A case series of intracranial dural arteriovenous fistulae mimicking cervical myelitis: a diagnosis not to be missed

Objective To describe the diagnostic features of intracranial dural arteriovenous fistulae (DAVF) presenting with cervical cord or brainstem swelling. Methods Retrospective case note and neuroimaging review of patients with angiographically confirmed DAVF diagnosed during January 2015–June 2020 at a tertiary neuroscience centre (Walton Centre NHS Foundation Trust, Liverpool, UK). Results Six intracranial DAVF causing cervical cord or brainstem oedema (all males aged 60–69 years) and 27 spinal DAVF (88% thoracolumbar) were detected over a 5.5-year period. Significantly more patients with intracranial DAVF received steroids for presumed inflammatory myelitis than those with spinal DAVF (5/6 vs 1/27, p = 0.0001, Fisher’s exact test). Several factors misled the treating clinicians: atypical rostral location of cord oedema (6/6); acute clinical deterioration (4/6); absence (3/6) or failure to recognise (3/6) subtle dilated perimedullary veins on MRI; intramedullary gadolinium enhancement (2/6); and elevated CSF protein (4/5). Acute deterioration followed steroid treatment in 4/5 patients. The following features may suggest DAVF rather than myelitis: older male patients (6/6), symptomatic progression over 4 or more weeks (6/6) and acellular CSF (5/5). Conclusion Intracranial DAVF are uncommon but often misdiagnosed and treated as myelitis, which can cause life-threatening deterioration. Neurologists must recognise suggestive features and consider angiography, especially in older male patients. Dilated perimedullary veins are an important clue to underlying DAVF, but may be invisible or easily missed on routine MRI sequences.

Transverse-igmoid sinus dural arteriovenous fistula presenting with recurrent transient ischaemic attack: A technical case report.

Intracranial dural arteriovenous fistulae (DAVF) are uncommon and account for approximately 1% of all strokes. All transverse-sigmoid sinus DAVFs require treatment because of the low rate of spontaneous regression and frequent association with aggressive neurologic symptoms. Endovascular embolization which aims for permanent obliteration of the lesion is now the primary treatment modality for all DAVFs. We present a 56-year-old patient with long standing history of intermittent TIA-like symptoms. Magnetic resonance (MR) angiography revealed right transverse-sigmoid sinus DAVF and bilateral vertebral arteries occlusion. The patient’s symptoms completely resolved upon successful trans-arterial embolization of the DAVF.

Microsurgical Findings of Pial Arterial Feeders in Intracranial Dural Arteriovenous Fistulae: A Case Series

BACKGROUND Pial arterial feeders in an intracranial dural arteriovenous fistula (dAVF) are risk factors for both ischemic and hemorrhagic complications during endovascular embolization. Microsurgery in dAVF with pial arterial feeders has rarely been reported. OBJECTIVE To assess our original experience with microsurgery for dAVF with pial arterial feeders by investigating surgical findings and outcomes. METHODS In 40 patients with intracranial dAVF who underwent microsurgery, we found 8 patients who had pial arterial feeders. A retrospective review was conducted. RESULTS The locations of the dAVFs were as follows: tentorium, 2 patients; Galenic system, 1; straight sinus, 1; torcula, 1; transverse sinus, 1; ethmoid, 1; and convexity, 1. Preoperative angiography revealed that the pial arterial feeders originated from the middle cerebral artery in 2 patients, the anterior cerebral artery in 1, the posterior cerebral artery in 2, and the posterior medial choroidal artery in 1. Of note, in 2 patients (6.7%), feeders from the superior cerebellar artery were determined to be angiographically occult during preoperative examination and were detected through careful intraoperative observation and arachnoid dissection. In 5 cases, the additional obliteration of the pial arterial feeders and/or more distal cortical venous drainers after the simple disconnection of proximal cortical drainers was necessary to cure the dAVF. Finally, all shunts were cured with only 1 minor complication. CONCLUSION Although microsurgical results were acceptable, the surgeon should be aware of the presence of pial arterial feeders even after the simple disconnection of cortical venous drainage. Angiographically occult feeders may also exist.

The role of angiogenesis in dural arteriovenous fistulae: the story so far

Intracranial dural arteriovenous fistulae are a commonly encountered pathology that can present with a variety of different clinical symptoms. Although there is a significant body of work relating to the natural history and treatment of dural arteriovenous fistulae the exact underlying pathogenesis remains elusive. Various different pathogenetic models have been put forward but there is now a growing body of evidence implicating angiogenesis and the involvement of angiogenetic factors. In this review we attempt to show how the various animal and human studies performed over the past two decades have contributed to the proposed hypothesis on the development of dural arteriovenous fistulae.

Progressive Neurological Decline with Deep Bilateral Imaging Changes: A Protean Presentation of Dural Arteriovenous Fistulae

Intracranial dural arteriovenous fistulae (DAVF) within the deep cerebral vasculature are diagnostically challenging because of their variable clinical presentation and typical bilateral neuroimaging findings mimicking inflammatory, infectious, and metabolic processes. Increasingly, reports have emerged highlighting the diagnostic and treatment challenges of these lesions and their associated high morbidity and rapid clinical deterioration when untreated. We describe here a case series of 4 patients with deep cerebral DAVF who presented with impaired arousal or memory and behavioral changes. In all patients, the initial differential diagnosis included metabolic, inflammatory, infectious, or neoplastic disease, with an eventual correct diagnosis obtained after catheter angiography had demonstrated arterialization of the deep venous structures, including the vein of Galen. All patients were successfully treated with endovascular embolization, with 1 patient requiring additional surgical treatment. We review the contemporary diagnostic evaluation and management of DAVF within the deep cerebral vasculature. With rapid diagnosis and treatment, a favorable outcome is possible.