Cerebral venous thrombosis as a rare thromboembolic complication of celiac disease: a case report

Abstract Background Celiac disease is a digestive inflammatory syndrome with several complications. It is associated with coagulation and platelets abnormalities leading to thromboembolic events. Cerebral venous thrombosis is an exceptional localization of thrombosis in celiac disease and could be life-threatening. Case summary A 17-year-old female patient with history of celiac disease and not following a gluten-free diet, checked in to the emergency department for a sudden, 2-week-old, and deteriorating, onset of intense headache and muscle weakness. The cerebral computed tomography-scan showed bilateral fronto-parietal hypodensity with micro-bleeds. We investigated using a cerebral magnetic resonance imaging that revealed superior longitudinal sinus thrombosis and right transverse and sigmoid sinuses thrombosis, along with right haematoma and ischaemic areas. The patient was prescribed anticoagulation therapy. Follow-ups over a 2-year period confirmed a favourable outcome and a complete regression of symptoms. Discussion Evolution of celiac disease could be associated with several complications. Eighty-five percent of patients is potentially exposed to thromboembolic events due to the hypercoagulability state of the disease and different coagulation and fibrinolysis abnormalities (e.g. hyperhomocysteinaemia, protein C and S deficiencies, vitamin K and B deficiencies). Cerebral venous thrombosis is a rare thromboembolic localization. Anticoagulation is efficient in most cases though endovascular treatment might be required.

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Abstract 1122‐000044: Endovascular Thrombectomy for Straight Sinus Thrombosis

Stroke: Vascular and Interventional Neurology ◽

10.1161/svin.01.suppl_1.000044 ◽

2021 ◽

Vol 1 (S1) ◽

Author(s):

Stefano H Byer ◽

Shweta Goswami ◽

Abid Y Qureshi ◽

Michael G Abraham

Keyword(s):

Venous Thrombosis ◽

Cerebral Venous Thrombosis ◽

Sinus Thrombosis ◽

Vasogenic Edema ◽

Anticoagulation Therapy ◽

Clinical Signs ◽

Mri Brain ◽

Straight Sinus ◽

Significant Difference ◽

Level Of Consciousness

Introduction : Cerebral venous thrombosis (CVT) is an under‐recognized disease that accounts for roughly 1% of all strokes 1 . It presents a diagnostic challenge due to its varied and ambiguous presentation, which on average, delays diagnosis by 7 days. Pre‐disposing factors include trauma, pregnancy, dehydration, and medications such as oral contraceptives. In one study, 50% of CVT occurred during pregnancy or puerperium 2 . Seven out of 8 cases of CVT presented postpartum among 50,700 deliveries in Canada 3 . Clinical signs and symptoms range from a mild headache to decreased level of consciousness depending on the location of thrombosis. Up to 13% of individuals can have poor outcomes despite anticoagulation therapy. Methods : A 28‐year‐old, 7‐week postpartum female awoke with a headache and difficulty speaking, followed by urinary and bowel incontinence. Her clinical status worsened and was admitted to an outside hospital where CT‐head without contrast revealed right temporal lobe hypodensity. A hyperdense straight sinus sign was present but not recognized at that time. She was transferred to our hospital and MRI brain demonstrated extensive vasogenic edema in the basal ganglia, thalami, and deep white matter with cytotoxic edema in bilateral watershed areas from severe hydrocephalus. MR‐Venography showed extensive cerebral venous thrombosis in the inferior sagittal sinus, vein of Galen, straight sinus, and left transverse and sigmoid sinuses. Upon transfer to our facility, NIHSS was 9 for decreased level of consciousness and aphasia with episodes of left‐sided clonic movements. Despite adequate anticoagulation therapy, she continued to decline with extensor posturing and a comatose state. Results : Since the findings on MRI‐brain were predominantly vasogenic edema, thrombectomy was performed with a stent‐retriever and aspiration, with complete recanalization of her straight sinus. Subsequent MRIs demonstrated improvement and resolution of the edema and hydrocephalus. Hypercoagulable work‐up revealed an elevated protein C and antithrombin III and she was transitioned to enoxaparin and discharged to LTACH. At four‐month follow‐up she was able to speak and walk with physical therapy. Conclusions : We highlight the importance of early recognition of deep venous thrombosis as it commonly affects level of consciousness. A subtle finding, it should be in the differential diagnosis of alteration in level of consciousness without obvious neuroimaging findings. The AAN guidelines for management of CVT do not advocate for thrombectomy in all patients as large randomized controlled trials do not currently exist. However, they recognize that thrombectomy may be considered if deterioration occurs despite intensive anticoagulation treatment. The TO‐ACT trial found no significant difference in mortality between intervention and medical therapy, aggressive intervention with thrombectomy prevented a larger stroke burden in our patient. Therefore, thrombectomy should be considered in patients suffering from CVT, particularly in refractory and extensive cases.

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Anesthetic Management of a Child with β-Thalassemia Major and Cortical Venous Thrombosis

Journal of Neuroanaesthesiology and Critical Care ◽

10.1055/s-0040-1715357 ◽

2020 ◽

Author(s):

Mayank Tyagi ◽

Surya K. Dube ◽

Vanitha Rajagopalan ◽

Gyaninder P. Singh

Keyword(s):

Venous Thrombosis ◽

Sinus Thrombosis ◽

Anesthetic Management ◽

Thalassemia Major ◽

Severe Form ◽

Thromboembolic Events ◽

Blood Disorders ◽

Decompressive Hemicraniectomy ◽

Cerebral Sinus ◽

Cortical Venous Thrombosis

Abstractβ-thalassemia are a group of inherited blood disorders with reduced hemoglobin levels. β-thalassemia major is the severe form of disease, and the patients often display an array of associated organ dysfunction which thus increase the risk associated with surgery and anesthesia. Patients with β-thalassemia major can have multiple pathological defects that may lead to thromboembolic events. Here, we report such a case who was complicated by occurrence of cerebral sinus thrombosis and presented for decompressive hemicraniectomy under general anesthesia. The anesthetic challenges during in such scenario have been discussed.

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Scrub typhus with cerebral venous sinus thrombosis: a rare presentation

BMJ Case Reports ◽

10.1136/bcr-2020-241401 ◽

2021 ◽

Vol 14 (4) ◽

pp. e241401

Author(s):

Sayonee Das ◽

Sidhartha Chattopadhyay ◽

Kausik Munsi ◽

Sagar Basu

Keyword(s):

Venous Thrombosis ◽

Cerebral Venous Thrombosis ◽

Scrub Typhus ◽

Sinus Thrombosis ◽

Hormonal Contraception ◽

Neurological Involvement ◽

Rare Presentation ◽

Specific Test ◽

Satisfactory Outcome ◽

The Brain

This is a rare presentation of scrub typhus with cerebral venous thrombosis. A 32-year-old woman presented with signs of raised intracranial tension. Examination revealed maculopapular skin rashes and an ‘eschar’ over the right thigh. Nuchal rigidity and bilateral papilloedema were found. Scrub typhus was diagnosed by the presence of IgM antibody in serum. CT scan of the brain showed cerebral oedema. MRI of the brain was normal. Magnetic resonance venography of the brain showed thrombosis of several venous sinuses. Cerebrospinal fluid analysis revealed lymphocytic pleocytosis with raised protein level. Other causes of prothrombotic states were ruled out by doing specific test results. There was no history of hormonal contraception and prolonged bed rest. A case of scrub typhus complicated with meningoencephalitis and cerebral venous thrombosis was diagnosed. She responded to treatment with doxycycline, anticoagulants, antipyrectics and intravenous saline. Early identification of such atypical neurological involvement in scrub typhus was helpful in satisfactory outcome.

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Cerebral Venous Thrombosis in an Adolescent with Celiac Disease

Scholars Journal of Medical Case Reports ◽

10.36347/sjmcr.2020.v08i04.024 ◽

2020 ◽

Vol 08 (04) ◽

pp. 502-504

Author(s):

Sara Sabiry ◽

Salma Louzi ◽

Zainab Abdulhakeem ◽

Hicham EL Otmani ◽

Mohamed Abdoh Rafai ◽

...

Keyword(s):

Celiac Disease ◽

Venous Thrombosis ◽

Cerebral Venous Thrombosis

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Cerebral venous thrombosis and Covid 19: Literature review

Journal of Neurology & Stroke ◽

10.15406/jnsk.2021.11.00457 ◽

2021 ◽

Vol 11 (2) ◽

pp. 57-68

Author(s):

Carlos Alexandre Martins Zicarelli ◽

Jonathan Vinicius Martins ◽

Wesley Vieira Doni ◽

Rafael Rodrigues Pinheiro dos Santos ◽

Ana Carla Mondek Rampazzo ◽

...

Keyword(s):

Literature Review ◽

Venous Thrombosis ◽

Cerebral Venous Thrombosis ◽

Sinus Thrombosis ◽

Rare Complication ◽

Clinical Manifestations ◽

Risk Of Death ◽

Cranial Sinus Thrombosis ◽

Stent Retrievers ◽

Low Incidence

Introduction: Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) was first detected in December 2019 in the city of Wuhan, China, and has since taken on worldwide proportions. It is known that individuals with Coronavirus disease-19 (COVID-19) have systemic clinical manifestations. Among the multisystemic effects, cerebral venous thrombosis (CVT) is responsible for high mortality rates. In this sense, understanding the association between CVT and SARS-CoV-2 infection directly impacts the disease's morbidity and mortality. Methodology: Literature review in the PubMed and Embase databases, with the following search terms: “COVID-19”, “SARS-CoV-2”, “Venous thromboembolism”, “Thrombosis”, “Cerebral Venous Thrombosis”, “Intracranial Sinus Thrombosis” and “Cranial Sinus Thrombosis”. The selected articles were written in English, which addressed the various aspects of COVID-19. Results and discussion: CVT are a rare complication of COVID-19, with an incidence between 0.02 to 1% of hospitalized patients. However, it can reach about 75% of mortality in affected individuals. Pathophysiology seems to be associated with the state of hypercoagulability and the systemic inflammatory process resulting from viral infection. Thus, recent studies show a consensus on the early anticoagulation of patients affected by the virus, to reduce mortality in these cases. However, the differences between the types of anticoagulation, Low Molecular Weight Heparin (LMWH), Unfractionated Heparin (UFH), Dabigatran have not yet been well established, although there is a predilection for the use of LMWH. Also, thrombectomy is a therapeutic intervention option that should be evaluated, due to the risk of additional endothelial injury from the use of stent retrievers. Conclusion: Although it has a relatively low incidence, CVT aggravates the condition and increases the risk of death for patients with COVID-19. Because of this, early diagnosis and evaluation of therapeutic options for CVT are essential for the development of clinical management.

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Risk factors and diagnosis of cerebral venous thrombosis: Data from a cohort of 45 Romanian patients

Acta Medica Marisiensis ◽

10.2478/amma-2014-0044 ◽

2014 ◽

Vol 60 (5) ◽

pp. 207-214

Author(s):

Rodica Bălaşa ◽

M Daboczi ◽

Oana Costache ◽

Smaranda Maier ◽

Z Bajko ◽

...

Keyword(s):

Risk Factors ◽

Venous Thrombosis ◽

Brain Imaging ◽

Cerebral Venous Thrombosis ◽

Sinus Thrombosis ◽

Demographic Data ◽

Brain Mri ◽

Clinical Aspects ◽

Initial Symptom ◽

Clinical Criteria

Abstract Cerebral venous thrombosis (CVT) represents 1% of the total stroke pathology but is a real challenge both regarding the diagnosis and the treatment. Objective: Evaluate different etiological, demographical, clinical, imaging and therapeutic aspects of CVT. Material: Prospective study during 4 years. From the total 3658 patients hospitalized with acute stroke, 45 (1.23%) had CVT. For each patient, were recorded: demographic data, symptom of onset, type of onset, daily habits, medical history, neurological examination, brain imaging (CT and MRI with venography). Statistical analysis: data are presented as mean and SD and Student t test was applied. Results: Mean age was 44.07± 23,12 years; female: male ratio 2.21:1. The most frequent type of onset was acute (77.78%). Headache was found in 80% of cases as initial symptom, followed by neurological focal deficits. As risk factors, thrombophilia was found most often (59.5%), followed by local infections. No risk factors were found in 17.8% of cases. The brain imaging was positive in 29 patients. In 16 cases, the imaging workout was negative and the diagnosis consisted of clinical criteria, risk factors, response to heparin treatment. Conclusions: CVT is a rare pathology that affects mainly young women and that needs a complex diagnostic evaluation. The patient prototype diagnosed with CVT in our region: female of 44 years old, with an intense acute headache, with MRI showing direct signs of transverse sinus thrombosis, with a thrombophilic state and good response to anticoagulants. Brain MRI is the imaging investigation required but clinical aspects play a decisive role.

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The Performance of CT versus MRI in the Differential Diagnosis of Cerebral Venous Thrombosis

Thrombosis and Haemostasis ◽

10.1055/s-0038-1642636 ◽

2018 ◽

Vol 118 (06) ◽

pp. 1067-1077 ◽

Cited By ~ 2

Author(s):

Weilin Xu ◽

Liansheng Gao ◽

Tao Li ◽

Neha Ramdoyal ◽

Jianmin Zhang ◽

...

Keyword(s):

Differential Diagnosis ◽

Diagnostic Accuracy ◽

Venous Thrombosis ◽

Cerebral Venous Thrombosis ◽

Sinus Thrombosis ◽

Meta Analysis ◽

Area Under The Curve ◽

Significant Heterogeneity ◽

Summary Receiver Operating Characteristic ◽

Ct And Mri

Background Cerebral venous thrombosis (CVT) is a rare disease, and with poor prognosis. Computed tomography (CT) and magnetic resonance imaging (MRI) are the most commonly used image modalities for patients with non-specific neurologic symptoms. We present here a meta-analysis to assess the accuracy of CT and MRI in the differential diagnosis of CVT and cerebral venous sinus thrombosis (CVST). Materials and Methods A comprehensive search of the PubMed, EMBASE, Web of Science, Cochrane Database and Chinese Biomedical (CBM) databases was conducted prior to March 20, 2017. In this report, we assess the methodological quality of each article individually and perform a meta-analysis to obtain the summary of the diagnostic accuracy of CT and MRI in correctly identifying CVT and CVST. Results Twenty-four eligible articles comprising 48 studies (4,595 cases) were included. The pooled sensitivity for CT–CVT/CT–CVST groups is 0.79 (95% confidence interval [CI]: 0.76, 0.82)/0.81(95% CI: 0.78, 0.84), and pooled specificity is 0.90 (95% CI: 0.89, 0.91)/0.89 (0.88, 0.91), with an area under the curve (AUC) for the summary receiver operating characteristic (SROC) of 0.9314/0.9161, respectively. No significant heterogeneity and publication bias was observed across each study. For MRI–CVT/MRI–CVST, the pooled sensitivity is 0.82 (95% CI: 0.78, 0.85)/0.80 (95% CI: 0.76, 0.83), and pooled specificity is 0.92 (95% CI: 0.91, 0.94)/0.91(0.89, 0.92), with an AUC for the SROC of 0.9221/0.9273, respectively. Conclusion This meta-analysis indicates that both CT and MRI have a high level of diagnostic accuracy in the differential diagnosis of CVT and CVST, independent of stage, target for analysis or analysis methods. They could be chosen as alternative sub-optimal gold standards for diagnosing CVT and CVST, especially in emergency.

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A young woman with headache and seizures

Italian Journal of Medicine ◽

10.4081/itjm.2008.4.25 ◽

2013 ◽

pp. 25-29

Author(s):

A.M. Pizzini ◽

M. Silingardi ◽

I. Iori

Keyword(s):

Venous Thrombosis ◽

Cerebral Venous Thrombosis ◽

Mr Angiography ◽

Sinus Thrombosis ◽

Factor V Leiden ◽

Transverse Sinus ◽

Acute Onset ◽

Factor V ◽

Neuroimaging Techniques

CASE REPORT We describe a 31 year-old woman with headache and acute onset of seizures. Medical history and physical examination were unremarkable. She has been on therapy with oral contraceptives for many years for dysmenorrhea. A CT scan was negative, but MRI and MR-angiography showed left transverse sinus thrombosis. Screening for thrombophilia revealed hyperhomocysteinemia and Factor V Leiden heterozigousity. The patient received unfractionated heparin, followed by long-term anticoagulation with warfarin (INR 2-3). CONCLUSIONS Cerebral venous thrombosis is a rare cerebrovascular disorder, frequently in young adult (about 75% are women). The diagnosis might be difficult with consequent high long-term morbidity and mortality rate. New neuroimaging techniques (MRI and MR-angiography) and more effective treatment (anticoagulation and endovascular thrombolysis) have improved the prognosis and the natural history. The risk factors, the clinical presentation, the diagnostic evaluation and the management of cerebral venous thrombosis are reviewed.

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