Congestive Myelopathy Research Articles

Specific electromyography characteristics can distinguish longitudinally extensive transverse myelitis from congestive myelopathy due to spinal dural arteriovenous fistula: a retrospective study

Specific electromyography characteristics can distinguish longitudinally extensive transverse myelitis from congestive myelopathy due to spinal dural arteriovenous fistula: a retrospective study

Abstract 081: Clinical Presentation and Treatment of 26 Spinal Epidural Arteriovenous Fistulas: A Single Center Experience

Introduction Spinal epidural arteriovenous fistulas (SEAVFs) are rarely diagnosed vascular malformations that can cause spinal cord compression and congestive myelopathy. Methods This is a single center, retrospective case series of patients with SEAVF who underwent observation or treatment at UCLA medical center from 1993 to 2023. Results A total of 26 patients were found to have a SEAVF at UCLA from 1993 to 2023. The median age at treatment was 59 years (range 4 months to 91 years). 16 of 26 patients (55.2%) were male. 12 were located in the cervical spine, 2 in the thoracic spine, 11 in the lumbar spine, and 1 in the sacral spine. Sacral, lumbar, and thoracic SEAVFs demonstrated a strong male predominance (12 of 14, 85.7%), while cervical SEAVFs were more common in women (8 of 12, 66.7%). The median duration of symptoms prior to treatment was 6.5 months (range 1 day to 8 years). Possible triggers included prior spinal surgery (n=3), turning neck (n=1), trauma to the neck (n=1), lifting a heavy box (n=1), prolonged period of bending over (n=1). The remaining patients did not have any particular trigger. All patients with lumbar and thoracic SEAVFs (except the 3‐month‐old) demonstrated flow voids and cord edema. Patients with cervical SEAVF did not demonstrate cord edema and only 5 of 12 explicitly mentioned flow voids. 22 patients were treated strictly endovascularly, 1 patient endovascularly and then surgically, 1 patient surgically, and 2 patients refused treatment. No patients treated endovascularly experienced complications. 19 of 23 patients (82.6%) treated endovascularly received complete cure after the first treatment. 1 patient had a successful subsequent embolization 2 days later. Of the 3 other incomplete treatments, one patient received subsequent successful surgery, one patient was a 3‐month‐old patient with Parkes‐Weber syndrome who subsequently died of other medical issues, and one was lost to follow‐up. One patient underwent surgical treatment alone due to the feeding arteries being too small to catheterize and unfortunately experienced a failed surgery on the first attempt and developed a surgical site infection after the second successful attempt at treatment. Onyx was used in 10 cases (9 of 10 successful). 2 cases treated with NBCA were unsuccessful. Cervical and Sacral SEAVFs were treated successfully (mostly prior to 2006) with coils and/or detachable balloons. Conclusion SEAVF is a rare disease that can be treated effectively and safely with endovascular embolization in most cases. Pre‐operative MRI demonstrated abnormalities in all cases. We found a strong male predominance in our cohort.

Extradural lumbar arteriovenous fistula imitating a synovial cyst: illustrative case.

Spinal dural arteriovenous fistula is the most common spinal vascular malformation. It usually presents with neurological deficits due to venous congestive myelopathy. Sometimes, however, the symptoms are radicular and can imitate those of a disc hernia or synovial cyst. In this illustrated case, a 34-year-old male patient presented with radicular pain due to nerve root compression. In the magnetic resonance imaging (MRI) examination of the spine, a synovial cyst was suspected, so the patient underwent no further examination before surgery. Intraoperatively, the authors were surprised to see a vascular malformation, which was shown to be an extradural arteriovenous fistula according to the histopathological examination. In atypical MRI findings of the spine, vascular malformations should be considered. In cases with a spinal dural arteriovenous fistula, the thrombosis risk can be high and lead to a need for prolonged anticoagulation.

Treatment of high cervical arteriovenous fistulas in the craniocervical junction region.

The craniocervical junction (CCJ) is a complex region. Rarely, arteriovenous fistulas (AVFs) can occur in the CCJ region. Currently, it is accepted that CCJ AVFs should only refer to AVFs at the C1-C2 levels. It is reasonable to assume that high cervical CCJ AVFs are being referred to when discussing CCJ AVFs. High cervical CCJ AVFs can be divided into the following four types: dural AVF, radicular AVF, epidural AVF and perimedullary AVF. Until now, it was difficult to understand high cervical CCJ AVFs and provide a proper treatment for them. Therefore, an updated review of high cervical CCJ AVFs is necessary. In this review, the following issues are discussed: the definition of high cervical CCJ AVFs, vessel anatomy of the CCJ region, angioarchitecture of high cervical CCJ AVFs, treatment options, prognoses and complications. Based on the review and our experience, we found that the four types of high cervical CCJ AVFs share similar clinical and imaging characteristics. Patients may present with intracranial hemorrhage or congestive myelopathy. Treatment, including open surgery and endovascular treatment (EVT), can be used for symptomatic AVFs. Most high cervical CCJ AVFs can be effectively treated with open surgery. EVT remains challenging due to a high rate of incomplete obliteration and complications, and it can only be performed in superselective AVFs with simple angioarchitecture. Appropriate treatment can lead to a good prognosis.

Congestive myelopathy due to craniocervical junction arteriovenous fistulas mimicking transverse myelitis: a multicenter study on 27 cases.

The purpose was to clarify diagnostic clues and pitfalls in cranio-cervical junction arteriovenous fistulas (CCJ AVFs) with congestive myelopathy. In a multicenter observational study by the Neurospinal Society of Japan, we described the demographics, clinical courses, imaging findings, and outcomes of consecutive patients with CCJ AVFs presenting with congestive myelopathy between 2009 and 2019. Twenty-seven patients were included (mean age, 70years; male, 96%). Progressive symptoms within one day to one month were more common (63%) than chronic symptoms. Myelopathic symptoms were characterized by ascending paralysis beginning from the legs, involving the trunk and arms, and sometimes ending in the brainstem. Fifteen patients (56%) received a misdiagnosis, including acute transverse myelitis. The most common MRI findings were venous congestive edema of the cervical cord (96%) and the brainstem (63%) and surrounding vascular flow voids (100%). The mean extension of congestive edema was 5.5 ± 2.9 vertebral segments. The most common angiographic findings were a dural AVF (78%) at the C1 level (81%) with descending venous drainage (85%). Seven patients (26%) were administered steroids, which resulted in neurological decline in 3. Neurosurgical obliteration of the AVF led to improvements in MRI findings in 75% and a functional status in 67%; however, 44% remained dependent. The myelopathy of CCJ AVFs was characterized by acute ascending paralysis in elderly men. A misdiagnosis was common because of the acute presentation due to a longitudinally extensive spinal cord lesion. Dilated vessels on MRI were a key finding for the correct diagnosis. What is already known on this topic? Slowly progressive myelopathy is a well-known symptom that results from impaired spinal venous drainage due to thoracolumbar AVFs. Although cranio-cervical junction arteriovenous fistulas (CCJ AVFs) constitute a treatable cause of congestive myelopathy, detailed information is not currently available due to their rarity. What does this study add? CCJ AVFs often presented with acute ascending myelopathy in elderly men due to a longitudinally extending cervical cord lesion with surrounding flow voids. Steroid pulse therapy was not effective or even harmful to congestive myelopathy, while neurosurgical treatment effectively obliterated AVFs. How might this study affect research, practice or policy? The results obtained revealed diagnostic clues and pitfalls from the largest dataset of patients with CCJ AVFs in a multicenter cohort.

Arteriovenous malformations of the filum terminale: clinical characteristics, angioarchitecture, and management of a rare spinal vascular pathology.

The goal of this study was to describe clinical and neuroradiological features of arteriovenous malformations of the filum terminale (FT AVMs) and to present the authors' diagnostic and therapeutic management in this rare disease. The presented cases were retrieved from a retrospectively collected database of all spinal vascular malformations treated between June 1992 and December 2021 at the Rheinisch-Westfälische Technische Hochschule (RWTH) University Hospital Aachen. Pretreatment and follow-up clinical and neuroradiological data were analyzed for this study. Data in 15 patients with FT AVM with a mean age of 60 years were included, with an overall incidence of FT AVM of 19% among all spinal AVMs in our cohort. Twelve of 15 (80%) patients were men. Nonspecific but typical clinical and MR findings of thoracolumbar congestive myelopathy were found in all patients. Spinal MR angiography, performed in 10 patients, identified in all cases the arterialized FT vein as well as a lumbar/lumbosacral location of an AV shunt. Digital subtraction angiography (DSA) showed an arterial supply solely via the FT artery in 12/15 (80%) patients and via an additional feeder from the lumbosacral region in the other 3/15 (20%) patients. All patients were treated surgically. During 1-year follow-up, 2 patients presented with recurrent FT AVM due to further arterial supply from the lumbosacral region, and were treated surgically. Neurological status was improved in all patients within the 1-year follow-up, with marginal further changes during long-term follow-up. Congestive myelopathy is the major pathological mechanism of symptoms in these patients, with no evidence for intradural bleeding. Missing the presence of possible multiple arterial supply of FT AVM during DSA may result in misdiagnosis and/or insufficient treatment. Due to the frequently prolonged course of FT artery, resection of the FT AVM may be a favorable treatment modality in comparison with endovascular treatment. Follow-up examinations are obligatory within the first 3 years after treatment, and further MR angiography and DSA examinations are indicated if congestive myelopathy persists.

Arterioectatic Spinal Angiopathy of Childhood: Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology.

Pediatric patients with myelopathy expressing intradural spinal vascular ectasia without arteriovenous shunting were studied at four tertiary referral neuropediatric centers. Patients were identified by retrospective review of institutional records and excluded if spinal vascular pathology could be classified into a previously described category of spinal vascular malformation. Four patients meeting the study criteria were enrolled in the study. Clinical, magnetic resonance imaging, catheter-directed angiography, laboratory, histological and genetic data were analyzed to characterize the disease process and elucidate underlying pathomechanisms. Our study revealed a highly lethal, progressive multi-segmental myelopathy associated with a unique form of non-inflammatory spinal angiopathy featuring diffuse enlargement and tortuosity of spinal cord arteries, spinal cord hyperemia, and spinal cord edema (Arterioectatic Spinal Angiopathy of Childhood). The condition was shown to mimic venous congestive myelopathy associated with pediatric spinal cord arteriovenous shunts on MRI but to have distinct pathognomonic findings on catheter-directed angiography. Clinicopathological, genetic, and neuroimaging features, which are described in detail, closely overlap with those of mitochondrial disease.

Reversible Symptom Aggravation by Intake of Taurine-Rich Foods in Patients with Venous Congestive Myelopathy: Controlled Case Series Study.

PurposeReversible 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 MethodsThe 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.ResultsTen 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.ConclusionThe 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.

P.156 Spinal subdural hematoma from a type I spinal arteriovenous malformation: case report and literature review

Background: Type 1 spinal arteriovenous malformations (AVM) are dural fistulas which classically present due to progressive congestive myelopathy. Acute hemorrhage resulting in a spinal subdural hematoma is exceedingly rare with only three reported cases. Methods: This 46-year-old female experienced rapidly progressive paraplegia over several hours. There was no history of anti-coagulant use, trauma, fever, or infection. On examination, she had 0/5 in the lower extremities bilaterally and decreased sensation to pinprick below the T6 level on both sides. She also had loss of bowel and bladder control. MRI demonstrated evidence of an intra-dural extramedullary lesion located anteriorly causing significant compression on the spinal cord from T4 down to T7 level. Results: She underwent thoracic laminectomy from T4 – T7. After dural opening, a well organized sub-dural hematoma with significant mass effect on a distorted spinal cord was evacuated. Subsequently, a large abnormal artery leading into a dilated serpiginous vein on the dorsal aspect of spinal cord at the T7 level was visualized. Indocyanine green angiography confirmed our suspicion of a dural arteriovenous fistula which was disconnected. Conclusions: Type I spinal AVM in rare cases can present acutely as subdural hematoma and should be kept in the differential diagnosis for acute paraplegia.

411 Treatment Results of Arteriovenous Fistulas at the Craniocervical Junction: A Multicenter Cohort Study of 108 Patients

INTRODUCTION: No standard treatment has been established for the arteriovenous fistulas (AVFs) at the craniocervical junction (CCJ). METHODS: A total of 103 consecutive patients with the CCJ AVFs were collected from 29 centers that are members of Neurospinal society of Japan. Angiographic findings and the clinical characteristics of the CCJ AVFs were evaluated. RESULTS: Among 103 cases, dural AVF (59 cases), radicular AVF (12 cases), epidural AVF (16 cases), and perimedullary AVF (5 cases) were included. There were 11 cases in which combinations of the pathologies occurred in one case. 51 and 7 patients presented with subarachnoid hemorrhage (SAH) and intramedullary hemorrhage, respectively. 28 cases presented with congestive myelopathy, while there were 17 asymptomatic cases. Overall, 78 cases (76%) and 19 cases (18 %) underwent primary surgical or endovascular treatments, respectively. 4 cases (4%) underwent combined treatments. 14 cases (14%) required second treatments. The rate of initial treatment failure or recurrence was 2.6% after the surgical treatments (Surgery Group), while it was 63% of the endovascular treatment group (Endovascular Group). In multivariate analysis, endovascular treatment was the independent risk factor with significantly higher odds of initial treatment failure or recurrence (odds ratio [OR], 59; 95% confidence interval [CI], 11-324). Treatments related complications occurred in 28 cases (27 %). The rates of complications were 21% in the Surgery Group. While, it was 42% in the Endovascular Group. Complications included medulla/spinal/cerebellar infarction (12 cases), the cerebrospinal fluid leak (3 cases), and postoperative hematomas (3 cases).Post-operatively, an improvement of 1 or more in modified Rankin Scale (mRS), was observed in 60% and 37% of Surgery and Endovascular Groups, respectively with a median follow-up of 23.0 months. CONCLUSION: Based on the largest and latest multicenter cohort, this study showed that primary microsurgery was superior to endovascular treatment to achieve complete obliteration of the CCJ AVF in a single procedure.

Case Report: Differential Diagnosis of Lower Extremity Weakness in a Young Male - Consider Foix Alajouanine Syndrome

IntroductionThere is a limited list of emergent spinal cord pathology that must be considered in patients with focal neurological deficits in the emergency department. Identification of these conditions requires a detailed history and neurological exam and may also require advanced testing and imaging.Case ReportHere we present the case of a patient with a rare arteriovenous malformation of the spinal cord vessels causing congestive myelopathy (Foix-Alajouanine syndrome) that presented as a clinical mimic of spinal cord compression.ConclusionEmergency physicians should be aware of Foix-Alajouanine syndrome, as its workup and management differ from more common pathologies that may present similarly.

Subacute progressive paraparesis (Foix-Alajouanine syndrome) in an elderly African-American man

First described in 1926 by Foix and Alajouanine, the Foix-Alajouanine syndrome is a rare congestive myelopathy typically arising secondary to dural arteriovenous (AV) fistulas, often of cryptogenic origin. We here present a case of the rare and overlooked Foix-Alajouanine syndrome.

Embolization Tactics of Spinal Epidural Arteriovenous Fistulas.

PurposeSpinal epidural arteriovenous fistulas (SEDAVFs) show an epidural venous sac often with venous congestive myelopathy (VCM) due to intradural reflux at a remote level to which a transarterial approach would be difficult. We present 12 cases of SEDAVF with VCM and describe 3 main tactics for effective transarterial embolization.Materials and MethodsAmong 152 patients with spinal vascular malformations diagnosed in our tertiary hospital between 1993 and 2019, 12 SEDAVF patients with VCM were included. Three different transarterial embolization tactics were applied according to the vascular configuration and microcatheter accessibility. We evaluated treatment results and clinical outcomes before and after treatment.ResultsTransarterial embolization with glue (20–30%) was performed in all patients. The embolization tactics applied in 12 patients were preferential flow (n=2), plug-and-push (n=6), and filling of the venous sac (n=4). Total occlusion of the SEDAVF, including intradural reflux, was achieved in 11 (91.7%) of 12 patients, and partial occlusion was achieved in 1 patient. No periprocedural complications were reported. Spinal cord edema was improved in all patients for an average of 18 months after treatment. Clinical functional outcome in terms of the pain, sensory, motor, and sphincter scale and modified Rankin scores improved during a mean 25-month follow-up (6.3 vs. 3.3, P=0.002; 3.6 vs. 2.3, P=0.002, respectively).ConclusionEndovascular treatment for 12 SEDAVF patients with VCM achieved a total occlusion rate of 91.7% without any periprocedural complication. The combined embolization tactics can block intradural reflux causing VCM, resulting in overall good clinical outcomes.

Early Diagnosis of Spinal Dural Arteriovenous Fistula

Spinal dural arteriovenous fistulas (SDAVF) are rare and most commonly affect men aged >50 years. Patients with SDAVF develop an abnormal vascular dural shunt between the dural branch of a segmental artery and a subdural radicular vein that drains the perimedullary venous system, leading to venous hypertension and secondary congestive myelopathy. Most SDAVFs are located in the thoracolumbar region, and usually patients present with slowly progressive paraparesis and urinary disturbances. SDAVF is diagnostically challenging; this condition may be misdiagnosed as lumbar spinal stenosis or myelitis. Clinicians should be aware of fluctuating symptoms in the early stages to avoid misdiagnosis of SDAVF. Claudication is associated with various activities including walking, bathing, drinking, and singing. On T2-weighted magnetic resonance imaging of the spinal cord, SDAVFs show a high signal intensity with a low signal intensity peripherally and dilated spinal cord veins in the subarachnoid space.

Foix-Alajouanine Syndrome Mimicking Longitudinally Extensive Transverse Myelitis.

Spinodural arteriovenous fistula (SDAF) can present with longitudinally extensive transverse myelitis (LETM), progressive gait instability, and lower extremity weakness.Failure to promptly recognize this condition and misdiagnosis can result in a poor outcome.Microsurgical obliteration of the SDAF provides better results than embolization.

Syringomyelia Associated with Spinal Dural Arteriovenous Fistula: Clinical and Radiological Improvement after Embolization

Spinal dural arteriovenous fistulae (AVF) are rare and can result in spinal cord dysfunction. We present one such case wherein the patient presented with a venous congestive myelopathy. Magnetic resonance imaging showed a syrinx formation, spinal cord edema, and flow voids. Digital subtraction angiography confirmed the dural AVF, which was treated with embolization. The syrinx disappeared, other spinal cord changes improved, and the patient had remarkable clinical improvement. The case is presented to draw attention to the rare formation of a syrinx in a spinal dural arteriovenous fistula and its disappearance after successful embolization.

Clinical and angioarchitectural characteristics of spinal vascular malformations of the cervical spine.

Vascular malformations of the cervical spine are exceedingly rare. To date there have been no large case series describing the clinical presentation and angioarchitectural characteristics of cervical spine vascular malformations. The authors report their institutional case series on cervical spine vascular malformations diagnosed and treated at their institution. The authors retrospectively reviewed all patients with spinal vascular malformations from their institution from January 2001 to December 2018. Patients with vascular malformations of the cervical spine were included. Lesions were characterized by their angioarchitectural characteristics by an interventional neuroradiologist and endovascular neurosurgeon. Data were collected on clinical presentation, imaging findings, treatment outcomes, and long-term follow-up. Descriptive statistics are reported. Of a total of 213 patients with spinal vascular malformations, 27 (12.7%) had vascular malformations in the cervical spine. The mean patient age was 46.1 ± 21.9 years and 16 (59.3%) were male. The most common presentations were lower-extremity weakness (13 patients, 48.1%), tetraparesis (8 patients, 29.6%), and lower-extremity sensory dysfunction (7 patients, 25.9%). Nine patients (33.3%) presented with hemorrhage. Fifteen patients (55.6%) had modified Rankin Scale scores of 0-2 at the time of diagnosis. Regarding angioarchitectural characteristics, 8 patients (29.6%) had intramedullary arteriovenous malformations (AVMs), 5 (18.5%) had epidural arteriovenous fistulas (AVFs), 4 (14.8%) had paraspinal fistulas, 4 (14.8%) had mixed epidural/intradural fistulas, 3 (11.1%) had perimedullary AVMs, 2 (7.4%) had dural fistulas, and 1 patient (3.7%) had a perimedullary AVF. This retrospective study of 27 patients with cervical spine vascular malformations is the largest series to date on these lesions. The authors found substantial angioarchitectural heterogeneity with the most common types being intramedullary AVMs followed by epidural AVFs, paraspinal fistulas, and mixed intradural/extradural fistulas. Angioarchitecture dictated the clinical presentation as intradural shunts were more likely to present with hemorrhage and acute onset myelopathy, while dural and extradural shunts presented as either incidental lesions or gradually progressive congestive myelopathy.

Intradural radicular arteriovenous fistula that mimics dural arteriovenous fistula: report of three cases.

We herein present three cases of a rare type of spinal AVF, an intradural radicular AVF, which mimicked a dural AVF. A 65-year-old male presented with congestive myelopathy. On angiography, right vertebral angiogram (VAG) showed a suspected dural AVF; however, left VAG showed the same intradural dilated vein fed by the anterior spinal artery (ASA). Intraoperative and histological results suggested that a single AVF was located on the right C1 nerve root fed by the right C1 radicular artery and branch of the anterior spinal artery. Two additional patients with a radicular AVF at the C3 or C5 level were presented. Intradural radicular AVFs and dural AVFs have very similar appearances; however, there is a difference that makes the risk of the endovascular treatment of radicular AVFs markedly higher because of blood supply from the ASA. In our case, the AVF was completely occluded by direct surgery without major complications.

Primary conus medullaris arteriovenous shunt and secondary lumbo-sacral epidural arteriovenous fistula: One malformation can hide another

We report an anatomical-based association between conus medullaris pial arteriovenous shunt that drain caudally towards the lumbosacral area with very delayed onset of an acquired lumbar epidural shunt, draining secondarily towards intradural veins and responsible for a venous congestive myelopathy with identical clinical symptoms. These patients require close clinical and imaging follow-ups in order to propose adequate treatments before onset of irreversible neurological deficits. MRA should include the lumbo-sacral area in its field of view.

Osseous versus Nonosseous Spinal Epidural Arteriovenous Fistulas: Experiences of 13 Patients.

Spinal epidural arteriovenous fistulas are rare vascular malformations. We present 13 patients with spinal epidural arteriovenous fistulas, noting the various presenting symptom patterns, imaging findings related to bone involvement, and outcomes. Among 111 patients with spinal vascular malformations in the institutional data base from 1993 to 2017, thirteen patients (11.7%) had spinal epidural arteriovenous fistulas. We evaluated presenting symptoms and imaging findings, including bone involvement and mode of treatment. To assess the treatment outcome, we compared initial and follow-up clinical status using the modified Aminoff and Logue Scale of Disability and the modified Rankin Scale. The presenting symptoms were lower back pain (n = 2), radiculopathy (n = 5), and myelopathy (n = 7). There is overlap of symptoms in 1 patient (No. 11). Distribution of spinal epidural arteriovenous fistulas was cervical (n = 3), thoracic (n = 2), lumbar (n = 6), and sacral (n = 2). Intradural venous reflux was identified in 7 patients with congestive venous myelopathy. The fistulas were successfully treated in all patients who underwent treatment (endovascular embolization, n = 10; operation, n = 1) except 2 patients who refused treatment due to tolerable symptoms. Transarterial glue (n = 7) was used in nonosseous types; and transvenous coils (n = 3), in osseous type. After 19 months of median follow-up, the patients showed symptom improvement after treatment. Although presenting symptoms were diverse, myelopathy caused by intradural venous reflux was the main target of treatment. Endovascular treatment was considered via an arterial approach in nonosseous types and via a venous approach in osseous types.