scholarly journals MOG-IgG1 and co-existence of neuronal autoantibodies

2020 ◽  
pp. 135245852095104
Author(s):  
Amy Kunchok ◽  
Eoin P Flanagan ◽  
Karl N Krecke ◽  
John J Chen ◽  
J Alfredo Caceres ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Immunoglobulin G ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Control Cohort ◽  
Leptomeningeal Enhancement ◽  
Radiological Features ◽  
Neuronal Autoantibodies ◽  
Serum Testing

Background: The presence of co-existent neuronal antibodies (neuronal-IgG) in patients with myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG1) is not yet well understood. Objectives: The aim of this study was to investigate the co-existence of a broad range of neuronal-IgG in MOG-IgG1+ patients. Methods: MOG-IgG1+ patients were tested for 17 neuronal-IgGs in cerebrospinal fluid (CSF) and serum including NMDA-R-IgG, AMPA-R-IgG, GABAB-R-IgG, LGI1-IgG, CASPR2-IgG, GABAA-R-IgG, GAD65-IgG, mGLUR1-IgG, DPPX-IgG, CRMP5-IgG, amphiphysin-IgG, PCA1,2,Tr, and ANNA1,2,3. Clinical and radiological features of MOG-IgG1+ with NMDA-R-IgG in CSF were compared to a control cohort of MOG-IgG1+ patients without NMDA-R-IgG. Results: A total of 376 MOG-IgG1+ patients underwent testing for neuronal-IgGs. Serum testing for neuronal-IgGs (113 adults, 142 children) identified one child with NMDA-R-IgG (0.7%), one child with CASPR2-IgG (0.7%), one adult with LGI1-IgG (0.9%) and one adult with GABAA-R-IgG (0.9%). CSF testing for neuronal-IgGs (97 adults, 169 children) identified seven children (4%) and seven adults (7%) with NMDA-R-IgG, and one adult with GABAA-R-IgG (1%). The MOG-IgG1+/NMDA-R-IgG+ patients had a median age of 17 (range: 2–39) years. Features associated with MOG-IgG1+/NMDA-R-IgG+ included encephalopathy ( p = 0.001), seizures ( p = 0.045), and leptomeningeal enhancement ( p = 0.045). Conclusion: NMDA-R-IgG was the most frequently detected neuronal-IgG to co-exist with MOG-IgG1. MOG-IgG1+/NMDA-R-IgG+ patients most often presented with encephalopathy and seizures. Testing for MOG-IgG1 and NMDA-R-IgG may be warranted in patients with encephalopathy and inflammatory demyelinating syndromes.

Rate of Leptomeningeal Enhancement in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody–Associated Encephalomyelitis

2021 ◽  
Vol 36 (11) ◽  
pp. 1042-1046
Author(s):  
Judith A. Gadde ◽  
David S. Wolf ◽  
Stephanie Keller ◽  
Grace Y. Gombolay
Keyword(s):  
Cerebrospinal Fluid ◽  
Blood Cell ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Demyelinating Diseases ◽  
Blood Cell Count ◽  
Leptomeningeal Enhancement ◽  
Cortical Edema ◽  
Demyelinating Lesions ◽  
Mog Antibodies ◽  
Cerebrospinal Fluid Pleocytosis

Introduction: Myelin oligodendrocyte glycoprotein antibodies (MOG-abs) are associated with demyelinating diseases. Leptomeningeal enhancement occurs in 6% of adult MOG-abs patients but rates in pediatric MOG-abs patients are unknown. Methods: Retrospective review of pediatric MOG-abs patients was performed. Results: Twenty-one patients (7 boys, 14 girls) were included with an average age of 8.6 years (range 2-15 years). Seven of 21 (33%) pediatric MOG-abs patients had leptomeningeal enhancement. Two patients’ relapses were manifested by leptomeningeal enhancement alone and another patient presented with seizures, encephalopathy, and aseptic meningitis without demyelinating lesions. Cerebrospinal fluid pleocytosis was seen in both leptomeningeal (4/7 patients) and nonleptomeningeal enhancement (10/14 patients). Interestingly, 3 patients with leptomeningeal enhancement had normal cerebrospinal fluid white blood cell count. Cortical edema was more likely in patients with leptomeningeal enhancement ( P = .0263). Conclusion: We expand the clinical spectrum of anti-MOG antibody–associated disorder. Patients with recurrent leptomeningeal enhancement without demyelinating lesions should be tested for MOG antibodies.

Treatment of myelin oligodendrocyte glycoprotein immunoglobulin G–associated disease

2021 ◽  
Vol 12 (1) ◽  
pp. 22-41 ◽  
Author(s):  
Sarah Healy ◽  
Kariem Tarik Elhadd ◽  
Emily Gibbons ◽  
Dan Whittam ◽  
Michael Griffiths ◽  
...  
Keyword(s):  
Immunoglobulin G ◽  
Myelin Oligodendrocyte Glycoprotein

Serum Immunoglobulin G level and Neutrophils to Lymphocytes Ratio Associated with the Prognosis of Neuromyelitis Optica Spectrum Disorder

2021 ◽  
Vol 18 ◽  
Author(s):  
Ying Tong ◽  
Li Wang ◽  
Kai Liu ◽  
Weishi Liu ◽  
Shen Li ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Neuromyelitis Optica ◽  
Immunoglobulin G ◽  
Peripheral Blood ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Positive Group ◽  
Serum Igg ◽  
High Level ◽  
Igg Level

Objective: To investigate the factors related to the prognosis of neuromyelitis optica spectrum disorder (NMOSD) in cerebrospinal fluid and peripheral blood examination. Methods: In this study, we collected 111 patients who were admitted to the First Affiliated Hospital of Zhengzhou University between January 2016 and January 2018 and diagnosed with NMOSD. The patients were divided into the relapse group (n=48) and remission group (n=67). Before treatment, all the patients underwent a routine cerebrospinal fluid (CSF) and peripheral blood test on the second morning of admission. The association between laboratory data and disease prognosis was evaluated. Results: The immunoglobulin G (IgG) level in the serum showed a strong correlation with the relapse of patients, especially in the aquaporin-4-Antibody (AQP4-Ab) positive group (p<0.01). A high level of serum IgG concentration was associated with the relapse of NMOSD, especially in the anti-AQP4 positive group. The area under the receiver operating characteristic (ROC) curve of serum IgG level was 0.888 (p0.001, 95%CI: 0.808-0.968). The ratio of neutrophils to lymphocytes (NLR) was associated with the disability degree of NMOSD patients in 3 years. The NLR value was a linear correlation with final Expanded Disability Status Scale (EDSS) scores. Patients with a high level of NLR value presented an increased degree of disability in the following three years (R2=0.053, p=0.015). Conclusion: The serum IgG level and NLR of first-attack patients were correlated with the prognosis of NMOSD.

scholarly journals Cerebrospinal-fluid-derived Immunoglobulin G of Different Multiple Sclerosis Patients Shares Mutated Sequences in Complementarity Determining Regions

2013 ◽  
Vol 12 (12) ◽  
pp. 3924-3934 ◽  
Author(s):  
Vaibhav Singh ◽  
Marcel P. Stoop ◽  
Christoph Stingl ◽  
Ronald L. Luitwieler ◽  
Lennard J. Dekker ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Cerebrospinal Fluid ◽  
Immunoglobulin G ◽  
Complementarity Determining Regions ◽  
Multiple Sclerosis Patients

“Restlessness” After Cancer Diagnosis and Treatment

2021 ◽  
pp. 157-159
Author(s):  
Anastasia Zekeridou
Keyword(s):  
Cerebrospinal Fluid ◽  
Cancer Diagnosis ◽  
Immunoglobulin G ◽  
Immune Checkpoint ◽  
Immune Checkpoint Inhibitor ◽  
Checkpoint Inhibitor ◽  
Hyperkinetic Movement Disorder ◽  
Phosphodiesterase 10A ◽  
Cancer Remission ◽  
Inhibitor Treatment

A 76-year-old woman sought care for unintentional weight loss, hematuria, and fatigue. She was diagnosed with plurimetastatic renal cell carcinoma. After resection of the primary tumor and metastases, she was treated with pembrolizumab, an immune checkpoint inhibitor. The patient experienced involuntary tongue and face movements with dysphagia and weight loss. She was also described as “restless.” At that point, the patient was in cancer remission with ongoing immune checkpoint inhibitor treatment. Blood testing was unremarkable. Brain magnetic resonance imaging showed basal ganglia T2/fluid-attenuated inversion recovery hyperintensities without gadolinium enhancement. Cerebrospinal fluid testing showed slightly increased protein concentration and 8 cerebrospinal fluid-restricted oligoclonal bands. Serum and cerebrospinal fluid testing for neural autoantibodies showed immunoglobulin G immunoreactivity in a mouse tissue indirect immunofluorescence assay, predominantly staining the basal ganglia. The immunoglobulin G was subsequently identified to bind to phosphodiesterase 10A. The patient was diagnosed with paraneoplastic phosphodiesterase 10A-immunoglobulin G autoimmunity manifesting as hyperkinetic movement disorder triggered by immune checkpoint inhibitor treatment. Given the patient’s cancer remission, the immune checkpoint inhibitor treatment was discontinued. She was treated with high-dose intravenous corticosteroids, with improvement of her hyperkinetic movement disorder but persistence of some dystonic movements. Further treatment with oral prednisone did not produce further improvement. The patient was treated symptomatically with onabotulinumtoxinA injections and tetrabenazine, which ameliorated her dystonic movements. Three years after her cancer diagnosis, she was alive and in cancer remission with minimal residual movements. Immune checkpoint inhibitors are monoclonal antibodies targeting “stop signs” of the immune response, which lead to enhanced endogenous responses, including those against cancer. Autoimmune complications are consequences of the enhanced immunity and can affect all organs, including the nervous system.

A Woman With Subacute Painful Vision Loss

2021 ◽  
pp. 3-6
Author(s):  
Jiraporn Jitprapaikulsan ◽  
M. Tariq Bhatti ◽  
Eric R. Eggenberger ◽  
Marie D. Acierno ◽  
John J. Chen
Keyword(s):  
Optic Neuritis ◽  
Eye Movement ◽  
Immunoglobulin G ◽  
Vision Loss ◽  
Visual Fields ◽  
Corticosteroid Treatment ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Intravenous Methylprednisolone ◽  
Eye Pain

A 51-year-old White woman sought care for vision loss 1 week after a nonspecific upper respiratory tract infection. She reported pain in both eyes exacerbated by eye movement, which lasted for several days, followed by bilateral vision loss to the level of counting fingers–only vision. Optic neuritis was diagnosed, and she was treated with 1 g intravenous methylprednisolone for 3 days. Her vision improved substantially, and the pain resolved during the corticosteroid treatment. However, 1 week later, she woke up with right eye pain and vision loss. She was again treated with 5 days of intravenous methylprednisolone, with visual improvement nearly back to baseline. Two weeks later, she had recurrence of painful vision loss in both eyes. A diagnosis of chronic relapsing inflammatory optic neuropathy was made. Tests for serum angiotensin-converting enzyme, antineutrophil cytoplasmic antibody, antinuclear antibody, Lyme disease, syphilis, tuberculosis, and aquaporin-4-immunoglobulin G antibodies were negative. Serum was definitively positive for myelin oligodendrocyte glycoprotein-immunoglobulin G antibodies at a titer of 1:1,000. Myelin oligodendrocyte glycoprotein-immunoglobulin G–associated recurrent optic neuritis was diagnosed. After her diagnosis of recurrent corticosteroid-dependent optic neuritis associated with myelin oligodendrocyte glycoprotein-immunoglobulin G positivity, the patient was treated with 5 days of intravenous methylprednisolone. The eye pain resolved, and her vision returned to normal. At follow-up evaluation, the patient’s visual acuity, color vision, and visual fields were normal in both eyes, but there was mild bilateral optic disc pallor. She has not had recurrent demyelinating episodes while on chronic immunotherapy. Optic neuritis is an inflammatory demyelination of the optic nerve manifesting as acute to subacute vision loss, classically associated with pain with eye movement. The long-term prevention and prognosis depend on the cause of the optic neuritis.

High performance of cerebrospinal fluid immunoglobulin G analysis for diagnosis of multiple sclerosis

2019 ◽  
Vol 266 (4) ◽  
pp. 902-909
Author(s):  
Simon Gamraoui ◽  
Guillaume Mathey ◽  
Marc Debouverie ◽  
Catherine Malaplate ◽  
René Anxionnat ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Cerebrospinal Fluid ◽  
Immunoglobulin G ◽  
High Performance
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