.82–1) and right jugular vein ( κ range: 0.84–0.95); good to excellent for the right transverse/sigmoid sinus ( κ range: 0.75–0.90) and the left jugular vein ( κ range: 0.65–0.85); moderate to excellent for the left lateral sinus ( κ range: 0.59–0.78) and the straight sinus ( κ range: 0.59–0.92); poor to good for the cortical veins ( κ range: 0.02–0.65). Agreement between observers varies with the location of CVT. It is good or excellent for most of the occluded sinus and veins, except for the cortical veins. This study suggests that information on the location of CVT can be reliably collected and used in multicentre studies.     

Cerebral venous sinus thrombosis and thrombophilia presenting as pseudo-tumour syndrome following mild head injury

Cerebral venous sinus thrombosis (CVT) after mild head injury is infrequent. A 38-year-old patient presented with a Glasgow Coma score (GCS) of 15 after a road traffic accident. CT scan revealed a temporal contusion. He was treated with measures to prevent cerebral oedema and anticonvulsants. Three weeks later he presented with features of pseudo-tumour syndrome. Investigations revealed the presence of cerebral venous sinus thrombosis, protein C deficiency and elevated titres of antiphospholipid antibodies. He was treated with anticoagulants and showed improvement. This case report highlights that multiple "hits" may lead to CVT and hence laboratory screening of patients with CVT is necessary even if the clinical situation seemingly provides sufficient explanation for a thrombotic event. The presence of acquired and inherited causes of thrombophilia need not always lead to symptomatic thrombosis. As illustrated by this case, a second hit, such as trauma, may be the precipitating factor that unmasks the prothrombotic state.     

Post-dural puncture related complications after diagnostic lumbar puncture, myelography and spinal anaesthesia

Objectives – This study was conducted to investigate complications after dural puncture. Material and methods – A 15 months' prospective observation study of routine clinical practice with dural puncture at a university hospital was conducted. Quincke spinal needles 0.90 to 1.0 mm O.D. (20–19 g) were used for diagnostic lumbar puncture, 0.70 mm O.D. (22 g) for myelography and 0.40 to 0.50 mm O.D. (27–25 g) for spinal anaesthesia. A questionnaire about post-puncture discomfort was given to the patients, to be returned after 1 week. Results – Of 679 questionnaires 537 (79.1%) were returned. Discomfort was experienced by 53.8% of the patients, most often after diagnostic lumbar puncture and myelography. The difference in incidence of headache after diagnostic lumbar puncture and myelography compared with spinal anaesthesia were 27.9% (95% CI: 18.6 to 37.2) and 18.3% (95% CI: 9.1 to 27.5). Conclusion – Small diameter and atraumatic spinal needles will reduce patients' discomfort after dural puncture.     

Contribution of multiple thrombophilic and transient risk factors in the development of cerebral venous thrombosis

INTRODUCTION: Cerebral venous thrombosis (CVT), deep vein thrombosis (DVT) and/or pulmonary embolism (PE) have been associated with thrombophilic defects. However, in contrast to DVT or PE, CVT is a rare disease. We performed a study to identify differences in thrombotic risk profile, predisposing to CVT rather than DVT or PE, particularly the contribution of oral contraception and 11 thrombophilic defects. MATERIALS AND METHODS: A single center case-control study (63 CVT cases and 209 controls with DVT or PE) was performed. RESULTS: Of CVT patients, 11% had experienced prior DVT or PE, and none had recurrent CVT at 5 years follow-up. CVT was more frequently observed in females (79% versus 51%, P<0.001). It was more often secondary (75% versus 50%, P<0.001), mainly due to the difference in age between both groups. At presentation of CVT and DVT/PE, oral contraceptives were used by 78% and 74% of non-pregnant fertile women (P=0.8), respectively. Any thrombophilic defect was demonstrated in 88% of CVT and 75% of DVT/PE patients (P=0.22), sex and age matched. Individual and two or more defects were equally distributed among both groups. CONCLUSIONS: We conclude that a majority of CVT and DVT or PE patients show single or multiple thrombophilic defects. At presentation, oral contraceptive intake was observed more frequently in CVT patients. However, no differences were observed in thrombotic risk profile between both groups of comparable age. Hence, additional unknown risk factors should be considered to explain the different sites of thrombosis in these patients.     

Cerebral venous thrombosis--clinical aspects and consequences

The clinical picture of cerebral venous thrombosis (CVT) depends on the site of thrombosis in the venous system including superficial veins, deep veins and venous sinuses. Thrombotic changes may occur simultaneously in various parts of the venous system. Since CVT may have various causes, the knowledge of its etiology helps to make the diagnosis. In systemic diseases multiple intravascular clots may result, while in localized pathological conditions thrombosis maybe restricted to the lesion site. The clinical picture is often serious, leading to death, or to severe complications such as pulmonary embolism, and to distant complications--like epilepsy or intracranial hypertension being the cause of chronic headaches (lumbar puncture and CSF pressure measurement are helpful in the diagnosis of intracranial hypertension). In order to prevent complications of crucial importance is not only the proper diagnosis (with MRI and venography as the diagnostic techniques of choice), but also an early and prolonged treatment with anticoagulants. Heparin or fractionated heparin is recommended even though there is a possibility of cerebral haemorrhagic lesions.     

Simultaneous thrombosis of cerebral artery and venous sinus

Cerebral venous thrombosis (CVT) is infrequent among cerebrovascular diseases. The simultaneous thrombosis involving both cerebral artery and venous sinus is even extremely rare. We reported a 41-year-old woman who presented with acute headache and left hemiparesis due to concomitant arterial ischemic stroke and recurrent CVT. Extensive investigation disclosed acquired protein C and protein S deficiency, iron deficiency anemia (IDA) and cryoglobulinemia. She was treated with intravenous injection of heparin followed by oral anticoagulant therapy. The headache rapidly subsided; however, left hemiparesis persisted over five months. The rare condition of simultaneous thrombosis of cerebral artery and venous sinus may be caused by the synergistic effect of coagulation disorders, IDA and cryoglobulinemia.     

Cerebral venous thrombosis: a rare complication of herpes simplex encephalitis

We report a case of classic HSE with early neurological relapse 7 days after onset of acyclovir treatment secondary to cerebral venous thrombosis (CVT). The development of CVT after meningoencephalitis has been described with neurotropic viruses such as HSV, HIV, or enteroviruses and also bacterial or fungal agents. CVT is probably the consequence of the inflammation secondary to these infections. A diagnosis of CVT, although rarely described, should be systematically suspected in patients with HSE who present no or only moderate improvement, or early relapse of symptoms despite adapted acyclovir treatment.

     

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.