Bilateral optic disc collaterals secondary to high-flow dural arteriovenous fistula: a diagnostic dilemma.

A man in his 80s, with a history of diabetes, hypertension and coronary artery disease, presented with bilateral painless progressive vision loss 2 years prior. His examination showed subnormal best corrected visual acuity of 20/50 and 20/80 in the right eye and left eye (LE), respectively, grade II relative afferent pupillary defect in LE, normal anterior segment, intra-ocular pressure (IOP) and defective colour vision in both eyes (BE). Fundus examination revealed optic disc pallor, disc collaterals and grade 2 hypertensive retinopathy in BE. Automated perimetry showed advanced field loss in BE. MRI of the brain and orbits with contrast showed signs of raised intracranial pressure, and magnetic resonance angiogram of the brain showed multiple arterio-venous channels along with the right transverse and sigmoid sinuses. The patient was referred to a neuroradiologist for further evaluation, and cerebral angiogram confirmed multifocal high-flow dural arterio-venous fistulae at right jugular foramen, transverse and sigmoid sinuses. He underwent Onyx liquid embolization.

Stereotactic radiosurgery with versus without embolization for intracranial dural arteriovenous fistulas: a systematic review and meta-analysis.

OBJECTIVE: Stereotactic radiosurgery (SRS) has been established as a safe and alternative treatment for dural arteriovenous fistulas (dAVFs). While embolization alone is the most commonly used modality for the treatment of dAVFs, the adjunctive use of embolization with SRS, with the growing use of SRS, has gained increasing interest in the past few years. However, the relative efficacy and safety of SRS combined with embolization versus SRS alone for dAVFs remains uncertain. Hence, this systematic review aimed to evaluate the efficacy of SRS with adjunctive embolization for intracranial dAVFs. METHODS: A systematic review and meta-analysis was conducted by searching electronic databases, including PubMed, Embase, and the Cochrane Library, up to August 2023. All studies evaluating the utilization of adjunctive embolization and SRS for dAVFs were included. Risk of bias was assessed using the Newcastle-Ottawa Scale. A meta-analysis was conducted on the suitable outcomes. RESULTS: Eighteen studies involving 715 patients were included. The mean age of the participants in the study was 64.30 years in the adjunctive embolization group and 60.51 years in the SRS-alone group. In the adjunctive embolization group 41.3% of patients were female, compared with 47.1% in the SRS-only group. The dAVF obliteration rates were 64.7% and 65.7% in the adjunctive embolization and SRS-alone groups, respectively. These obliteration rates were comparable between the two groups (p = 0.96), as were the symptom improvement rates (p = 0.35). Adverse events were rare, and were more commonly associated with the adjunctive embolization procedure, although further causal analysis was not possible. CONCLUSIONS: This study provides evidence that adjunctive embolization plus SRS provides similar obliteration and symptom improvement rates compared with SRS alone, with both having very limited SRS-related adverse events. Considering the added burden and adverse events of additional endovascular treatment, the authors recommend embolization be reserved for more complex dAVFs or when embolization can potentially be curative alone or provide more rapid symptomatic relief or protection during the radiosurgical latency period.

Simultaneous Parkinsonism and Dementia as Initial Presentation of Intracranial Dural Arteriovenous Fistulas: A Systematic Review.

BACKGROUND: Intracranial dural arteriovenous fistulas (IDAVFs) are abnormal vascular connections between dural arteries and various venous structures within the brain. IDAVFs, rarely present with parkinsonism and dementia concurrently, making this a unique and underexplored clinical scenario. To the best of our knowledge, this is the first systematic review to comprehensively analyze cases of IDAVFs manifesting as both parkinsonism and dementia. METHODS: We assessed databases from inception to September 18, 2023. We identified studies describing patients with IDAVFs initially presenting with dementia or parkinsonism. Inclusion criteria encompassed case reports and case series, while excluding review articles, guidelines, technical notes, comments, conference abstracts, and editorials. RESULTS: The systematic search resulted in the initial screening of 383 studies, with 33 articles meeting the inclusion criteria. Among these, 29 were case reports, often describing 3 or fewer patients. From the remaining 4 case series, data pertinent to patients presenting both parkinsonism and dementia were selectively extracted, yielding a total study population of 43 patients. The anatomical distribution of IDAVFs within this cohort was diverse, with the transverse and sigmoid sinuses being the most common locations. Although most of these patients received endovascular therapy, a few underwent microsurgical occlusion or combined surgical and endovascular treatment. CONCLUSIONS: IDAVFs presenting with both parkinsonism and dementia represent a rare clinical entity. This systematic review provides valuable insights into the clinical characteristics, treatment options, and outcomes for such cases. However, additional research involving larger cohorts is essential to better comprehend the underlying mechanisms and establish standardized therapeutic guidelines.

Usefulness of time-resolved MR angiography in spinal dural arteriovenous fistula (SDAVF)-a systematic review and meta-analysis.

Spinal dural arteriovenous fistulas (SDAVFs) constitute the most common type of spinal vascular malformations. Their diagnosis requires spinal digital subtraction angiography (DSA), which is time-consuming, requires catheterizing many vessels, and exposes patient to a high radiation and contrast doses. This study aims to evaluate the usefulness of time-resolved MR angiography (TR-MRA) in SDAVF diagnosis. We performed a systematic review of the PubMed and EMBASE databases followed by a meta-analysis. TR-MRA was an index test, and spinal DSA was a reference. Of the initial 324 records, we included 4 studies describing 71 patients with SDAVFs. In 42 cases, TR-MRA was true positive, and in 21 cases, it was true negative. We found 7 false-positive cases and 1 false negative. TR-MRA allowed for shunt level identification in 39 cases. Of these, the predicted level was correct in 23 cases (59%), to within 1 level in 38 cases (97.4%) and to within 2 levels in 39 cases (100%). The diagnostic odds ratio was 72.73 (95% CI [10.30; 513.35]), z = 4.30, p value < 0.0001. The pooled sensitivity was 0.98 (95% CI [0.64; 1.00]), and the pooled specificity was 0.79 (95% CI [0.10; 0.99]). The AUC of the SROC curve was 0.9. TR-MRA may serve as a preliminary study to detect SDAVFs and localize the shunt level with sensitivity and specificity as high as 98% and 79%, respectively. Unless the TR-MRA result is unequivocal, it should be followed by a limited spinal DSA.

Dural arteriovenous fistula with progressive dementia and parkinsonism: Two case reports and a literature review.

RATIONALE: Dural arteriovenous fistulas (DAVFs) are rare cerebral abnormal arteriovenous anastomoses. It is uncommon for DAVFs with parkinsonism and dementia, so it is easily misdiagnosed. Neuroimaging examinations show that multifocal DAVFs are related to venous thrombosis and white matter changes, suggesting that cerebral circulatory disorders caused by venous hypertensive encephalopathy lead to dementia in patients. Digital subtraction angiography confirmed the diagnosis and subsequent treatment of DAVFs. PATIENT CONCERNS: We report 2 cases, one caused by bilateral white matter lesions and the other caused by bilateral thalamus lesions. Their symptoms are all manifested as progressive dementia and parkinsonism. DIAGNOSIS: They were diagnosed with dural arteriovenous fistulas by digital subtraction angiography. OUTCOMES: The first patient developed progressive cognitive impairment, 6 months later, the patient developed bedridden, incontinence, and severe cognitive function.The second patient became increasingly bedridden 3 months after discharge and died of aspiration pneumonia. LESSONS: There are few reports of progressive dementia and parkinsonism in DAVF patients, and neurologists should be vigilant to avoid misdiagnosing DAVF.

Secondary ocular hypertension due to tentorial dural arteriovenous fistula: a case report.

BACKGROUND: Tentorial dural arteriovenous fistulas (TDAVFs) are abnormal shunts between meningeal arteries and the intradural venous system located in the tentorial dura mater, which typically manifest with haemorrhage or progressive neurological disorders. TDAVFs with pure ocular presentation have been rarely reported. CASE PRESENTATIONS: The case of a 56-year-old man presented with unilateral eye redness, proptosis and elevated intraocular pressure was reported herein, which was caused by a TDAVF. The fistula was fed by the left posterior cerebral artery and posterior meningeal artery. The drainage was into the basal vein and internal cerebral veins, which led the arterial blood flow forward to the left superior ophthalmic vein directly. The redundant blood flow caused the rise of episcleral venous pressure, leading to the clinical presentations. Gamma knife radiosurgery was performed then considering the delicate vascular structure and its deep location. The corkscrew hyperaemia was gradually alleviated after the surgery, but the intraocular pressure remained elevated at follow-ups. CONCLUSION: Dural arteriovenous fistulas which are not directly connected to cavernous sinus could cause ocular presentations like proptosis, eye redness and ocular hypertension.

Dural arteriovenous fistula presenting with stroke-like symptoms and regional cerebral hyperperfusion: a case report.

BACKGROUND: Non-hemorrhagic focal neurological deficit is one of the clinical manifestations of intracranial dural arteriovenous fistulas (DAVF). When symptoms appear suddenly, it is difficult to distinguish it from ischemic stroke in certain circumstances, which might easily lead to misdiagnosis. Here, we report a rare case of DAVF with sudden onset sensory aphasia mimicking hyperacute stroke but presented with unexpected regional hyperperfusion on the site corresponding to its symptoms. CASE PRESENTATION: A 76-year-old male with histories of atrial fibrillation and hypertension was admitted to the emergency department due to sudden sensory aphasia. The diagnosis of ischemic stroke was made based on clinical experience after non-contrast CT excluding hemorrhage. As in the absence of clear contraindication, the patient received intravenous thrombolysis. On the cerebral CT perfusion, the left temporal lobe, where the sensory speech center is located, was manifested as regional hyperperfusion. Thrombolysis was subsequently halted, but scheduled cranial imaging indicated hemorrhagic transformation. According to the radiological hint from cranial MRI, the patient was suspected of having DAVF, which was finally confirmed by cerebral digital subtraction angiography. CONCLUSION: When DAVF is presented as sudden onset focal neurological deficit, cranial CT perfusion at an early stage might reveal an abnormal hyperperfusion pattern. Clinicians should be aware of the diagnostic possibility of DAVF in this situation and double-review the CT angiography image to reduce missed diagnoses.

[Surgical treatment of spinal dural arteriovenous fistulas: Management and long-term results in a series of patients operated in an Argentinian Center]. / Resultados a largo plazo del tratamiento quirúrgico de las fístulas arteriovenosas durales espinales en un Centro argentino.

INTRODUCTION: Spinal dural arteriovenous fistula (SDAVF) is a rare vascular disease, of unknown etiology, frequently underdiagnosed. Treatment can be microsurgical or endovascular. METHODS: Retrospective and monocentric analysis of 12 SDAVF patients treated by microsurgery between 2010 and 2021. Parameters including age, sex, pre and postoperative clinical condition were analyzed according to modified Aminoff-Logue and Rankin scales. Diagnostic studies such as magnetic resonance imaging (MRI), magnetic resonance angiogram (MRA) and spinal digital subtraction angiography (DSA), were evaluated for lesion level, as were surgical results. RESULTS: Twelve patients (10 men and 2 women), average age: 60 years, were operated. The interval from symptom onset to diagnosis was < 12 months in all cases except one (32 months). SDAVF locations were thoracic in 8 cases, between T6 and T12, 3 at lumbar spine (L1-L2) and at S1 in one case, with no difference regarding side. The Adamkiewicz artery was identified in 5 cases at L1, 2 at D12, 2 at D10, 2 at D9 and 1 at D7 (7 left-sided and 5 right-sided). Three of the 12 patients operated had undergone prior embolization. Postoperative neurological outcomes showed: 2 patients remained stable and 10 improved one or more points on the mRs; no postoperative complications were observed. Follow-up MRI images improved in all cases and spinal DSA was negative at 6 months. Average follow-up was 40 months (range 6 to 122) and no patient presented recurrence. CONCLUSION: Microsurgical treatment of SDAVF proved to be efficient, with low morbidity and lower recurrence rates compared to endovascular results.

Introducción: La fístula dural arteriovenosa espinal (FDAVE) es una enfermedad vascular frecuentemente subdiagnosticada. El tratamiento puede ser microquirúrgico o endovascular. Métodos: Estudio retrospectivo de una serie de pacientes con FDAVE tratados por microcirugía entre los años 2010 y 2021. Fueron evaluados parámetros como edad, sexo, cuadro clínico pre y postoperatorio medido con las escalas de Aminoff-Logue y Rankin modificada (mRs). Los estudios diagnósticos se utilizaron para determinar nivel lesional y resultados quirúrgicos. Resultados: Se incluyeron doce pacientes (10 hombres y 2 mujeres) con un promedio de edad de 60 años. El tiempo de evolución del cuadro clínico al diagnóstico fue menor a 12 meses salvo un caso de 32 meses. Las FDAVE fueron localizadas, 8 a nivel dorsal, 3 a nivel lumbar y una a nivel sacro. La arteria de Adamkiewicz se identificó en 5 casos en L1, 2 en D12, 2 en D10, 2 en D9 y un caso en D7. De los 12 pacientes operados, 3 fueron embolizados previamente; dos permanecieron estables en su evolución y 10 mejoraron uno o más puntos del mRs. No hubo complicaciones en el postoperatorio. Todos mostraron mejoría del edema medular en resonancia magnética y la angiografía digital, luego de los 6 meses, fue negativa. El seguimiento promedio fue de 40 meses con un rango de 6 a 122 meses y ningún paciente presentó recidiva de la FDAVE. Conclusión: El tratamiento quirúrgico de las FDAVE es un método muy eficaz, de baja morbilidad y menor tasa de recurrencia comparado con el tratamiento endovascular.

[Left occipital dural arteriovenous fistulas presenting as right red eye: a case report].

A 63-year-old male with a healthy history presented with a red and swollen right eye for 3 months. Neuro-ophthalmic examination showed slight bulging of the right eyeball, and multiple spiral conjunctival vessels were visible on the surface of the right conjunctiva, suggesting a right carotid cavernous fistula. Cerebral angiography showed left occipital dural arteriovenous fistulas. After endovascular embolization treatment, the patient's abnormal craniocerebral venous drainage and right eye syndrome resolved, and there was no recurrence during the one-month clinical follow-up after surgery.

Role of Stereotactic Radiosurgery in the Management of Dural AV Fistula.

Dural arteriovenous fistulas (DAVFs) are a relatively rare intracranial vascular malformation. The various treatment options for DAVFs include observation, compression therapy, endovascular therapy, radiosurgery, or surgery. A combination of these therapies may also be used. The treatment choice for DAVFs depends on the type of fistula, the severity of symptoms, DAVF angioarchitecture, and the efficacy and safety of treatments. The use of stereotactic radiosurgery (SRS) in DAVFs started in the late 1970s. There is a delay before the fistula gets obliterated after SRS and there is a risk of hemorrhage from the fistula till the fistula gets obliterated. Initial reports described the role of SRS in small DAVFs without severe symptoms, which were inaccessible by endovascular or surgical measures or in combination with embolization in larger DAVFs. SRS can be appropriate for indirect cavernous sinus DAVF fistulas (Barrow type B, C, and D). Borden types II and III and Cognard types IIb-V DAVFs have a high risk of hemorrhage and are traditionally considered less favorable to be treated with SRS as immediate treatment is required to decrease the risk of hemorrhage. However, recently SRS has been tried in these high-grade DAVF as a monotherapy. Factors that have a positive impact on the obliteration rates of DAVF following SRS are the location of DAVF with the cavernous sinus DAVF having much better obliteration rates than DAVF located at other locations, Borden Type I or Cognard Types III or IV DAVFs, absence of CVD, hemorrhage at the time of initial presentation, and target volume lesser than 1.5 mL.

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

BACKGROUND: The purpose was to clarify diagnostic clues and pitfalls in cranio-cervical junction arteriovenous fistulas (CCJ AVFs) with congestive myelopathy. METHODS: 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. RESULTS: Twenty-seven patients were included (mean age, 70 years; 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. CONCLUSIONS: 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.

Neuronal dysfunction and hemodynamic disturbance due to venous congestion in dural arteriovenous fistula revealed by 123I-iomazenil SPECT.

OBJECTIVE: Retrograde leptomeningeal venous drainage (RLVD) of a dural arteriovenous fistula (dAVF) is associated with neurological morbidity and unfavorable outcomes. However, the direct damage to cortical neurons by dAVF with RLVD has not been elucidated. 123I-iomazenil (123I-IMZ) SPECT can reveal cerebral blood flow and cortical neuronal damage in early and late images, respectively. This study aimed to assess the cerebral venous congestive encephalopathy caused by dAVF using 123I-IMZ SPECT. METHODS: Based on the pre- and posttreatment MRI findings, patients were divided into three groups: a normal group, an edema group, and an infarction group. Radioactive counts in the early and late images of 123I-IMZ SPECT were investigated using the affected-to-contralateral side asymmetry ratio (ACR). RESULTS: None of the patients in the normal group showed any symptoms related to venous congestion. In contrast, all the patients in the edema and infarction groups developed neurological symptoms. The ACR in early images in the edema group was significantly lower than that in the normal group and significantly higher than that in the infarction group. The ACR in the late images of the infarction group was significantly lower than those of the normal and edema groups. After treatment, the neurological signs disappeared in the edema group, but only partial improvement was observed in the infarction group. The ACR in early images significantly improved after treatment in the edema group, but the ACR in late images did not change in any groups. CONCLUSIONS: 123I-IMZ SPECT is useful for evaluating hemodynamic disturbances and neuronal damage in dAVFs. The reduction in early images was correlated with the severity of venous congestive encephalopathy, and the significant reduction in late images is a reliable indicator of irreversible venous infarction caused by RLVD.

Petrous apex's dural arteriovenous fistula (DAVF) mimicking a pineal region tumour: a rare cause of Hakim triad.

A dural arteriovenous fistula (DAVF) is a dural-based shunt between meningeal arteries and meningeal veins, sinuses and/or cortical veins; they have been classified and named according to the location and the flow pattern. Petrous apex DAVFs are located where the petrosal vein penetrates the dura mater into the superior petrosal sinus; there are only few cases reported in the literature, they can show an aggressive behaviour (subarachnoid haemorrhage, severe brainstem oedema) with a high mortality rate. The described case is, to the best of our knowledge, the first case of a DAVF presenting with symptoms mimicking idiopathic normal pressure hydrocephalus. After worsening of gait impairment, memory loss and urinary incontinence an urgent CT of the brain showed hydrocephalus and a hyperdense mass in the pineal region mimicking a pineal tumour; an emergent digital subtraction angiogram showed a left petrous apex Borden type III DAVF. A transvenous embolisation was performed obtaining a complete obliteration.

Optic neuropathy, a warning for cerebral venous sinus thrombosis and underlying dural arteriovenous fistulae.

Cerebral venous sinus thrombosis (CVST) typically presents as headache, papilledema, and seizures. A dural arteriovenous fistula (DAVF) is a rare intracranial vascular malformation, and common symptoms include headache, pulsatile tinnitus, and stroke. The occurrence of CVST as a complication of DAVF is infrequent. Moreover, optic neuropathy presenting as the initial symptom of CVST and secondary DAVF is also unusual. We present a case of a patient with optic neuropathy and persistent intracranial hypertension who underwent head magnetic resonance imaging, which indicated CVST. She received normative anticoagulant and dehydration therapy; however, a repeated lumbar puncture showed dramatically increased intracranial pressure. Further digital subtraction angiography revealed an intracranial DAVF. The patient was finally diagnosed with a DAVF and secondary CVST. This case indicates that intractable optic neuropathy could be an uncommon indicator for CVST and secondary DAVF. Early diagnosis and early treatment are essential for visual rehabilitation and prognosis improvement.

[Capillary telangiectasia]. / Kapilläre Teleangiektasie.

Brain capillary telangiectasia is usually a small collection of dilated capillary-like vessels. In most cases it is a harmless incidental finding with no clinical significance. They are most commonly located in the pons. In terms of image morphology, they show brush-like signal extinction in T2*/SWI (susceptibility-weighted imaging) sequences and contrast enhancement in T1-weighted images. Other sequences are usually unremarkable unless they involve unusually large capillary telangiectasias. Angiographically they usually remain silent. Sometimes they are associated with venous abnormalities and/or cavernomas. Their distinctive radiographic features usually allow for a reliable diagnosis. Differential diagnostic considerations, such as differentiation from a tumorous or inflammatory process, are sometimes necessary.