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J Neuroophthalmol 2022 Jun 25;42(2):e469-e472. Epub 2022 Jan 25.

Baylor College of Medicine (JZ, SHB, AGL), Houston, Texas; Department of Ophthalmology (AGL, NB), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Houston Methodist Research Institute (AGL), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology (AGL), Neurology, and Neurosurgery, Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; and Department of Ophthalmology (AGL), the University of Iowa Hospitals and Clinics, Iowa City, Iowa.

Brain Behav Immun Health 2022 Jul 5;22:100464. Epub 2022 May 5.

Neurology & Neurophysiology Center, Vienna, Austria.

Mult Scler 2022 May 17:13524585221094241. Epub 2022 May 17.

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Background: Haemorrhagic demyelinating lesions are rare, and little is known about the demyelinating diseases with which they are associated, or how lesional haemorrhage affects treatment and outcomes.

Objective: To examine the clinical characteristics and outcomes of patients with demyelinating lesions and magnetic resonance imaging (MRI) evidence of haemorrhage seen at the Mayo clinic between 1990 and 2018.

Methods: The Mayo Clinic's medical-record diagnostic-linkage system was used to identify patients with CNS demyelinating disease and parenchymal haemorrhage on brain MRI cross-referenced against a database of patients with pathologically confirmed CNS demyelinating disease. Read More

BMJ Case Rep 2022 May 17;15(5). Epub 2022 May 17.

Internal Medicine, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India

Curr Med Imaging 2022 May 9. Epub 2022 May 9.

Department RADIOLOGY, Fortis Escorts Heart Institute & Research Centre, INDIA.

Introduction: A 67-year-old female with no significant past medical history presented to the critical care department with symptoms of encephalopathy.

Case Presentation: The patient's main concerns and important clinical findings-The patient had a history of COVID -19 vaccination [recombinant ChAdOX1 nCoV-19] 14 days prior to the symptoms. The patient underwent MRI brain and cervical spine and lumbar puncture. Read More

Nervenarzt 2022 May 12. Epub 2022 May 12.

Klinik für Neurologie und Experimentelle Neurologie, Charité - Universitätsmedizin Berlin, Hindenburgdamm 30, 12200, Berlin, Deutschland.

Numerous diseases of the central nervous system (CNS), especially in the postacute phase after an infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been described. These include neuroimmunologically mediated diseases, such as encephalopathy, encephalitis, myelitis, acute disseminated encephalomyelitis (ADEM), acute necrotizing hemorrhagic leukoencephalitis (ANHLE) and neuromyelitis optica spectrum disorder (NMOSD) as well as others, such as posterior reversible encephalopathy syndrome (PRES), opsoclonus myoclonus ataxia (OMA) and cerebrovascular diseases. A parainfectious or postinfectious association is discussed but the pathophysiological mechanisms are so far unknown. Read More

Vaccine 2022 May 27;40(24):3305-3312. Epub 2022 Apr 27.

Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada; ICES, Toronto, ON, Canada; Public Health Ontario, ON, Canada; Department of Family and Community Medicine, University of Toronto, Toronto, ON, Canada; University Health Network, Toronto, ON, Canada. Electronic address:

Background: Background incidence rates are critical in pharmacovigilance to facilitate identification of vaccine safety signals. We estimated background incidence rates of 11 adverse events of special interest related to COVID-19 vaccines in Ontario, Canada.

Methods: We conducted a population-based retrospective observational study using linked health administrative databases for hospitalizations and emergency department visits among Ontario residents. Read More

Brain 2022 May 5. Epub 2022 May 5.

Department of Neurology, Seoul National University Hospital, Jongno-gu, Seoul 03080, South Korea.

Seronegative autoimmune encephalitis (AE) is AE without any identifiable pathogenic antibody. Although it is a major subtype of AE, many unmet clinical needs exist in terms of clinical characteristics, treatments, and prognosis. In this institutional cohort study, patients diagnosed with seronegative AE with available 2-year outcomes were analysed for the disease course, 2-year outcome prediction system, effect of immunotherapy, necessity of further immunotherapy at 6 or 12 months, and pattern of brain atrophy. Read More

Acta Neurol Belg 2022 May 2. Epub 2022 May 2.

Department of Neurology, Vardhman Mahavir Medical College and Safdarjung Hospital, Room 837, Superspeciality Block, New Delhi, 110029, India.

Ann Med Surg (Lond) 2022 May 22;77:103662. Epub 2022 Apr 22.

Disciplina de Neurociência, Universidade Federal de São Paulo/Escola Paulista de Medicina (UNIFESP/EPM), São Paulo, Brazil.

Nucleus 2022 Dec;13(1):154-169

Department of Neurology, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, and the New York Presbyterian Hospital, New York.

Dominant missense mutations in RanBP2/Nup358 cause Acute Necrotizing Encephalopathy (ANE), a pediatric disease where seemingly healthy individuals develop a cytokine storm that is restricted to the central nervous system in response to viral infection. Untreated, this condition leads to seizures, coma, long-term neurological damage and a high rate of mortality. The exact mechanism by which RanBP2 mutations contribute to the development of ANE remains elusive. Read More

Cureus 2022 Mar 22;14(3):e23405. Epub 2022 Mar 22.

Pediatric Neurology, Nemours Children's Hospital, Wilmington, USA.

Viral infections can serve as a trigger for variable autoimmune, antibody-mediated demyelinating disorders. There is accumulating evidence that the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus, causing coronavirus disease 2019 (COVID-19) infection and responsible for the current worldwide pandemic, can lead to a cascade of immune-mediated brain and spinal cord demyelinating injuries. However, such observation in the pediatric age group was only reported in very few patients. Read More

Neurologia (Engl Ed) 2022 May 23;37(4):313-315. Epub 2022 Apr 23.

Servicio de Neurología, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.

Vaccine 2022 May 18;40(23):3150-3158. Epub 2022 Apr 18.

Research and Evaluation, Kaiser Permanente Southern California, Pasadena, CA, United States.

Background: The COVID-19 pandemic caused an abrupt drop in in-person health care (inpatient, Emergency Department, outpatient) and an increase in telehealth care, which poses challenges in vaccine safety studies that identify outcomes from in-person encounters. We examined the changes in incidence rates of selected encounter-based outcomes during the COVID-19 pandemic.

Methods: We assembled a cohort of members from 8 Vaccine Safety Datalink sites from January 1, 2017 through December 31, 2020. Read More

Front Neurol 2022 6;13:830488. Epub 2022 Apr 6.

Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.

Background: The clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is expanding over time. However, the long-term management and prognosis of this disorder are still controversial. Therefore, this study aimed to report the clinical profiles and treatment outcomes of MOGAD in our center. Read More

Life (Basel) 2022 Mar 28;12(4). Epub 2022 Mar 28.

Dipartimento di Diagnostica per Immagini, Radioterapia Oncologica ed Ematologia, U.O.C. Radiologia Diagnostica e Interventistica Generale, Fondazione Policlinico Universitario, "A. Gemelli"-IRCCS, L.go A. Gemelli 8, 00168 Rome, Italy.

The magnetic resonance characteristics of autoimmune demyelinating diseases are complex and represent a challenge for the radiologist. In this study we presented two different cases of detected autoimmune demyelinating diseases: one case of acute disseminated encephalomyelitis and one case of neuromyelitis optica, respectively. Expected and unexpected findings of magnetic resonance imaging examination for autoimmune demyelinating diseases were reported in order to provide a valuable approach for diagnosis. Read More

Mult Scler Relat Disord 2022 Mar 12;62:103738. Epub 2022 Mar 12.

Department of Radiology, Diagnostic Centre, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.

Background: MRI of the nervous system is the critical in distinguishing pediatric MS from acute disseminated encephalomyelitis (ADEM). Our aim was to propose MRI criteria to distinguish MS from monophasic ADEM based on the first MRI and to validate previously proposed MRI criteria.

Methods: A neuroradiologist undertook retrospective evaluation of the MRI at the first demyelinating event in children (<18 years) with medical record-validated MS and ADEM in Denmark during 2008-15. Read More

J Assoc Physicians India 2022 Mar;70(3):11-12

Consultant, Seven Hills Hospital, Mumbai, Maharashtra.

As of August 2021, the COVID -19 pandemic has affected approximately 200 million cases worldwide. Most of the reported medical literature about the COVID-19 infection discusses its respiratory and haematological manifestations, with limited information about its neurological complications. Encephalitis, meningitis, acute disseminated encephalomyelitis, stroke and encephalopathy have been reported in patients with COVID-19 infection. Read More

J Child Neurol 2022 Apr 18:8830738221089476. Epub 2022 Apr 18.

Department of Pediatric Neurology, 14903Charité-Universitätsmedizin Berlin, Berlin, Germany.

Objective: To introduce and evaluate a modified version of the "zipper method"-a treatment strategy alternating intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) first reported for 9 pediatric cases of Guillain-Barré syndrome in 2018-for treatment of severe immune-mediated neurologic disorders in children.

Methods: The modified zipper method comprised longer intervals between PLEX-IVIG cycles (48hours instead of 24hours), more cycles (7-10 instead of 5), a consistent plasma volume exchange (instead of the original multistep approach), and variable infusion times for IVIGs (4-8hours). The modified zipper method was applied as an individual treatment approach once standard therapy failed. Read More

Cureus 2022 Mar 14;14(3):e23150. Epub 2022 Mar 14.

Neurosciences, Hamad General Hospital, Doha, QAT.

Introduction Worldwide, there are more than 424 million confirmed cases of COVID-19. Most of the hospitalized critical COVID-19 patients manifested neurological signs and symptoms and higher mortality. The majority of COVID-19 fatalities occurred mostly in patients with advanced age and underlying medical comorbidities. Read More

Int J Mol Sci 2022 Mar 30;23(7). Epub 2022 Mar 30.

Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy.

Autoimmune demyelinating diseases-including multiple sclerosis, neuromyelitis optica spectrum disorder, anti-myelin oligodendrocyte glycoprotein-associated disease, acute disseminated encephalomyelitis, and glial fibrillary acidic protein (GFAP)-associated meningoencephalomyelitis-are a heterogeneous group of diseases even though their common pathology is characterized by neuroinflammation, loss of myelin, and reactive astrogliosis. The lack of safe pharmacological therapies has purported the notion that cell-based treatments could be introduced to cure these patients. Among stem cells, mesenchymal stem cells (MSCs), obtained from various sources, are considered to be the ones with more interesting features in the context of demyelinating disorders, given that their secretome is fully equipped with an array of anti-inflammatory and neuroprotective molecules, such as mRNAs, miRNAs, lipids, and proteins with multiple functions. Read More

Int J Mol Sci 2022 Mar 24;23(7). Epub 2022 Mar 24.

Department of Immunology, School of Basic Medical Sciences, Fujian Medical University, Fuzhou 350108, China.

Ran Binding Protein 2 (RanBP2 or Nucleoporin358) is one of the main components of the cytoplasmic filaments of the nuclear pore complex. Mutations in the gene are associated with acute necrotizing encephalopathy type 1 (ANE1), a rare condition where patients experience a sharp rise in cytokine production in response to viral infection and undergo hyperinflammation, seizures, coma, and a high rate of mortality. Despite this, it remains unclear howRanBP2 and its ANE1-associated mutations contribute to pathology. Read More

Clin Nucl Med 2022 Apr 5. Epub 2022 Apr 5.

From the Department of Nuclear Medicine, Ewha Womans University College of Medicine, Seoul, South Korea.

Abstract: An 11-year-old boy who presented with headache and progressive right-sided weakness exhibited cortical swelling in the parafalcine area of both frontoparietal high convexity and splenium portion of corpus callosum on brain MRI. This suggested the possibility of encephalopathy, but required differential diagnosis from brain tumor. 18F-FET (O-(2-[18F]fluoroethyl)-l-tyrosine) PET/CT identified increased uptake along the parafalcine area of the frontoparietal lobes and the splenium portion of the corpus callosum. Read More

Front Aging Neurosci 2022 15;14:850743. Epub 2022 Mar 15.

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

Myelin oligodendrocyte glycoprotein-IgG-associated disorder (MOGAD) is a nervous system (NS) demyelination disease and a newly recognized distinct disease complicated with various diseases or symptoms; however, MOGAD was once considered a subset of neuromyelitis optica spectrum disorder (NMOSD). The detection of MOG-IgG has been greatly improved by the cell-based assay test method. In one study, 31% of NMOSD patients with negative aquaporin-4 (AQP-4) antibody were MOG-IgG positive. Read More

Ann Med Surg (Lond) 2022 May 28;77:103511. Epub 2022 Mar 28.

Firoozgar Clinical Research and Development Center FCRDC, Iran University of Medical Sciences IUMS, Tehran, Iran.

Introduction: and importance: Neurological ailments are reported during and after SARS-COV-2 infection.

Case Presentation: We report a 67-year-old Iranian man with COVID-19 infection and Acute Disseminated Encephalomyelitis (ADEM) whose neurological symptoms appeared before clinical and radiological pulmonary manifestations.

Clinical Discussion: COVID-19 can cause neurological complication without entering the CNS via para infectious inflammatory mechanisms. Read More

Ophthalmology 2022 Mar 29. Epub 2022 Mar 29.

Department of Ophthalmology, University of Arizona, Tucson, Arizona.

Purpose: Pediatric optic neuritis (ON) is a rare disease that has not been well characterized. The Pediatric ON Prospective Outcomes Study (PON1) was the first prospective study to our knowledge aiming to evaluate visual acuity (VA) outcomes, including VA, recurrence risk, and final diagnosis 2 years after enrollment.

Design: Nonrandomized observational study at 23 pediatric ophthalmology or neuro-ophthalmology clinics in the United States and Canada. Read More

Radiol Case Rep 2022 May 26;17(5):1789-1793. Epub 2022 Mar 26.

Medical Imaging Research center, Department of Radiology, Shiraz University of Medical Sciences, Shiraz, Iran.

Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system that is ordinarily monophasic. ADEM can develop following infection or vaccination. Here, we present a 37 y/o male patient with progressive muscle weakness in all limbs along with dysphagia following COVID-19 vaccination. Read More

J Neurol 2022 Jun 30;269(6):2827-2839. Epub 2022 Mar 30.

Centre de Référence National pour les Syndromes Neurologiques Paranéoplasique, Hôpital Neurologique, Hospices Civils de Lyon, Lyon, France.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the aetiologic agent of the coronavirus disease 2019 (COVID-19), is now rapidly disseminating throughout the world with 147,443,848 cases reported so far. Around 30-80% of cases (depending on COVID-19 severity) are reported to have neurological manifestations including anosmia, stroke, and encephalopathy. In addition, some patients have recognised autoimmune neurological disorders, including both central (limbic and brainstem encephalitis, acute disseminated encephalomyelitis [ADEM], and myelitis) and peripheral diseases (Guillain-Barré and Miller Fisher syndrome). Read More

Cureus 2022 Feb 23;14(2):e22551. Epub 2022 Feb 23.

Hospital Medicine, Marshfield Medical Center, Marshfield, USA.

Acute disseminated encephalomyelitis (ADEM) is a rare monophasic immune-mediated inflammatory disorder characterized by multifocal demyelinating lesions of the central nervous system. Clinically, it is distinguished by a variety of acute neurological deficits, including varying degrees of mental state changes and white matter abnormalities detected by magnetic resonance imaging (MRI). We present a challenging case of a young woman who developed ADEM as a result of chronic cannabis abuse. Read More

Neurol Sci 2022 Jun 25;43(6):4019-4022. Epub 2022 Mar 25.

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.