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J Neuroinflammation 2022 Jun 22;19(1):165. Epub 2022 Jun 22.

Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, No 45 Changchun Street, Xicheng District, Beijing, 100053, China.

Background And Purpose: A major challenge in spinal dural arteriovenous fistula (SDAVF) is timely diagnosis, but no specific predictive biomarkers are known.

Methods: In the discovery cohort (case, n = 8 vs. control, n = 8), we used cerebrospinal fluid (CSF) and paired plasma samples to identify differentially expressed proteins by label-free quantitative proteomics. Read More

Neurointervention 2022 Jun 15. Epub 2022 Jun 15.

Neurointervention Clinic, Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

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. Read More

Ulus Travma Acil Cerrahi Derg 2022 Jun;28(6):871-875

Department of Neurosurgery, Ordu University Faculty of Medicine, Ordu-Turkey.

The arteriovenous fistulas (AVFs) of the vertebral artery are usually caused by iatrogenic and penetrating traumas. Vertebral AVF is rarely seen after blunt cervical trauma. A 65-year-old male patient applied to the emergency clinic due to falling from a height of about 3 m. Read More

World Neurosurg 2022 May 18;164:239. Epub 2022 May 18.

Neurosurgical Service, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA. Electronic address:

Vascular malformations in the dura of the spinal root sleeve in the thoracic or lumbar region can cause venous hypertension with resultant spinal cord edema and neurologic deterioration. However, occasionally an intracranial vascular malformation can have venous drainage into the spinal canal causing symptoms, signs, and radiographic appearance that can mimic spinal lesions. Herein, we present the case of a 73-year-old man who developed lower extremity weakness and loss of sensation. Read More

J Radiol Case Rep 2022 Mar 1;16(3):15-22. Epub 2022 Mar 1.

Anatomical Pathology Department, Faculty of Medicine, Universitas Airlangga, Indonesia.

Intramedullary cavernous angioma is a rare vascular malformation compared to cerebral cavernous malformation. The incidence of cavernous angioma is about 3 - 5% of all central nervous system lesions, 5 - 12% of all spinal vascular lesions, and 1% of all intramedullary lesions in pediatric patients. Although intramedullary cavernous angioma has the same histological picture as cerebral cavernous angioma, the natural history, and surgical approach are different from cerebral cavernous angioma. Read More

Hum Genet 2022 Apr 30. Epub 2022 Apr 30.

Department of Molecular Genetics and Microbiology, Duke University School of Medicine, Durham, NC, 27710, USA.

Cerebral cavernous malformations (CCM) are vascular malformations consisting of collections of enlarged capillaries occurring in the brain or spinal cord. These vascular malformations can occur sporadically or susceptibility to develop these can be inherited as an autosomal dominant trait due to mutation in one of three genes. Over a decade ago, we described a 77. Read More

Acta Neurochir (Wien) 2022 06 29;164(6):1669-1673. Epub 2022 Apr 29.

Department of Neurosurgery, La Timone Hospital (Assistance Publique - Hôpitaux de Marseille), Marseille, France.

Background: Spinal dural arteriovenous fistula (d-AVF) is the most common spinal vascular malformations. Management includes endovascular embolization, and/or surgical obliteration of the shunt.

Method: Applied to spinal d-AVF, mini-invasive surgical (MIS) obliteration is described as a mini-open approach using Mast Quadrant™ system. Read More

Neurochirurgie 2022 Apr 25. Epub 2022 Apr 25.

Department of Neurosurgery, GHU site Sainte-Anne, Paris, France; Université de Paris, 102-108 rue de la Santé, 75014 Paris, France; Institut de Psychiatrie et Neurosciences de Paris (IPNP), UMR S1266, Inserm, IMA-BRAIN, Paris, France. Electronic address:

Background: Spinal hemangiomas are rare vascular malformations resulting from proliferation of vascular endothelial cells. The cavernous form is the most common and represents 5-12% of spinal vascular malformations, while the capillary form is rare.

Case Description: A 56-year-old patient with no past medical history presented with progressive spinal cord compression symptoms localizing to the T10 level with MRC grade 4 proximal paraparesis. Read More

Neuroradiology 2022 Apr 22. Epub 2022 Apr 22.

Neuroradiology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

Purpose: The aim of the study was to assess the prevalence and characteristics of spinal cord cavernous malformations (SCCM) and intraosseous spinal vascular malformations (ISVM) in a pediatric familial cerebral cavernous malformation (FCCM) cohort and evaluate clinico-radiological differences between children with (SCCM +) and without (SCCM-) SCCM.

Methods: All patients with a pediatric diagnosis of FCCM evaluated at three tertiary pediatric hospitals between January 2010 and August 2021 with [Formula: see text] 1 whole spine MR available were included. Brain and spine MR studies were retrospectively evaluated, and clinical and genetic data collected. Read More

Neural Plast 2022 24;2022:4280410. Epub 2022 Mar 24.

Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Introduction: The association between arterial tortuosity and acute ischemic stroke (AIS) has been reported, but showing inconsistent results. We hypothesized that tortuosity of extra- and intracranial large arteries might be higher in AIS patients. Furthermore, we explored the correlation between artery tortuosity and white matter hyperintensity (WMH) severity in AIS patients. Read More

Neurosurg Clin N Am 2022 Apr;33(2):193-206

Department of Neurosurgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ 85013, USA. Electronic address:

With the rapid advancements in endovascular therapy over previous decades, the treatment of spinal arteriovenous malformations (AVMs) continues to evolve. The decision to use endovascular versus surgical therapy largely depends on the type of lesion and its anatomic location. Recent studies demonstrate that endovascular treatment is effective for extradural arteriovenous fistulas (AVFs), intradural ventral (perimedullary) AVMs, and intramedullary spinal AVMs. Read More

iScience 2022 Mar 18;25(3):103943. Epub 2022 Feb 18.

Vascular Biology Unit, The FIRC Institute of Molecular Oncology Foundation, Milan 20139, Italy.

Cavernomas are multi-lumen and blood-filled vascular malformations which form in the brain and the spinal cord. They lead to hemorrhage, epileptic seizures, neurological deficits, and paresthesia. An effective medical treatment is still lacking, and the available murine models for cavernomas have several limitations for preclinical studies. Read More

Neurol India 2022 Jan-Feb;70(1):54-56

Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Background And Introduction: Spinal dural arteriovenous fistula (SDAVF) is a rare but curable condition. Microsurgery is a highly effective and readily affordable treatment modality.

Objective: We present a surgical video of SDAVF to demonstrate the operative nuances involved. Read More

Neurol India 2022 Jan-Feb;70(1):1-2

Department of Neurosurgery, LSU Health Sciences, Shreveport, US.

J Med Case Rep 2022 Mar 1;16(1):99. Epub 2022 Mar 1.

Department of Respiratory Medicine, Mercy University Hospital, Cork, Ireland.

Introduction: Hereditary hemorrhagic telangiectasia is an autosomal dominant condition with an estimated prevalence of 1 in 5000. It is characterized by the presence of abnormalities of vascular structures, and may affect many organ systems, including the lungs, brain, spinal cord, gastrointestinal tract, and liver. A causative mutation is identified in approximately 97% of patients with definite hereditary hemorrhagic telangiectasia in one of three genes including a mutation in endoglin, a mutation in a locus mapped to chromosome 5, and an activin receptor-like kinase-1 (ACVRL1) mutation that is associated with an increased incidence of primary pulmonary hypertension. Read More

BMJ Case Rep 2022 Feb 28;15(2). Epub 2022 Feb 28.

Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Cobb syndrome is a rare neurocutaneous disorder characterised by spinal and cutaneous vascular malformations in a metameric distribution. Managing such cases is a challenge for the interventionists and neurosurgeons as the results are often suboptimal. We describe a case of Cobb syndrome in a young male child who presented clinically with acute paraparesis and lower backache. Read More

World Neurosurg 2022 Apr 29;160:67. Epub 2022 Jan 29.

Departments of Neurosurgery and Interventional Neuroradiology, Rothschild Foundation Hospital, Paris, France.

Dural arteriovenous fistulas are rare acquired vascular lesions that represent 15% of the vascular malformations. While endovascular treatment has recently became the first line of treatment, microsurgical ligation may still be indicated in specific cases. We present the case of a 75-year-old patient who presented a progressive tetraparesis culminating in a spastic paraplegia and urinary retention. Read More

NMC Case Rep J 2021 9;8(1):377-385. Epub 2021 Jul 9.

IRCCS/Institute of Neurological Science of Bologna, Department of Neurologic Surgery, Bellaria Hospital, Bologna, Italy.

Spinal intradural hemorrhage is a rare event; the most common causes of spinal bleeding are traumas, medical therapy with anticoagulants and thrombolytics, vascular malformations, and congenital defects of coagulation. Rarely, spinal cord tumors may cause hemorrhage. Herein, we report the case of a patient with acute and quickly worsening lumbar pain: the neurological examination revealed a flaccid paraplegia caused by an intradural lesion extending on the right side of the spinal cord from T1 to L2 vertebral level. Read More

Neurosurgery 2022 04;90(4):390-398

Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, China International Neuroscience Institute, Beijing, China.

Background: The natural history of spinal cord cavernous malformations (SCCMs) remains relatively unclear.

Objective: To investigate the natural history for hemorrhagic risks and neurological outcomes, as well as relevant predicting factors, of SCCMs.

Methods: All patients between 2002 and 2019 with diagnosis of SCCMs were identified retrospectively. Read More

BMJ Open 2022 01 3;12(1):e047390. Epub 2022 Jan 3.

Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China

Objective: To define the pattern of long-term clinical outcomes and prognostic factors in patients with spinal dural arteriovenous fistulas (SDAVFs).

Desgin: Prospective cohort study based on constantly recruiting patients with SDAVFs in two medical centres in China.

Setting: Patients with SDAVFs were recruited consecutively between March 2013 and December 2014 in two referral centres. Read More

J Neurosurg Spine 2021 Dec 17:1-5. Epub 2021 Dec 17.

1Department of Neurosurgery and Spine Surgery.

Objective: Cavernous spinal cord malformations (SCMs) are believed to have a high rate of bleeding. The risk of intramedullary hemorrhage (IMH) or recurrent IMH and the neurological impact of bleeding events are important for clinical decision-making and could impact current treatment strategies.

Methods: The authors screened their institutional database for patients with cavernous SCM treated between 2003 and 2020. 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

No Shinkei Geka 2021 Nov;49(6):1346-1352

Department of Neurosurgery, Osaka City University.

Spinal arteriovenous malformation(AVM)is a rare disease in which blood is received from the spinal feeding arteries. Owing to its rarity, there is limited experience regarding its diagnosis and treatment. In addition, the various classifications that have been proposed historically make it more difficult for the young neurosurgeons to understand this disease. Read More

Stroke 2022 05 1;53(5):1682-1691. Epub 2021 Dec 1.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, China (X.D., H.Y., Y.Z., D.Z., S.W., Y.C., M.L., J.Z.).

Background: Language dysfunction is rarely seen in patients with unruptured brain arteriovenous malformation (AVM) albeit the AVM nidus involving language areas, which provides a unique disease model to study language reorganization. The objective of this study was to investigate the impairment and reorganization patterns and characteristics of language-related white matter in AVMs located at different brain areas.

Methods: Thirty-three patients with AVMs involving language areas were prospectively enrolled. Read More

Br J Hosp Med (Lond) 2021 Nov 23;82(11):1-9. Epub 2021 Nov 23.

Liverpool Head and Neck Centre, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK.

Patients with hereditary haemorrhagic telangiectasia can present with a multitude of symptoms caused by telangiectasia and arteriovenous malformations in the nose, brain, gastrointestinal tract, liver and spinal cord. Clinicians should be aware of the potential diagnosis of hereditary haemorrhagic telangiectasia and how to manage these patients both in the acute and chronic setting. Identifying these patients and optimising their management can help reverse the reduced life expectancy back to that of the normal population. Read More

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

Neurol Med Chir (Tokyo) 2022 Feb 12;62(2):65-74. Epub 2021 Nov 12.

Department of Neurosurgery, Kurume University School of Medicine.

Spinal arteriovenous (AV) shunts are rare conditions that sometimes present with myelopathy symptoms. The progression of the symptoms is usually gradual; however, some cases show rapid deterioration. We retrospectively investigated the factors that induced the rapid deterioration of myelopathy symptoms in patients with spinal AV shunts. Read More

J Neurosurg Spine 2022 04 29;36(4):670-677. Epub 2021 Oct 29.

1Department of Neurological Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama.

Objective: There have been no accurate surveillance data regarding the incidence rate of spinal arteriovenous shunts (SAVSs). Here, the authors investigate the epidemiology and clinical characteristics of SAVSs.

Methods: The authors conducted multicenter hospital-based surveillance as an inventory survey at 8 core hospitals in Okayama Prefecture between April 1, 2009, and March 31, 2019. Read More

AJNR Am J Neuroradiol 2021 12 14;42(12):2110-2118. Epub 2021 Oct 14.

Division of Neuroradiology (J.S., S.R.), Department of Radiology, University of Massachusetts Medical School, Worcester, Massachusetts.

Spinal vascular shunts, including fistulas and malformations, are rare and complex vascular lesions for which multiple classification schemes have been proposed. The most widely adopted scheme consists of 4 types: type I, dural AVFs; type II, intramedullary glomus AVMs; type III, juvenile/metameric AVMs; and type IV, intradural perimedullary AVFs. MR imaging and angiography techniques permit detailed assessment of spinal arteriovenous shunts, though DSA is the criterion standard for delineating vascular anatomy and treatment planning. Read More

Pediatr Neurosurg 2021 11;56(6):555-562. Epub 2021 Oct 11.

Neurosurgery Division, Department of Pediatric Surgery, Montreal Children's Hospital, McGill University, Montreal, Québec, Canada.

Introduction: The purpose of this report is to present a rare case of Hirayama disease (HD) in a patient with a history of late-onset symptomatic vein of Galen aneurysmal malformation (VGAM). This report may provide new insights into the pathophysiology of HD, a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the forearm and intrinsic hand muscles. These symptoms are believed to result from cervical myelopathy affecting the anterior horn cells due to abnormal compressive forces on the spinal cord from adjacent anatomical structures (i. Read More