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BACKGROUND: Three-dimensional time-of-flight magnetic resonance angiography (TOF-MRA) is a largely adopted non-invasive technique for assessing cerebrovascular diseases. We aimed to optimize the 7-T TOF-MRA acquisition protocol, confirm that it outperforms conventional 3-T TOF-MRA, and compare 7-T TOF-MRA with digital subtraction angiography (DSA) in patients with different vascular pathologies. METHODS: Seven-tesla TOF-MRA sequences with different spatial resolutions acquired in four healthy subjects were compared with 3-T TOF-MRA for signal-to-noise and contrast-to-noise ratios as well as using a qualitative scale for vessel visibility and the quantitative Canny algorithm. Four patients with cerebrovascular disease (primary arteritis of the central nervous system, saccular aneurism, arteriovenous malformation, and dural arteriovenous fistula) underwent optimized 7-T TOF-MRA and DSA as reference. Images were compared visually and using the complex-wavelet structural similarity index. RESULTS: Contrast-to-noise ratio was higher at 7 T (4.5 ± 0.8 (mean ± standard deviation)) than at 3 T (2.7 ± 0.9). The mean quality score for all intracranial vessels was higher at 7 T (2.89) than at 3 T (2.28). Angiogram quality demonstrated a better vessel border detection at 7 T than at 3 T (44,166 versus 28,720 pixels). Of 32 parameters used for diagnosing cerebrovascular diseases on DSA, 27 (84%) were detected on 7-T TOF-MRA; the similarity index ranged from 0.52 (dural arteriovenous fistula) to 0.90 (saccular aneurysm). CONCLUSIONS: Seven-tesla TOF-MRA outperformed conventional 3-T TOF-MRA in evaluating intracranial vessels and exhibited an excellent image quality when compared to DSA. Seven-tesla TOF-MRA might improve the non-invasive diagnostic approach to several cerebrovascular diseases. RELEVANCE STATEMENT: An optimized TOF-MRA sequence at 7 T outperforms 3-T TOF-MRA, opening perspectives to its clinical use for noninvasive diagnosis of paradigmatic pathologies of intracranial vessels. KEY POINTS: ⢠An optimized 7-T TOF-MRA protocol was selected for comparison with clinical 3-T TOF-MRA for assessing intracranial vessels. ⢠Seven-tesla TOF-MRA outperformed 3-T TOF-MRA in both quantitative and qualitative evaluation. ⢠Seven-tesla TOF-MRA is comparable to DSA for the diagnosis and characterization of intracranial vascular pathologies.
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Angiografia Digital , Transtornos Cerebrovasculares , Angiografia por Ressonância Magnética , Humanos , Angiografia por Ressonância Magnética/métodos , Masculino , Feminino , Pessoa de Meia-Idade , Transtornos Cerebrovasculares/diagnóstico por imagem , Adulto , Angiografia Digital/métodos , Idoso , Razão Sinal-Ruído , Imageamento Tridimensional/métodosRESUMO
Cerebrofacial venous metameric syndrome (CVMS) is a complex low-flow vascular malformation affecting bone and soft tissues including brain, dura mater, and eye. We show images of CVMS in an 18-month-old boy presenting facial venous malformations, developmental venous anomalies, dural sinus malformations, and dilated great cerebral vein, suggesting a vein of Galen aneurysmal malformation. Although Sturge-Weber syndrome is the most known form of CVMS, its presentations are variable and include several venous malformations. Recognizing the various manifestations of CVMS is necessary for adequate screening, treatment, and follow-up.
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Prompt medical attention is crucial for congenital intracranial vascular malformations in children and newborns due to potential severe outcomes. Imaging is pivotal for accurate identification, given the diverse risks and treatment strategies. This article aims to enhance the identification and understanding of congenital intracranial vascular abnormalities including arteriovenous malformation, arteriovenous fistula, cavernous malformation, capillary telangiectasia, developmental venous anomaly, and sinus pericranii in pediatric patients.
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Pediatric central nervous system (CNS) vascular malformations are a group of abnormal blood vessel formations within the brain or spinal cord in children. The most crucial point of pediatric CNS vascular malformation is that no golden standard classifications exist. In addition, there is a big gap in knowledge and the viewpoint of clinicians, radiologists, and pathologists. In addition, many genes associated with pediatric CNS vascular malformation, such as Sturge-Weber-Dimitri syndrome with guanine nucleotide-binding protein G(q) subunit alpha (GNAQ) gene mutation, and cavernous malformations with cerebral cavernous malformations 1 (CCM1), CCM2, and CCM3 gene mutation, were recently revealed. For proper therapeutic approaches, we must understand the lesions' characterizations in anatomical, morphological, and functional views. In this review, the author would like to provide basic pediatric CNS vascular malformation concepts with understandable diagrams. Thus, the author hopes that it might be helpful for the proper diagnosis and treatment of CNS pediatric vascular malformations.
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Giant cerebellar cavernomas in children are rare and must be differentiated from hemorrhagic cerebellar tumors. The diagnosis and treatment of giant cerebellar cavernomas is challenging, but complete surgical resection can lead to favorable outcomes and complete neurological recovery in most cases. We present a case of eight months old baby who was diagnosed with a giant cavernoma resulting in secondary obstructive hydrocephalus with neuropsychiatric presentations. The patient underwent a paramedian craniotomy surgery with a suboccipital approach and complete surgical resection of the cavernoma was done. Over nine months of observation, the child showed improvement in their ability to walk and fully recovered from a neurological perspective. We also conducted a literature review to identify eleven cases of giant cerebellar cavernomas in children, including our case. The data were analyzed to determine the clinical features, treatment, and outcomes of giant cerebellar cavernomas in children.
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A previously healthy 21-year-old Caucasian female G1P0 at 32 weeks gestation presented to the ED for an episode of syncope. She also complained of headaches, neck pain, and blurry vision. Physical examination revealed a healthy pregnant female. Neurological examination demonstrated Grade III papilledema but was otherwise unremarkable. CT brain revealed hydrocephalus and intraventricular hemorrhage of unclear etiology MRI of the head was negative for a mass lesion. MRA/MRV of the head was negative, ruling out cavernous sinus thrombosis. Lumbar puncture was bloody but negative for infection. Infectious workup, including HSV, toxoplasmosis, and neurocysticercosis, was negative. An intraventricular drain was placed for hydrocephalus. While in the hospital, she developed sudden left-sided weakness, prompting an emergency C-section. Further workup with CT angio of the brain and neck revealed an arteriovenous malformation (AVM) involving the anterior spinal artery and adjacent venous plexus. Digital subtraction angiography showed a C2-3 pial AVM with a partially thrombosed nidal aneurysm. She was transferred to an outside hospital for embolization. Embolization obliterated the aneurysm, but residual flow remained in the AVM. Blood products are visible on sagittal MRI after embolization. At hospital discharge, her left-sided weakness had resolved, and her neurological examination was normal. The hydrocephalus had resolved.
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Dural arteriovenous fistula (DAVF) is a rare condition affecting approximately 1.5% of 1,000,000 individuals annually. It frequently occurs in the transsigmoid and cavernous sinuses. An isolated sigmoid sinus is extremely rare and is treated by performing transfemoral transvenous embolization along the opposite transverse sinus. A 69-year-old woman presented with asymptomatic Borden type III/Cognard type III DAVF involving an isolated sigmoid sinus. She underwent a staged operation in which a navigation system was used to expose the sigmoid sinus in the operating room before transferring the patient to the angio suite for transvenous embolization. Various modalities have been used to treat DAVF, including surgical disconnection, transarterial embolization, transvenous embolization, and stereotactic radiosurgery. However, treating DAVF cases where the affected sinus is isolated can be challenging because an easily accessible surgical route may not be available. In this case, direct sinus cannulation and transvenous embolization were the most effective treatments.
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Fístula Arteriovenosa , Malformações Vasculares do Sistema Nervoso Central , Lactente , Humanos , Cavidades Cranianas/diagnóstico por imagem , Cavidades Cranianas/cirurgia , Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/cirurgia , Malformações Vasculares do Sistema Nervoso Central/diagnóstico por imagem , Malformações Vasculares do Sistema Nervoso Central/cirurgia , Angiografia CerebralRESUMO
Intracranial non-galenic pial arteriovenous fistula (PAVF) is an extremely rare vascular malformation, where one or more pial arteries feeds directly into a cortical vein without any intervening nidus. Though occasionally they can be asymptomatic, neurological symptoms such as headache, seizure, or focal neurological deficit are more common presenting features. Life threatening or fatal hemorrhage is not uncommon, hence needed to be treated more often than not. Spontaneous occlusion of PAVF is reported only four times before. We report a 49-year-old gentleman, who was diagnosed to have a PAVF, possibly secondary to trauma. He presented 5 months and 22 days from initial digital subtraction angiography (DSA) for treatment, and follow-up angiogram showed complete obliteration. He denied any significant event, medication or alternate treatment during this period. His clinical symptoms were stable as well. We postulate iodinated contrast medium induced vasculopathy as a possible cause, which has been described for other vascular pathologies, but never for PAVF.
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Knowledge of the venous anatomy is essential for appropriately treating dural arteriovenous fistulas (AVFs). It is challenging to determine the overall venous structure despite performing selective angiography for dural AVFs with feeder from multiple selected arteries. This is because only a part of the veins can be observed through the shunt in the selected artery. Therefore, after performing selective angiography of all vessels to understand the approximate venous anatomy, the venous anatomy can be easily understood by closely examining the source image of computed tomographic angiography or magnetic resonance angiography. Through this, it is possible to specify the vein that is to be blocked (target embolization), thereby avoiding extensive blocking of the vein and avoiding various complications. In the case of dural AVF with feeder from single selected artery, if the multiplanar reconstruction image of the three-dimensional rotational computed tomography obtained by performing angiography is analyzed thoroughly, a shunted pouch can be identified. If embolization is performed by targeting this area, unnecessary sinus total packing can be avoided.
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ABSTRACT BACKGROUND: Embolization is a promising treatment strategy for cerebral arteriovenous malformations (AVMs). However, consensus regarding the main complications or long-term outcomes of embolization in AVMs remains lacking. OBJECTIVE: To characterize the most prevalent complications and long-term outcomes in patients with AVM undergoing therapeutic embolization. DESIGN AND SETTING: This systematic review was conducted at the Federal University of Alagoas, Arapiraca, Brazil. METHODS: This systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria. Data were obtained from MEDLINE, PubMed, LILACS, and SciELO databases, which included the epidemiological profile of the population, characteristics of the proposed therapy, complications (hemorrhagic events and neurological deficits), and long-term outcomes (modified Rankin scale pre- and post-treatment, AVM recanalization, complete obliteration, and deaths). RESULTS: Overall, the analysis included 34 articles (2,799 patients). Grade III Spetzler-Martin AVMs were observed in 34.2% of cases. Notably, 39.3% of patients underwent embolization combined with radiosurgery. The most frequently reported long-term complication was hemorrhage, which occurred in 8.7% of patients at a mean follow-up period of 58.6 months. Further, 6.3% of patients exhibited neurological deficits after an average of 34.7 months. Complete obliteration was achieved in 51.4% of the cases after a mean period of 36 months. Recanalization of AVMs was observed in 3.5% of patients. Long-term death occurred in 4.0% of patients. CONCLUSION: Embolization of AVMs is an increasingly safe strategy with low long-term complications and satisfactory outcomes, especially in patients who have undergone combination therapies. SYSTEMATIC REVIEW REGISTRATION: https://www.crd.york.ac.uk/prospero/ Registration number CRD42020204867.
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Unilateral subcortical calcifications are unique radiographic findings indicating specific focal pathologies. When the lesion is accompanied by edema, cerebral neoplasm usually leads to a differential diagnosis. This report presents a case of unilateral subcortical calcification and edema that resulted in cerebral hemorrhage and a subsequent diagnosis of an aggressive dural arteriovenous fistula. A man in his 60s presented with left hemianopsia and a progressive headache for over 6 months. Initial computed tomography revealed unilateral subcortical calcification and cerebral edema in the right occipital lobe, raising the suspicion of oligodendroglioma. However, 10 days later, a cerebral hemorrhage occurred in the lesion. Magnetic resonance imaging revealed flow void clusters and dilatation of the bilateral external carotid arteries and cortical veins, indicating a dural arteriovenous fistula. Cerebral angiography confirmed the presence of a parasagittal dural arteriovenous fistula (Borden type III). The patient was successfully treated with trans-arterial embolization using Onyx. Thus, calcifications with edema are more commonly associated with cerebral neoplasms; however, in this case, they indicated the presence of a dural arteriovenous fistula with severe corticovenous reflux. The presented case highlights the importance of recognizing these imaging features in dural arteriovenous fistulas and raises awareness of the potential danger of early hemorrhage after diagnosis. Therefore, timely evaluation of cranial vessels is essential in cases of unilateral subcortical calcification and edema to facilitate the early detection and management of aggressive dural arteriovenous fistulas.
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Dural supply from the external carotid system in cerebral arteriovenous malformations (AVMs) is well known, but actual angiographic evidence of dural supply to spinal cord AVMs (SCAVMs) has not been reported. Here, we report a case of dural supply to the conus SCAVM in the spinal arteriovenous metameric syndrome segment 25-30. Thirteen years after spinal surgery (T12-L2 laminoplasty), spinal angiography showed multiple dural supplies from the dorsal somatic branches, prelaminar arteries, and radiculomeningeal arteries to the SCAVM at the level of the previous spinal surgery. To the best of our knowledge, this is the first reported case with dural supply to the spinal cord. This case demonstrates that the extradural and extraspinal branches can supply the spinal cord in rare instances of spinal dural adhesions following repeated hemorrhages and surgical intervention under a metameric link background.
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Malformações Vasculares do Sistema Nervoso Central , Malformações Arteriovenosas Intracranianas , Humanos , Medula Espinal/diagnóstico por imagem , Medula Espinal/cirurgia , Artérias , Angiografia , Procedimentos Neurocirúrgicos , Síndrome , Malformações Vasculares do Sistema Nervoso Central/diagnóstico por imagem , Malformações Vasculares do Sistema Nervoso Central/cirurgiaRESUMO
Spinal osseous arteriovenous fistula (AVF) is a rare disease with a characteristic angioarchitecture involving an intraosseous venous pouch (VP) of the vertebral body where the feeders are converging. Using spinal angiography alone, it is difficult to distinguish spinal osseous AVF from classical spinal epidural AVF (EDAVF) with fistulas in the epidural VP and bone erosion because both subtypes have an angiographically similar dilated VP appearance. Thus, spinal osseous AVF may be often misdiagnosed as spinal EDAVF. With advancing imaging techniques, it would be possible to determine the exact location of the fistula. Here, we present the case of a 37-year-old woman with a pure spinal thoracic osseous AVF and radiculopathy. She was diagnosed with spinal intraosseous AVF using high-resolution three-dimensional rotational angiography (3D-RA). The fistula was located in the Th1 lateral mass with a VP where multiple osseous feeders were converging. There was paravertebral venous drainage without intradural venous drainage. Transvenous embolization with Onyx and coils was performed through the azygos vein to the lateral epidural venous plexus, resulting in complete obliteration. This case suggests that 3D-RA reconstructed images are essential for accurate diagnosis and successful treatment of this condition. It is important to occlude only intraosseous VPs by accurate subtype diagnosis. Transvenous embolization is a treatment option for spinal intraosseous AVF with paravertebral epidural venous drainage.
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Fístula Arteriovenosa , Embolização Terapêutica , Feminino , Humanos , Adulto , Imageamento por Ressonância Magnética/efeitos adversos , Embolização Terapêutica/métodos , Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/cirurgiaRESUMO
Objective:To investigate the imaging characteristics and diagnosis of thalamic dementia caused by medial tentorial dural arteriovenous fistula (TDAVF).Methods:The clinical and imaging data of 4 patients with medial TDAVF diagnosed at Nanjing Brain Hospital from August 2022 to September 2023 were retrospectively collected, summarized and analyzed.Results:Four patients were all males, with an average age of 67 years. The duration of cognitive impairment varies from 13 days to 2 months, with an average of about 1 month. The average score on the Mini-Mental State Examination (MMSE) was 13, while the average score on the Montreal Cognitive Assessment (MoCA) was 15. One patient was unable to complete these tests. MRI showed bilateral thalamic swelling, with scattered microbleeding lesions on diffusion-weighted imaging and susceptibility-weighted imaging ( n=3). Enhanced scans showed patchy or clumpy enhancement of the bilateral thalamus. Magnetic resonance angiography showed abnormal blood vessels along the straight sinus area ( n=4), while magnetic resonance venography showed no straight sinus development ( n=4). Magnetic resonance spectroscopy showed neuronal swelling and damage ( n=3), with no increase in choline peak. Arterial spin labeling perfusion imaging showed decreased perfusion in the lesions. Digital subtraction angiography confirmed TDAVF with straight sinus thrombosis. Two patients underwent embolization, with significant improvement in postoperative memory impairment. One patient underwent surgical clipping, while the other was transferred to other hospital for treatment. Conclusions:Patients with medial TDAVF often present with thalamic dementia. Imaging examinations show typical bilateral thalamic swelling with microbleeds, and early visualization of vascular shadows, without development of straight sinus. These features would be beneficial for the early diagnosis of TDAVF.
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Objective To observe the therapeutic effect of endovascular embolization for perimedullary arteriovenous fistula(PMAVF).Methods Data of 8 PMAVF patients who underwent endovascular embolization were retrospectively analyzed.Findings of digital subtraction angiography(DSA)immediately and 6 months after treatment were reviewed,and the modified Aminoff-Logue score(mALS)was compared before and 6 months after treatment.Results Among 8 cases of PMAVF,there were 2 cases of type Ⅰ,5 cases of type Ⅱand 1 case of type Ⅲ,among them 3 were treated with Onyx glue while 5 with Glubrane glue.The immediate DSA results after treatment showed complete embolization of the fistula in all 8 cases,while those 6 months after treatment showed no recurrence.One case recovered and the other 7 cases improved,and mALS(2.25±2.12)6 months after treatment was lower than that(5.50±2.39)before treatment(P<0.05).Conclusion Endovascular embolization could safely and effectively treat PMAVF.
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A vascular malformation is an abnormal development of blood vessels that can be found in arteries, veins, or the lymphatic system. While pathology such as aneurysms, arteriovenous malformations (AVMs), dissections, and dolichoectasia have been documented frequently and linked to an increased incidence of stroke, we present a rare finding of a 'corkscrew' appearance of the basilar artery causing recurrent posterior circulation symptoms. Imaging during 3 separate hospitalizations showed recurrent acute strokes within the basilar artery vascular territory. A conventional cerebral angiogram revealed a corkscrew appearance of the basilar artery with no dilatation, outpouching, or aneurysm.
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OBJECTIVE: Transvenous embolization (TVE) via an occluded inferior petrosal sinus (IPS) in a cavernous sinus dural arteriovenous fistula (CSDAVF) is challenging, often requiring navigation of a microcatheter through resistive obstacles between the occluded IPS and shunted pouch (SP), although the reopening technique was successfully performed. We report five cases of successful access to the cavernous sinus (CS) or SP using the rigid-tipped microguidewire such as chronic total occlusion (CTO) wire aiming to share our initial experience with this wire. METHODS: In this retrospective study, four patients with CSDAVF underwent five procedures using the CTO wire puncture during transfemoral transvenous coil embolization. Puncture success, shunt occlusion, and complications including any hemorrhage and cranial nerve palsy were evaluated. RESULTS: Despite successful access through the occluded IPS, further entry into the target area using neurointerventional devices was impossible due to a short-segment stricture before the CS (three cases) and a membranous barrier within the CS (two cases). However, puncturing these structures using the rigid-tipped microguidewire was successful in all cases. We could advance the microcatheter over the rigid-tipped microguidewire for the navigation to the SP and achieved complete occlusion of the SP without complications. CONCLUSION: The use of the rigid-tipped microguidewire in the TVE via the occluded IPS of the CSDAVF would be feasible and safe.
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BACKGROUND AND PURPOSE: To assess the long-term outcomes of intracranial dural arteriovenous fistula (DAVF) treated with stereotactic radiosurgery (SRS) alone or embolization and SRS (Emb-SRS) and to develop a grading system for predicting DAVF obliteration. METHODS: This multi-institutional retrospective study included 200 patients with DAVF treated with SRS or Emb-SRS. We investigated the long-term obliteration rate and obliteration-associated factors. We developed a new grading system to estimate the obliteration rate. Additionally, we compared the outcomes of SRS and Emb-SRS by using propensity score matching. RESULTS: The 3- and 4-year obliteration rates were 66.3% and 78.8%, respectively. The post-SRS hemorrhage rate was 2%. In the matched cohort, the SRS and Emb-SRS groups did not differ in the rates of obliteration (P=0.54) or post-SRS hemorrhage (P=0.50). In multivariable analysis, DAVF location and cortical venous reflux (CVR) were independently associated with obliteration. The new grading system assigned 2, 1, and 0 points to DAVFs in the anterior skull base or middle fossa, DAVFs with CVR or DAVFs in the superior sagittal sinus or tentorium, and DAVFs without these factors, respectively. Using the total points, patients were stratified into the highest (0 points), intermediate (1 point), or lowest (≥2 points) obliteration rate groups that exhibited 4-year obliteration rates of 94.4%, 71.3%, and 60.4%, respectively (P<0.01). CONCLUSIONS: SRS-based therapy achieved DAVF obliteration in more than three-quarters of the patients at 4 years of age. Our grading system can stratify the obliteration rate and may guide physicians in treatment selection.
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PURPOSE: Reversible aggravation of myelopathy symptoms was observed after the intake of taurine-rich foods in patients with venous congestive myelopathy (VCM) caused by a spinal arteriovenous shunt (SAVS), and the taurine-challenge test was applied to demonstrate an association between taurine and VCM. MATERIALS AND METHODS: The current study reviewed any aggravation history of myelopathy symptoms, including walking difficulty, after consuming taurine-rich foods among 133 consecutive patients with a SAVS from a prospective institutional database from June 2013 to February 2021. The type of taurine-rich foods, demographic data, arteriovenous shunt level, and follow-up periods were obtained. For the controlled taurine challenge test, Bacchus® (Dong-A Pharmaceutical, Seoul, Korea), a taurine-rich drink, was given to patients who fulfilled test criteria of recovered VCM (pain-sensory-motor-sphincter scale ≥2, improvement of spinal cord signal intensity on magnetic resonance imaging, and follow-up >6 months after SAVS treatment) to confirm the disappearance of such aggravation. RESULTS: Ten patients had an aggravation history related to food. Webfoot octopus, small octopus, squid, crab, scallop, and taurine-rich energy drink (Bacchus®) were related to such aggravation in patients with VCM. Aggravation appeared about 30 minutes after food intake followed by expressions such as 'I could not walk and collapsed to the ground' and usually lasted for about 3 hours, followed by a slow recovery after taking rest. Four patients who met the test criteria underwent the taurine challenge with Bacchus® and revealed no further symptom aggravation, suggesting that taurine did not affect patients after recovery from VCM. CONCLUSION: The association between taurine-rich food and reversible symptom aggravation can appear in patients with VCM and disappear after VCM treatment. Aggravation of venous hypertension in the spinal cord is suggested as a mechanism but further elucidation is needed.