Recurrent ataxia and dysarthria in myelin oligodendrocyte glycoprotein antibody-associated disorder

2021 ◽  
Vol 14 (11) ◽  
pp. e245341
Author(s):  
Uddalak Chakraborty ◽  
Shrestha Ghosh ◽  
Amlan Kusum Datta ◽  
Atanu Chandra
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Male Patient ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Aquaporin 4 ◽  
Clinical Presentations ◽  
Neurological Presentation ◽  
New Biomarkers ◽  
Demyelinating Disorders

The spectrum of central nervous system demyelinating disorders is vast and heterogeneous and, often, with overlapping clinical presentations. Misdiagnosis might occur in some cases with serious therapeutic repercussions. However, introduction of several new biomarkers such as aquaporin-4 IgG and myelin oligodendrocyte glycoprotein IgG has made distinction between diseases such as multiple sclerosis and myelin oligodendrocyte glycoprotein antibody-associated disorder easier. Here, we report a case of a 15-year-old male patient with subacute multifocal neurological presentation without encephalopathy, eventually diagnosed as myelin oligodendrocyte glycoprotein antibody-associated disorder.

scholarly journals No association of AQP4 polymorphisms with neuromyelitis optica and multiple sclerosis

2016 ◽  
Vol 7 (1) ◽  
Author(s):  
Ting-Ting Yang ◽  
Yang He ◽  
Ya-Juan Xiang ◽  
Dong-Hui Ao ◽  
Yang-Yang Wang ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Environmental Factors ◽  
Neuromyelitis Optica ◽  
Water Channel ◽  
Aquaporin 4 ◽  
The Central Nervous System ◽  
Genetic And Environmental Factors ◽  
Demyelinating Disorders

AbstractMultiple sclerosis (MS) and neuromyelitis optica (NMO) are inflammatory demyelinating disorders of the central nervous system (CNS). Various genetic and environmental factors have been identified to contribute to etiology of MS and NMO. Aquaporin 4 (AQP4), is the most abundant water channel in CNS.

Demyelinating disorders of the central nervous system

2010 ◽  
pp. 4948-4963
Author(s):  
Siddharthan Chandran ◽  
Alastair Compston
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
System P ◽  
The Central Nervous System ◽  
Demyelinating Disorders ◽  
Brain And Spinal Cord

Clinicians suspect demyelination when episodes reflecting damage to white matter tracts within the central nervous system occur in young adults. The paucity of specific biological markers of discrete demyelinating syndromes places an emphasis on clinical phenotype—temporal and spatial patterns—when classifying demyelinating disorders. The diagnosis of multiple sclerosis, the most common demyelinating disorder, becomes probable when these symptoms and signs recur, involving different parts of the brain and spinal cord. Other important demyelinating diseases include post-infectious neurological disorders (acute disseminated encephalomyelitis), demyelination resulting from metabolic derangements (central pontine myelinosis), and inherited leucodystrophies that may present in children or in adults. Accepting differences in mechanism, presentation, and treatment, two observations can usefully be made when classifying demyelinating disorders. These are the presence or absence of inflammation, and the extent of focal vs. diffuse demyelination. Multiple sclerosis is prototypic for the former, whereas dysmyelinating disorders, such as leucodystrophies are representative of the latter....

International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions

2013 ◽  
Vol 19 (10) ◽  
pp. 1261-1267 ◽  
Author(s):  
Lauren B Krupp ◽  
Marc Tardieu ◽  
Maria Pia Amato ◽  
Brenda Banwell ◽  
Tanuja Chitnis ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Study Group ◽  
Pediatric Multiple Sclerosis ◽  
Immune Mediated ◽  
International Pediatric ◽  
Demyelinating Disorders ◽  
Multiple Sclerosis Study Group

Background: There has been tremendous growth in research in pediatric multiple sclerosis (MS) and immune mediated central nervous system demyelinating disorders since operational definitions for these conditions were first proposed in 2007. Further, the International Pediatric Multiple Sclerosis Study Group (IPMSSG), which proposed the criteria, has expanded substantially in membership and in its international scope. Objective: The purpose of this review is to revise the 2007 definitions in order to incorporate advances in delineating the clinical and neuroradiologic features of these disorders. Methods: Through a consensus process, in which input was sought from the 150 members of the Study Group, criteria were drafted, revised and finalized. Final approval was sought through a web survey. Results: Revised criteria are proposed for pediatric acute disseminated encephalomyelitis, pediatric clinically isolated syndrome, pediatric neuromyelitis optica and pediatric MS. These criteria were approved by 93% or more of the 56 Study Group members who responded to the final survey. Conclusions: These definitions are proposed for clinical and research purposes. Their utility will depend on the outcomes of their application in prospective research.

Brainstem and cerebellar involvement in MOG-IgG-associated disorder versus aquaporin-4-IgG and MS

2020 ◽  
pp. jnnp-2020-325121
Author(s):  
Samantha A Banks ◽  
Padraig P Morris ◽  
John J Chen ◽  
Sean J Pittock ◽  
Elia Sechi ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Aquaporin 4 ◽  
Oligoclonal Bands ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Middle Cerebellar Peduncle ◽  
Cerebellar Involvement ◽  
Cerebellar Peduncle ◽  
Csf Oligoclonal Bands

ObjectiveTo determine the frequency and characteristics of brainstem or cerebellar involvement in myelin-oligodendrocyte-glycoprotein-antibody-associated-disorder (MOGAD) versus aquaporin-4-IgG-seropositive-neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS).MethodsIn this observational study, we retrospectively identified 185 Mayo Clinic MOGAD patients with: (1) characteristic MOGAD phenotype, (2) MOG-IgG seropositivity by live cell-based assay and (3) MRI lesion(s) of brainstem, cerebellum or both. We compared the symptomatic attacks to AQP4-IgG-NMOSD (n=30) and MS (n=30).ResultsBrainstem or cerebellar involvement occurred in 62/185 (34%) MOGAD patients of which 39/62 (63%) were symptomatic. Ataxia (45%) and diplopia (26%) were common manifestations. The median age in years (range) in MOGAD of 24 (2–65) was younger than MS at 36 (16–65; p=0.046) and AQP4-IgG-NMOSD at 45 (6–72; p=0.006). Isolated attacks involving the brainstem, cerebellum or both were less frequent in MOGAD (9/39 (23%)) than MS (22/30 (73%); p<0.001) but not significantly different from AQP4-IgG-NMOSD (14/30 (47%); p=0.07). Diffuse middle cerebellar peduncle MRI-lesions favoured MOGAD (17/37 (46%)) over MS (3/30 (10%); p=0.001) and AQP4-IgG-NMOSD (3/30 (10%); p=0.001). Diffuse medulla, pons or midbrain MRI lesions occasionally occurred in MOGAD and AQP4-IgG-NMOSD but never in MS. Cerebrospinal fluid (CSF) oligoclonal bands were rare in MOGAD (5/30 (17%)) and AQP4-IgG-NMOSD (2/22 (9%); p=0.68) but common in MS (18/22 (82%); p<0.001). Disability at nadir or recovery did not differ between the groups.ConclusionInvolvement of the brainstem, cerebellum or both is common in MOGAD but usually occurs as a component of a multifocal central nervous system attack rather than in isolation. We identified clinical, CSF and MRI attributes that can help discriminate MOGAD from AQP4-IgG-NMOSD and MS.

scholarly journals Inflammatory Demyelinating Central Nervous System Diseases in Childhood: Clinical and Paraclinical Profiles in 133 Patients

2012 ◽  
Vol 2012 ◽  
pp. 1-6
Author(s):  
Derya Kaya ◽  
Egemen İdiman ◽  
Serkan Özakbaş
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Disease Onset ◽  
Progressive Multiple Sclerosis ◽  
Western Turkey ◽  
Mri Features ◽  
Cranial Mri ◽  
Multiple Sclerosis Patients ◽  
Demyelinating Disorders

In a retrospective review of patients with acquired demyelinating disorders of the central nervous system, 133 patients (5.6%) whose diseases started in childhood, were selected from 2369 patients, who had medical records in the Neurology Department of Dokuz Eylul University. Out of 133, 98 had relapsing remitting multiple sclerosis, 21 had secondary progressive multiple sclerosis, 8 had clinically isolated syndrome, 3 had neuromyelitis optica, 2 had Marburg disease, and 1 had radiologically isolated syndrome. In 55 patients (41.3%), disease onset was before age 16. Polysymptomatic presentation (22.6%) was the most common initial feature. The EDSS scores ranged from 0 to 9 with a median of 2.0 () for 126 patients. MRI records of 111 patients were obtained. 97 patients had clinically definite multiple sclerosis. 11 MS patients (11.3%) did not initially present the diagnostic MRI features. All of the remaining multiple sclerosis patients fulfilled Barkhof-Tintore criteria (100%) and 88.7% fulfilled KIDMUS criteria. Cranial MRI of NMO patients was normal. Our findings demonstrate some important clinical and paraclinical features that can help the literature on acquired demyelinating disorders of childhood by utilizing data from Western Turkey.

Thyroid antibodies in aquaporin-4 antibody positive central nervous system autoimmunity and multiple sclerosis

2011 ◽  
Vol 75 (2) ◽  
pp. 271-272 ◽  
Author(s):  
Johann Sellner ◽  
Sudhakar R. Kalluri ◽  
Sabine Cepok ◽  
Bernhard Hemmer ◽  
Achim Berthele
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Aquaporin 4 ◽  
Thyroid Antibodies ◽  
Aquaporin 4 Antibody

The clinical spectrum associated with myelin oligodendrocyte glycoprotein antibodies (anti-MOG-Ab) in Thai patients

2015 ◽  
Vol 22 (7) ◽  
pp. 964-968 ◽  
Author(s):  
Sasitorn Siritho ◽  
Douglas K Sato ◽  
Kimihiko Kaneko ◽  
Kazuo Fujihara ◽  
Naraporn Prayoonwiwat
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Visual Recovery ◽  
Central Nervous System Disorders ◽  
Spectrum Disorders

Background: Myelin oligodendrocyte glycoprotein (anti-MOG) antibody was reported in anti-aquaporin-4 (anti-AQP4) seronegative neuromyelitis optica spectrum disorders (NMOSD) patients. Objectives: To describe clinical phenotypes associated with anti-MOG. Methods: Seventy consecutive Thai patients with inflammatory idiopathic demyelinating central nervous system disorders (IIDCD) who were previously anti-AQP4 seronegative were tested for anti-MOG. Results: Anti-MOG was positive in six patients, representing 20.7% of the IIDCD anti-AQP4 seronegative patients with a non-multiple sclerosis phenotype, and most had relapses. All first presented with optic neuritis with good visual recovery after treatment. Conclusions: Anti-MOG positive patients may have manifestations that mimic NMOSD but differ in their course and prognosis from anti-AQP4 positive NMOSD.

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