Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe four patients who experienced optic neuritis (ON) and seizures and were found to have antibodies to myelin oligodendrocyte glycoprotein (MOG) in serum. The index case was a previously healthy 39-year-old man who developed steroid dependent ON and had a generalized seizure when steroids were tapered. He tested positive for antibodies to MOG. We have reviewed the charts of all 11 anti-MOG antibody positive patients in our practice and found that 4 patients, all of whom had experienced one or more episodes of ON, also had a generalized seizure during the course of their illness. In 2 patients - including the index case - seizure occurred during steroid taper and in 2 others at the time of an episode of acute disseminated encephalomyelitis (ADEM). Association of anti-MOG antibodies and relapsing demyelinating disorders of the central nervous system is increasingly recognized. Testing for anti-MOG antibodies should be considered in patients with optic neuritis and seizures, especially in those with who also have a history of ADEM.
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PMID:Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in patients with optic neuritis and seizures. 2957 58

We report the case of a patient who presented with progressive unsteadiness and narcoleptic attacks followed by behavioral change and psychosis, without visual disturbances or seizures. MRI revealed multiple areas of fluid attenuation inversion recovery (FLAIR) high-intensity lesions involving the cerebellum, brainstem, thalamus and third ventricular peri-ependymal region consistent with demyelination. Both the serum myelin oligodendrocyte glycoprotein-antibodies (MOG-Abs) and cerebral spinal fluid (CSF) anti-N-methyl-d-as-partate receptor (NMDAR) antibodies were positive using transfected cell based assays. The patient presented simultaneously with symptoms of MOG antibody disease and anti-NMDAR encephalitis, an unusual clinical scenario, indicating the co-existence of the two disorders.
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PMID:An unusual case of anti-MOG CNS demyelination with concomitant mild anti-NMDAR encephalitis. 2966 38

A 27-year-old man developed acute encephalitis with headache, fever, seizures, and aphasia. Analysis of cerebrospinal fluid showed elevated levels of cell counts and protein. A brain MRI demonstrated increased FLAIR signals in the left cerebral cortex with cortical swelling. An MRA also showed mild vasodilatation of the left middle cerebral artery branches. After admission, severe psychomotor excitement developed. Immunotherapy with intravenous high-dose steroid and subsequent oral steroid was successful, and the patient returned to premorbid working position. Repeated MRI study showed complete resolution. Serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was positive, while anti-aquaporin-4 antibody, anti-N-methyl-D-aspartate (NMDA) receptor antibody, and other autoimmune antibodies were all negative. There were no relapses at final follow-up of 8 months after onset. Cerebral cortical encephalitis with unknown etiology can occur associated with anti-MOG antibody, and anti-MOG antibody may play certain role in the pathogenesis.
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PMID:[Cerebral cortical encephalitis with anti-myelin oligodendrocyte glycoprotein (MOG) antibody]. 3048 64

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) were first detected by immunoblot and enzyme-linked immunosorbent assay nearly 30 years ago, but their association with multiple sclerosis (MS) was not specific. Use of cell-based assays with native MOG as the substrate enabled identification of a group of MOG-Ab-positive patients with demyelinating phenotypes. Initially, MOG-Abs were reported in children with acute disseminated encephalomyelitis (ADEM). Further studies identified MOG-Abs in adults and children with ADEM, seizures, encephalitis, anti-aquaporin-4-antibody (AQP4-Ab)-seronegative neuromyelitis optica spectrum disorder (NMOSD) and related syndromes (optic neuritis, myelitis and brainstem encephalitis), but rarely in MS. This shift in our understanding of the diagnostic assays has re-invigorated the examination of MOG-Abs and their role in autoimmune and demyelinating disorders of the CNS. The clinical phenotypes, disease courses and responses to treatment that are associated with MOG-Abs are currently being defined. MOG-Ab-associated disease is different to AQP4-Ab-positive NMOSD and MS. This Review provides an overview of the current knowledge of MOG, the metrics of MOG-Ab assays and the clinical associations identified. We collate the data on antibody pathogenicity and the mechanisms that are thought to underlie this. We also highlight differences between MOG-Ab-associated disease, NMOSD and MS, and describe our current understanding on how best to treat MOG-Ab-associated disease.
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PMID:Myelin oligodendrocyte glycoprotein antibodies in neurological disease. 3055 66

Myelin oligodendrocyte glycoprotein is expressed in the central nervous system on the surface of oligodendrocytes and is associated with a broad range of adult and pediatric demyelinating phenotypes. The entire spectrum of clinical and radiologic features of myelin oligodendrocyte glycoprotein antibody spectrum disorder remains to be fully elucidated. We describe the case of a 9-year-old boy with immune-mediated myelitis undetectable by conventional magnetic resonance imaging in the context of relapsing anti-myelin oligodendrocyte glycoprotein spectrum disorder. Despite the severe clinical presentation, his symptoms improved significantly following treatment with corticosteroids. Because timely diagnosis and treatment is imperative to prevent disease recurrence and reduce long-term morbidity, serum anti-myelin oligodendrocyte glycoprotein antibody testing should be considered in all children with acute demyelinating syndromes and unusual clinical presentations-including seizures-both at presentation and at follow-up.
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PMID:MRI-Negative Myelitis Associated With Myelin Oligodendrocyte Glycoprotein Antibody Spectrum Demyelinating Disease. 3080 Jul

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, the most recognized type of autoimmune encephalitis, manifests with rapid cognitive decline, psychosis, and seizures that develop in 78-86% of patients. Recently, anti-NMDAR encephalitis was reported in association with demyelinating diseases which are accompanied by a characteristic clinical phenotype, imaging abnormalities, and the presence of antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) in bodily fluids. The patient presented herein suffered from bilateral optic neuritis followed by recurrent encephalitis with focal seizures and demonstrated anti-NMDAR and MOG-IgGs in the cerebrospinal fluid and serum, respectively. Her symptoms responded to immunotherapy and antiseizure medication. The recognition of the novel syndrome of MOG antibody-associated demyelination (MOGAD), encompassing the overlapping anti-NMDAR encephalitis and other MOG-IgG associated disorders, is important for the successful management of these patients.
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PMID:Overlapping demyelinating syndrome and anti-N-methyl-d-aspartate receptor encephalitis with seizures. 3173 64

The recent development of a cell-based assay that can detect specific autoantibodies revealed the clinical features of diseases associated with the anti-myelin oligodendrocyte glycoprotein (MOG) antibody. The anti-MOG antibody associated diseases may include inflammatory demyelinating central nervous system diseases such as neuromyelitis optica spectrum disorders, optic neuritis, myelitis, atypical multiple sclerosis, and encephalitis. Among them, anti-MOG antibody associated cortical encephalitis may develop seizure as one of the primary symptoms, present unique lateral or bilateral medial frontal cortical lesions on brain MRI FLAIR images. In acute phase, steroid pulse therapy and anti-epileptic drugs are required. In chronic phase, immunosuppressive drugs are often required to prevent relapses.
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PMID:[Anti-myelin oligodendrocyte glycoprotein antibody associated encephalitis]. 3195 98

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been associated with steroid-responsive cortical encephalitis and comorbid generalized epilepsy. A 44-year-old woman developed repeated epilepsia partialis continua (EPC) without generalized seizures and was anti-MOG antibody-positive. Radiological abnormalities were detected in the bilateral medial frontoparietal cortices, but there were no cerebrospinal fluid abnormalities. She achieved remission with anti-epileptic drugs alone. However, encephalitis recurred four months later when pleocytosis appeared, and steroid therapy was effective. Altogether, EPC without typical cerebrospinal fluid features can be an early sign of anti-MOG antibody-positive encephalitis. Thus, patients with EPC of unknown etiology need to be screened for anti-MOG antibodies.
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PMID:Epilepsia Partialis Continua as an Early Sign of Anti-Myelin Oligodendrocyte Glycoprotein Antibody-positive Encephalitis. 3213 31

A 22-year-old woman acutely developed recurrent convulsive seizures followed by fever and headache. Cerebrospinal fluid study showed leukocytosis without hypoglycorrhachia. These clinical features suggested acute viral or aseptic encephalitis. The patient was treated only with an antiviral agent and improved immediately with good prognosis. Afterwards, the characteristic brain MRI findings required us to check the patient's serum, and the final diagnosis of myelin oligodendrocyte glycoprotein (MOG) antibody-positive cerebral cortical encephalitis (CCE) was confirmed. Most previously reported cases with MOG antibody-positive CCE clinically showed fever and/or headache, and some were initially misdiagnosed of having central nervous system infection. All previously reported cases were treated with immunotherapy. However, our case showed the very benign clinical course and improved rapidly without any immunotherapy. We should be reminded that MOG-antibody-positive CCE could be self-remitting and mimic acute viral or aseptic encephalitis. In addition, the characteristic neuroradiological findings could be an important clue to the correct diagnosis of CCE.
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PMID:Self-remitting cerebral cortical encephalitis associated with myelin oligodendrocyte glycoprotein antibody mimicking acute viral encephalitis: A case report. 3214 29

Unilateral cerebral cortical encephalitis (UCCE) with myelin oligodendrocyte glycoprotein (MOG)-antibody comprises a new spectrum of disease entities generally presenting seizures. Here, we report a case of a young adult with anti-MOG antibody-associated UCCE who only presented persistent left pulsatile headache. Neurological examination revealed no deficits. Brain MRI showed a fluid-attenuated inversion recovery hyperintense lesion along the swollen left cerebral cortex. The patient was positive for anti-MOG antibodies. We diagnosed him with anti-MOG antibody-associated UCCE. Immediately after the administration of high-dose IV methylprednisolone, the headache diminished. Anti-MOG antibody-associated UCCE is a new differential diagnosis in patients with unilateral chronic pulsatile headache.
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PMID:Unilateral chronic pulsatile headache as the single manifestation of anti-MOG antibody-associated unilateral cerebral cortical encephalitis. 3268 39


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