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J Neurosurg 2022 May 20:1-10. Epub 2022 May 20.

3Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei.

Objective: The goal of the study was to define and quantify brain arteriovenous malformation (bAVM) compactness and to assess its effect on outcomes after Gamma Knife radiosurgery (GKRS) for unruptured bAVMs.

Methods: Unsupervised machine learning with fuzzy c-means clustering was used to differentiate the tissue constituents of bAVMs on T2-weighted MR images. The percentages of vessel, brain, and CSF were quantified. Read More

Clin Med (Lond) 2022 05;22(3):278-279

The First Affiliated Hospital of Guangdong Pharmaceutical University, Guangdong, China.

A 38-year-old man was admitted to the hospital due to a "suddenly developed right hemiplegia, unconsciousness and gaze to the right". Pulmonary arteriovenous fistulas (PAVFs) are rare but an important cause of stroke in young people, which is easy to be clinically neglected. Therefore, for young patients with pulmonary diseases and cerebral infarction, the possibility of PAVF should be considered. Read More

BMC Neurol 2022 May 2;22(1):164. Epub 2022 May 2.

The National Clinical Research Center for Mental Disorders & Beijing Key Laboratory of Mental Disorders, Beijing Anding Hospital, Capital Medical University, Beijing, China.

Objective: To describe the characteristics of patients with cerebral venous sinus thrombosis (CVST) and dural arteriovenous fistula (AVF) associated with protein S (PS) deficiency.

Methods: We conducted a search of medical records in Hainan General Hospital from January 2000 to December 2020 for coexistence of CVST and dural AVF associated with PS deficiency and searched PubMed、Embase and Chinese biomedical databases (CBM) for all literature describing CVST and dural AVF with PS. We analyzed clinical characteristics, location, sequence of CVST and dural AVF, level of PS, therapeutic methods and prognosis. Read More

Medicine (Baltimore) 2022 Mar 11;101(10):e29057. Epub 2022 Mar 11.

Department of Neurology, Chonnam National University Medical School & Hospital, Gwangju, South Korea.

Rationale: Septic cavernous sinus thrombosis (SCST) is a rare but life-threatening condition that commonly arises from infections, including paranasal sinusitis, otitis media, and skin infection. Meanwhile, head trauma as a predisposing factor of SCST has been scarcely reported. We report a case of SCST complicated by meningitis after minor head trauma, even in the absence of identifiable fractures. Read More

Clin Neurol Neurosurg 2022 06 31;217:107236. Epub 2022 Mar 31.

Neurosurgery Unit, Department of Neurosciences, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. Electronic address:

De novo arterio-venous malformations (AVMs) of the brain have been rarely previously reported, especially in the pediatric population. Although AVMs have possible connections with other diseases, the association with congenital portosystemic shunt (CPSS) has never been reported before. A child was followed for CPSS and cutaneous and hepatic angiomas. Read More

BMJ Case Rep 2022 Mar 31;15(3). Epub 2022 Mar 31.

Radiodiagnosis, Command Hospital Panchkula, Panchkula, India.

Isolated deep cerebral venous thrombosis (CVT), especially involving only the right thalamus, is one of the rarest forms of intracranial venous thrombosis in a child. The anatomy and flow patterns of the deep cerebral venous system are complex and usually, the thrombosis of the internal cerebral veins (ICV) results in thalamic infarction bilaterally. The focal infections, thalamic tumours and vascular malformations may have overlapping clinicoradiological patterns. Read More

J Clin Neurosci 2022 May 21;99:268-274. Epub 2022 Mar 21.

Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Level 3, 75 Talavera Rd, Macquarie University, Sydney, NSW 2109, Australia; The Centre for Complementary Medicine Research, Western Sydney University, 158 Hawkesbury Rd, Westmead, NSW 2145, Australia.

Brain arteriovenous malformation (bAVM) resection imposes several post-operative clinical challenges including intracranial haemorrhage (ICH). Daily non-invasive monitoring of haemodynamic measurements may be useful in predicting post-operative ICH. This prospective study used transcranial colour duplex (TCCD) and central aortic pressure (CAP) measurements to evaluate 15 bAVM patients pre-operatively and daily ≤ 14 days post-operatively. Read More

World Neurosurg 2022 Mar;159:390-401

The Neurosurgical Atlas, Carmel, Indiana, USA; Department of Neurological Surgery, Indiana University, Indianapolis, Indiana, USA. Electronic address:

Background: Surgical management of brainstem cavernous malformations (CMs) is a controversial topic in the field of neurosurgery. These lesions have a propensity to hemorrhage, thereby disrupting surrounding brainstem eloquence.

Methods: This article provides the personal reflections of the senior author and a narrative literature review on resection of brainstem CMs. Read More

Pediatr Neurol 2022 04 30;129:24-30. Epub 2021 Dec 30.

Department of Diagnostic Imaging & Image-Guided Therapy, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Background: Hereditary hemorrhagic telangiectasia (HHT) is a multiorgan vascular dysplasia with limited data regarding its neurovascular manifestations and genotype-phenotype correlation in children. The objective of this study was to describe the neurovascular findings in a large cohort of children with HHT and correlate between phenotype and genotype.

Methods: This retrospective study was conducted on 221 children (<18 years) with a definite or possible diagnosis of HHT based on Curacao criteria, or with positive genetics for the mutated genes of ENG, ACVRL-1, and SMAD-4, who also underwent brain MRI and/or conventional angiography. Read More

BMJ Case Rep 2022 Feb 9;15(2). Epub 2022 Feb 9.

Department of Neurology, G S Neuroscience Clinic and Research Center, Patna, Bihar, India.

A male in his early teens presented with redness of the right eye following a fall. This redness was progressive and increased suddenly over the week prior to presentation. Fundus evaluation revealed an exudative retinal detachment, and a bruit was audible over the right eye. Read More

Neurology 2022 03 4;98(13):550-553. Epub 2022 Feb 4.

From the Departments of Neurology (P.R.A., S.K., S.G.L., M.E.I., J.C.G.-M.), Neurosurgery (E.V.-L.), and Radiology (L.A.M., J.J.G.M., E.S.M.D.), Osakidetza Basque Health Service, Basurto University Hospital, Bilbao, Spain; and University of the Basque Country (J.C.G.-M.).

Deep cerebral venous thrombosis is an uncommon condition, which usually produces headache, altered consciousness, and ocular movement abnormality. Parkinsonism occasionally occurs when there is basal ganglia involvement. We report a case of a 78-year-old man who presented with a rapidly progressive parkinsonism with poor response to dopaminergic therapy. Read More

Adv Tech Stand Neurosurg 2022 ;44:17-53

Department of Neurosurgery, Yeditepe University School of Medicine, Istanbul, Turkey.

Over the past 30 years, the treatment of deep and eloquent arteriovenous malformations (AVMs) has moved away from microneurosurgical resection and towards medical management and the so-called minimally invasive techniques, such as endovascular embolization and radiosurgery. The Spetzler-Martin grading system (and subsequent modifications) has done much to aid in risk stratification for surgical intervention; however, the system does not predict the risk of hemorrhage nor risk from other interventions. In more recent years, the ARUBA trial has suggested that unruptured AVMs should be medically managed. Read More

World Neurosurg 2022 May 29;161. Epub 2022 Jan 29.

Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo, Sao Paulo, Brazil; Hospital Beneficência Portuguesa de São Paulo, São Paulo, Sao Paulo, Brazil. Electronic address:

Cerebral arteriovenous malformations (AVMs) are dynamic neurovascular disorders that occur mainly in young adults, presenting an annual risk of rupture of 2% - 4% per year. They can be asymptomatic, representing an incidental radiologic finding, or present with neurologic deficits according to their brain location, size, and presence or absence of bleeding. AVMs located in eloquent areas represent a great challenge for neurosurgeons, sometimes directed to alternatives therapies (e. Read More

Prog Brain Res 2022 6;268(1):99-114. Epub 2022 Jan 6.

Department of Neurosurgery, Haukeland University Hospital, Bergen, Norway. Electronic address:

Dural Arteriovenous Malformations (DAVFs) are rare vascular malformations involving the dura and cerebral veins. They occur in a variety of locations but in general the cavernous sinus lesion is more frequent among Asian patients while the transverse sinus is more usual among western patients. Treatments include microsurgery, endovascular embolization and GKNS. Read More

Sci Rep 2022 01 17;12(1):786. Epub 2022 Jan 17.

Department of Radiation Medicine and Applied Sciences, University of California San Diego, 3960 Health Sciences Dr, Mail Code 0865, La Jolla, CA, USA.

Stereotactic radiosurgery planning for cerebral arteriovenous malformations (AVM) is complicated by the variability in appearance of an AVM nidus across different imaging modalities. We developed a deep learning approach to automatically segment cerebrovascular-anatomical maps from multiple high-resolution magnetic resonance imaging/angiography (MRI/MRA) sequences in AVM patients, with the goal of facilitating target delineation. Twenty-three AVM patients who were evaluated for radiosurgery and underwent multi-parametric MRI/MRA were included. Read More

Eur J Med Res 2022 Jan 13;27(1):10. Epub 2022 Jan 13.

Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 100053, China.

Purpose: Unilateral jugular stenosis is easily mistaken as jugular hypoplasia for their similar jugular appearances. This study aimed to propose a scheme to differentiate acquired internal jugular vein stenosis (IJVS) from congenital jugular variation through two case examples.

Methods: We presented a dynamic evolution process of the IJVS formation, through a case of a 17-year-old female with paroxysmal nocturnal hemoglobinuria (PNH)-associated right internal jugular venous thrombosis (IJVT), which resulted in post-thrombotic IJVS in the rare context of rapid recanalization. Read More

Tomography 2021 11 25;7(4):804-814. Epub 2021 Nov 25.

Department of Neuroradiology, Ospedale del Mare, Via Enrico Russo, 80147 Naples, Italy.

(1) Background: Dural arteriovenous fistulas (DAVF) represent a rare acquired intracranial vascular malformation, with a variety of clinical signs and symptoms, which make their diagnosis difficult. Intracranial hemorrhage is one of the most serious clinical manifestations. In this paper the authors' goal was to verify the accuracy and utility of contrast-enhanced brain CT angiography (CTA) for the identification and the characterization of dural arteriovenous fistulas (DAVFs) in patients who presented with brain hemorrhage compared to 3D digital subtraction angiography (3D DSA); (2) a retrospective study of 26 patients with DAVFs who presented with intracranial hemorrhage to our institution was performed. Read More

Arq Neuropsiquiatr 2022 01;80(1):3-12

Hospital Beneficência Portuguesa, Departamento de Neurorradiologia Intervencionista, São Paulo SP, Brazil.

Background: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate.

Objective: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation.

Methods: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Read More

Cold Spring Harb Mol Case Stud 2021 12 9;7(6). Epub 2021 Dec 9.

Seattle Children's Hospital, Division of Genetic Medicine, Department of Pediatrics, University of Washington, Seattle, Washington 98105, USA.

Disorganized morphogenesis of arteries, veins, capillaries, and lymphatic vessels results in vascular malformations. Most individuals with isolated vascular malformations have postzygotic (mosaic), activating pathogenic variants in a handful of oncogenes within the PI3K-RAS-MAPK pathway (Padia et al., 4: 170-173 [2019]). Read More

Neurology 2022 02 8;98(7):292-295. Epub 2021 Dec 8.

From the Department of Clinical Neurosciences (J.I.R., A.K.);Department of Pediatrics (K.W., A.K., M.J.E.), Cumming School of Medicine, University of Calgary; Alberta Children's Hospital Research Institute (A.K., M.J.E.), University of Calgary; Hotchkiss Brain Institute (A.K., M.J.E.), University of Calgary, Alberta, Canada.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant condition that is linked to a myriad of neurologic complications arising from vascular malformations of the brain, spinal cord, and lungs. Our case describes a previously healthy 3-year-old male who presented to hospital with fever of unknown origin and was found to have a brain abscess stemming from a pulmonary arteriovenous malformation (PAVM). This etiology was identified after a period of diagnostic delay; the medical team was suspicious for a proximal embolic source due to the presence of multiple tiny infarcts seen on MRI of the brain, but transthoracic echocardiogram and head and neck angiogram were unremarkable. Read More

Ann Palliat Med 2021 Nov;10(11):12034-12038

Department of Neurology, Huzhou Cent Hospital, Affiliated Cent Hospital Huzhou University, Huzhou, China.

In recent years, the incidence of ischemic stroke in young people has gradually increased. The etiology and risk factors of young patients with ischemic stroke are complex, including migraine with aura, inherited thrombophilias, hyperhomocysteinemia, cardiovascular risk factors and malignancy. Pulmonary arteriovenous malformation (PAVM) is a rare cause of ischemic stroke in young people, which can easily be misdiagnosed and missed due to the lack of specific biomarkers. Read More

BMJ Case Rep 2021 Nov 30;14(11). Epub 2021 Nov 30.

Department of Neonatal Medicine, Shrewsbury and Telford Hospital NHS Trust, Telford, UK

Neurology 2022 02 19;98(5):e555-e560. Epub 2021 Nov 19.

From the Maxine Mesinger Multiple Sclerosis Comprehensive Care Center (S.A., G.J.H., F.X.C.) and Departments of Neurology and Neurosurgery (O.T.), Baylor College of Medicine, Houston, TX.

We present the case of a 57-year-old man with protein S deficiency and left leg deep vein thrombosis (DVT) 5 years earlier, who developed stepwise progressive bilateral lower limb weakness, numbness/paresthesia, gait imbalance, hesitancy of micturition, and constipation in the setting of recurrent left common femoral DVT treated with apixaban. Symptoms amplified with Valsalva, corticosteroids, and postlumbar puncture, with longitudinally extensive midthoracic T2-hyperintense lesion extending to the conus associated with hazy holocord enhancement on magnetic resonance imaging (MRI), raising suspicion for spinal dural arteriovenous fistula (sDAVF). Initial digital subtraction angiography (DSA) was negative for sDAVF. Read More

Medicine (Baltimore) 2021 Nov;100(46):e27892

Department of Ophthalmology, the Second Affiliated Hospital of Nanchang University, Nanchang, Jiangxi Province, China.

Rationale: Cavernous sinus dural arteriovenous fistula (CSDAVF) is a rare intracranial vascular malformation. Because of its complicated clinical manifestations, it is easy to miss or misdiagnose CSDAVF.

Patient Concerns: A 42-year-old female had chief complaint that the right eyeball had conjunctival congestion for half a year. Read More

BMJ Case Rep 2021 Nov 11;14(11). Epub 2021 Nov 11.

Neurological Surgery, University of Miami School of Medicine, Miami, Florida, USA.

Torcular dural sinus malformations (tDSMs) represent a rare subset of paediatric cerebrovascular malformations and are often diagnosed antenatally via ultrasound. The management of these in utero lesions remains controversial as previous studies suggested elective termination of the pregnancy because of their presumably high mortality and severe long-term morbidity. However, more recent evaluations have suggested that the overall prognosis for infants harbouring these lesions may be much better than previously believed. Read More

J Neurosurg 2021 Nov 5:1-12. Epub 2021 Nov 5.

1Barrow Aneurysm and AVM Research Center, Department of Translational Neuroscience, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix.

Objective: Hereditary hemorrhagic telangiectasia is the only condition associated with multiple inherited brain arteriovenous malformations (AVMs). Therefore, a mouse model was developed with a genetics-based approach that conditionally deleted the causative activin receptor-like kinase 1 (Acvrl1 or Alk1) gene. Radiographic and histopathological findings were correlated, and AVM stability and hemorrhagic behavior over time were examined. Read More

Radiographics 2021 Nov-Dec;41(7):2157-2175

From the Russell H. Morgan Department of Radiology and Radiological Science (A.R.K., A.J.S., C.B., M.A.L., C.R.W.), Department of Otolarygology-Head and Neck Surgery (N.R.R.), and Department of Medicine, Division of Pulmonology (P.G.), The Johns Hopkins University School of Medicine, 1800 Orleans St, Sheikh Zayed Tower, Baltimore, MD 21287.

Hemorrhagic hereditary telangiectasia (HHT) is a rare autosomal dominant disorder that causes multisystem vascular malformations including mucocutaneous telangiectasias and arteriovenous malformations (AVMs). Clinical and genetic screening of patients with signs, symptoms, or a family history suggestive of HHT is recommended to confirm the diagnosis on the basis of the Curaçao criteria and prevent associated complications. Patients with HHT frequently have epistaxis and gastrointestinal bleeding from telangiectasias. Read More

AJNR Am J Neuroradiol 2022 02 21;43(2):160-166. Epub 2021 Oct 21.

From the Divisions of Neuroradiology (K.D.B., H.L., H.K., J.K., W.G., T.K., V.M.P.).

Intracranial dural AVFs are abnormal communications between arteries that supply the dura mater and draining cortical veins or venous sinuses. They are believed to form as a response to venous insults such as thrombosis, trauma, or infection. Classification and management are dependent on the presence of drainage/reflux into cortical veins because such drainage markedly elevates the risk of hemorrhage or venous congestion, resulting in neurologic deficits. Read More

World Neurosurg 2022 01 12;157:88-90. Epub 2021 Oct 12.

Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, China. Electronic address:

Cauda equina arteriovenous malformation (AVM) is extremely rare. To our knowledge, only 2 cases have been reported previously and are supplied by a unilateral lateral sacral artery (LSA). Here, we report a rare cauda equina AVM at the level of L5-S1, which is supplied by the bilateral LSAs. Read More

AJNR Am J Neuroradiol 2022 04 14;43(4):510-516. Epub 2021 Oct 14.

From the Division of Neuroradiology (K.D.B., H.L., H.K., J.K., W.G., T.K., V.M.P.).

In this third review article on the endovascular management of intracranial dural AVFs, we discuss transvenous embolization approaches. Transvenous embolization is increasingly popular and now the first-line approach for ventral dural AVFs involving the cavernous sinus and hypoglossal canal. In addition, transvenous embolization is increasingly used in lateral epidural dural AVFs in high-risk locations such as the petrous and ethmoid regions. Read More