This type of cerebral cortical infarction and hemorrhage, the etiology is too rare...

For reference only by medical professionals When clinically encountering headaches, focal neurological deficits, epilepsy, or patients with such imaging findings, the possibility of cortical venous thrombosis should be considered
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Intracranial venous thrombosis (CVT) refers to the thrombosis of intracranial veins or venous sinuses caused by various causes, which obstructs blood return or cerebrospinal fluid circulation, leading to intracranial hypertension and focal brain damage.
A type of cerebrovascular disease, accounting for about 0.
5%-1% of all cerebrovascular diseases
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Case Information A 23-year-old female, right-handed, presented to the emergency department with persistent dull pain in the right forehead for 2 days after aerobic exercise
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History of present illness: On admission, the patient had 2 right-hand tonic-clonic seizures, each lasting less than 1 minute, with unconsciousness
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Right-sided facial paralysis, numbness and weakness of the right upper extremity occurred after the second attack
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Past history of migraine without aura
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Denied drinking and smoking history
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The only drug use is estrogen-containing oral contraceptives
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Physical examination: Vital signs on admission were normal
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Neurological examination: expressive aphasia and mild dysarthria
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The right nasolabial fold is slightly shallower
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The proximal muscle strength of the right upper extremity was grade 4, the wrist muscle strength was grade 1, and the finger muscle strength was grade 0
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Laboratory tests: thrombocytosis (496 × 103/μL)
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Basic metabolic screening, prothrombin time/activated partial thromboplastin time (PT/APTT), international normalized ratio (INR) normal range
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Urine drug tests and pregnancy tests were negative
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Auxiliary examinations: CT of the brain showed left frontal convexity vein infarction with peripheral edema, and high-density creeping blood vessels in the left frontal lobe, consistent with cortical venous thrombosis (“strip sign”) (Figures A, B)
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Cortical vein thrombosis was confirmed by CTV, and other venous systems were unobstructed (Panel C)
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Image from reference The patient was subsequently admitted to the NICU, and DSA examination further confirmed isolated cortical venous thrombosis, excluding other vascular diseases
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Susceptibility-weighted imaging (SWI) showed a bloody infarct (Panel D), and magnetic resonance imaging with fluid-attenuated inversion recovery sequence (FLAIR) showed a small amount of edema around the blood (Panel E)
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Image from reference Hypercoagulability screening includes lupus anticoagulant, high homocysteine, thrombin time, antithrombin activity, prothrombin mutation, factor V Leiden mutation, anticardiolipin antibody, protein S all No abnormality was seen
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Protein C activity was initially detected when patients were taking warfarin, resulting in low levels
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Platelets were elevated on admission and subsequently returned to normal
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CT of the chest, abdomen and pelvis showed no evidence of tumor
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The initial treatment is unfractionated heparin, followed by low molecular weight heparin and transition to oral warfarin, with a target INR of 2-3
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Short-term dexamethasone is used to treat cerebral edema, while oral contraceptives are discontinued
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On day 12 of rehabilitation, the patient presented with progressive bilateral frontotemporal throbbing headache with phonophobia and photophobia, and bilateral papilledema
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Physical examination showed no progression of neurological deficits
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CT showed progression of venous infarction and expansion of surrounding edema, resulting in mass effect and midline shift (Panel F)
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MRI and MRV showed a new cortical venous thrombosis in the left frontal lobe, and the dural venous sinus was open
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Image from Ref.
Patient re-entry into the NICU
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It is worth noting that the cortical venous thrombosis was still progressing despite the INR monitoring target, and the treatment was discontinued from oral warfarin and changed to low molecular weight heparin
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Since then, the patient's symptoms have gradually improved, and rehabilitation training has continued after discharge
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Protein C activity was normal after several weeks of discontinuation of warfarin
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Follow-up CT at 3 weeks after discharge showed that the venous infarction had resolved
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Understanding ICVT▎Differentiating ICVT from CVSTIsolated cortical vein thrombosis (ICVT) is a rare vascular disease caused by thrombosis in one or more cortical veins without dural venous sinus or deep vein thrombosis
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Intracranial venous sinus thrombosis (cerebral venous and sinus thrombosis, CVST) is a thrombus involving the venous sinuses and deep cerebral veins
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Therefore, the two are different concepts
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Both CVST and ICVT are more common in women and may be due to sex-specific risk factors such as oral contraceptives and pregnancy
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Increased intracranial pressure is less common with ICVT than with CVST, but parenchymal lesions (venous infarction, hemorrhagic infarction, or vasogenic edema) are more common in ICVT
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▎The etiology of ICVT The etiology of ICVT is more complicated, and most studies believe that it is consistent with the etiology of CVST
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The state of the blood, the changes of the blood vessel wall and the changes of blood components are the direct causes of its pathological changes
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Risk factors for ICVT can be divided into two categories: acquired (trauma, surgery, pregnancy, puerperium, anticardiolipin syndrome, tumors, and exogenous steroid use) and hereditary (hereditary thrombotic states)
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▎Clinical manifestations Focal neurological deficits are the most common manifestations of ICVT, including sensory loss, aphasia, hemiplegia, and dyslexia
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In addition, focal epilepsy and headache can also be present, and intracranial hypertension and disturbance of consciousness are rare
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Imaging recognition of ICVT Imaging technology is helpful for the diagnosis of ICVT, and CT "strip sign" suggests venous thrombosis
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Magnetic resonance venography (MRV) does not reliably identify ICVT due to cortical vein size and anatomical variability
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CT venography (CTV) is similarly limited but can show a hypodense filling defect, corresponding to the hyperdense streak sign on CT
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Due to the ferromagnetism of blood degradation products, T1 and T2 images take at least 5 days to detect thrombosis
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Conversely, SWI can show early thrombosis, manifesting as a marked tubular hypointense
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Intravenous hyperintense thrombus on DWI may be useful if more sensitive sequences are not available
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Digital subtraction angiography (DSA) can be helpful when other investigations are insufficient and can also rule out other vascular diseases, such as arteriovenous malformations and arteriovenous fistulas
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Treatment of ICVT Anticoagulation is the cornerstone of CVST treatment
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The international ISCVT study found that compared with unfractionated heparin, patients treated with low molecular weight heparin were more likely to achieve 6 months of functional independence and less new intracranial hemorrhage
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To rapidly reach therapeutic levels in the acute phase, unfractionated heparin has long been used as first-line therapy
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However, this rarely happens as it often takes more than 24 hours to reach the desired level
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The advantages of low molecular weight heparin lie in its long plasma half-life, more stable therapeutic effect, and lower risk of heparin-induced thrombocytopenia
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Other treatment options are novel oral anticoagulants, but there are currently no randomized controlled trials using novel oral anticoagulants for CVST or ICVT
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For the treatment time, please refer to the Chinese Guidelines for Diagnosis and Treatment of Intracranial Venous Thrombosis 2019
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For patients with unknown risk factors or mild hereditary thrombophilia, oral anticoagulation therapy should be continued for 6-12 months; for patients with more than 2 episodes or severe hereditary thrombophilia, long-term anticoagulation therapy may be considered; For those with rapidly controllable risk factors, such as pregnancy, oral hormonal contraceptives, anticoagulation therapy can be within 3 months
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It is well known that a transient prothrombotic state can occur when warfarin therapy is initiated due to reductions in vitamin K-dependent procoagulants, including protein C levels
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The prothrombotic state subsided after the anticoagulation therapy reached the target monitored by INR, and heparin bridging was stopped at this time
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It has been reported in the literature that despite the use of adequate warfarin doses and the INR is controlled within the recommended range, there is still warfarin ineffectiveness leading to recurrence of thrombosis
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Possible causes are pharmacokinetic (decreased absorption or increased elimination) or pharmacodynamics (excessive vitamin K intake, hyperlipidemia, or genetics)
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We report a case of a young female patient who developed ICVT with delayed recurrence in the absence of any underlying thrombus or malignancy despite adequate anticoagulation and INR control
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Considering the possible ineffectiveness of warfarin, close clinical and imaging monitoring is recommended when the anticoagulation regimen is changed
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Nodular cortical venous thrombosis is very rare in clinical practice, and it is easy to be missed and misdiagnosed
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When clinically encountering patients with headache, focal neurological deficit, epilepsy, or imaging of parenchymal hemorrhagic infarction, as well as patients with imaging manifestations such as convex subarachnoid hemorrhage, it should be considered that cortical vein thrombosis may be possible , early diagnosis and timely treatment are beneficial to the prognosis of patients
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References: [1].
Pearls & Oy-sters: Delayed progression of isolated cortical vein thrombosis despite therapeutic INR.
Neurology 2018;90:e727-e730.
doi:10.
1212/WNL.
000000000000499.
[2].
Intracranial vein thrombosis in China Formation of Diagnosis and Treatment Guidelines 2019.
Note: The content of this article is mainly translated from an article published in Neurology in 2018 (title: Delayed progression of isolated cortical vein thrombosis despite therapeutic INR.
)