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Spectrum of demyelinating diseases of the central nervous system. ADEM, acute disseminated encephalomyelitis; AQP4, aquaporin-4; BS, brainstem syndrome; CSF, cerebrospinal fluid; IgG, immunoglobulin G; MOG, myelin oligodendrocyte glycoprotein; MRI, magnetic resonance imaging; OCB, oligoclonal bands; ON, optic neuritis; TM, transverse myelitis.
Source publication
In the past few years, acquired demyelinating syndromes of the central nervous system associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) have evolved into a new inflammatory disease entity distinct from neuromyelitis optica spectrum disorders or multiple sclerosis. The meticulous clinical description of patients with MOG I...
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Background
Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a novel inflammatory demyelinating disease of the central nervous system. This study aims to characterize the MRI features of MOGAD and contrast our results with the findings previously described in the literature and its close differential diagnoses.
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Citations
... In the past, it was believed that MOGAD is a monophasic disease. However, we now know that MOGAD can be relapsing in approximately 35% of the cases [30,73]. ...
... It has been shown that prolonged tapering is essential to prevent disease rebound activity, which mostly occurs in doses less than 20 mg/day or shortly after steroid cessation [9,76]. Risk factors for a relapse include initial presentation with transverse myelitis or encephalitis, high titer of MOG-antibodies, and incomplete recovery [73]. ...
Clinical syndromes associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) are now recognized as a distinct neurological disease entity, and are gaining increasing attention. The pathogenic mechanisms underlying MOG-antibody disease (MOGAD) remain incompletely understood. Case series, facilitated by registries, and observational studies over the past few years have shed increasing light on the clinical aspects and therapeutic approaches of MOGAD. MOGAD may manifest with a variety of clinical syndromes, including acute disseminated encephalomyelitis (ADEM), autoimmune encephalitis, optic neuritis (ON) and transverse myelitis (TM). MOGAD can be either monophasic or relapsing. This review aims to provide a comprehensive updated description of the clinical spectrum, paraclinical features, and prognosis of MOG-antibody disease, as well as summarize its therapeutic considerations. Randomized clinical trials, standardized diagnostic criteria and treatment guidelines are the steps forward.
... Diseases of myelin sheaths in the central nervous system (CNS) can be divided into two categories including genetic dysmyelinating diseases with abnormal myelin formation and acquired inflammatory demyelinating diseases, so called central nervous system inflammatory demyelinating diseases (CNS IDDs). CNS IDDs include multiple sclerosis (MS), neuromyelitis optica spectrum 2 disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), acute disseminated encephalomyelitis (ADEM), optic neuritis (ON) and transverse myelitis (TM) [1]. ...
Various vaccines have been developed in response to the SARS-CoV-2 pandemic, and the safety of vaccines has become an important issue. COVID-19 vaccines-related central nervous system inflammatory demyelinating diseases (CNS IDDs) have been reported recently. We present one case of AstraZeneca vaccine-related myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease and literature review another 78 patients published from January 2020 to October 2022. Patients were divided into three vaccine groups (viral vector, mRNA and inactivated vaccines) for further analyses. Among 79 patients with COVID-19 vaccines-related CNS IDDs, 49 (62%) cases received viral vector vaccines, 20 (25.3%) received mRNA vaccines and 10 (12.7%) received inactivated vaccines. Twenty-seven cases (34.2%) were confirmed with autoantibodies, including 15 patients (19%) with anti-MOG, 11 (13.9%) with anti-aquaporin 4 (AQP4), and one (1.3%) with both antibodies. Significantly, more males developed CNS IDDs post viral vector vaccines compared to mRNA and inactivated vaccines. Patients receiving mRNA vaccines were older than other groups. Furthermore, mRNA and inactivated vaccines correlated more with anti-AQP4 antibodies, while viral vector vaccines showed higher MOG positivity. The research suggests potential associations between COVID-19 vaccines-related CNS IDDs and gender, age, and autoantibodies, contingent on vaccine types. Protein sequence analysis implies similarities between the S protein and AQP4/MOG. Further studies may elucidate the mechanisms of CNS IDDs, aiding vaccine selection for specific groups.
... [7][8][9][10] Compared with multiple sclerosis and aquaporin-4 antibody-associated disease, MOG antibody-associated disease is an age-dependent demyelinating disease; adults have a lower prevalence than children (the positive frequency in adults is nearly half of that in children [22-23% vs 39-40%]), and many patients present with the ophthalmoneuromyelitis phenotype. [11][12][13] Recent evidence increasingly indicates that patients with CNS demyelinating events who are MOG antibody-positive present with isolated seizures. 14,15 However, there are gaps in the epidemiological knowledge regarding seizures in adults with this disease. ...
... 4 Relapses frequently occur during steroid weaning, especially in patients with low-dose steroids (prednisone < 20 mg/day adults), short-term therapy (<3 months), and high MOG antibody-positive titers. 11,12,16,[33][34][35] After acute attack treatment, treating patients with an additional immunosuppressor (eg, rituximab or https://doi.org/10.2147/NDT.S444853 ...
Background
Myelin oligodendrocyte glycoprotein (MOG) antibody–associated encephalitis is a new clinical phenotype of inflammatory demyelinating diseases. Some MOG antibody–positive patients with central nervous system demyelinating events present with isolated seizures. However, there are gaps in the epidemiological knowledge regarding seizures with MOG antibody–associated encephalitis in adults. This study characterized the clinical features and treatment of MOG antibody–positive patients with isolated seizures.
Methods
We reviewed all the patients admitted to Tianjin Huanhu Hospital between Jan. 1st 2017 and Jan. 1st 2022, to screen the MOG antibody–positive patients with isolated seizures, and collected the concerned patients’ information regarding epidemiology, clinical presentations, laboratory and radiological characteristics, electroencephalogram (EEG), treatments, and prognoses.
Results
We collected six MOG antibody–positive adult patients who had isolated symptomatic seizures. The mean age of the patients was 33 years (range, 29–40 years), and five (83.3%) were men. All patients presented with motor seizures, five (83.3%) had cognitive dysfunction, and only one (16.7%) had status epilepticus. Five (83.3%) patients had a good response to immunotherapy and antiseizure medications; only one had a sequela. The cerebrospinal fluid or serum anti-MOG antibody test turned negative over time.
Discussion
The most common seizure type in patients with MOG antibody–associated encephalitis with isolated seizures was focal to bilateral tonic-clonic seizures, and most patients had a good prognosis. Adding antiseizure medications were beneficial for MOG antibody–positive patients with seizures. Relapses and sequelae were associated with low-dose, short-time, or delayed therapy, and wide-range demyelinating brain damage.
... MOG is a highly conserved transmembrane protein expressed exclusively on the surface of oligodendrocytes in the CNS [39]. This protein maintains the integrity of the myelin sheath and participates in immune regulation [40]. ...
... The complex clinical and imaging manifestations include optic neuritis, encephalitis, brainstem encephalitis, meningoencephalitis, encephalomyelitis, myelitis, or their combination [43]. Although immunotherapy is effective for most patients, the recurrence rate is often high [39]. ...
... Tumors were not detected in the patients with MNOS, which decreased the probability of female predominance. Furthermore, the female-to-male ratio of patients with MOGAD was ~1:1 [39], which may also affect the sex ratio of patients with MNOS. However, it is imperative to conduct studies with larger cohorts to further evaluate the gender difference in MNOS. ...
The overlapping of two or more types of neural autoantibodies in one patient has increasingly been documented in recent years. The coexistence of myelin oligodendrocyte glycoprotein (MOG) and N-methyl-d-aspartate receptor (NMDAR) antibodies is most common, which leads to a unique condition known as the MOG antibody and NMDAR antibody overlapping syndrome (MNOS). Here, we have reviewed the pathogenesis, clinical manifestations, paraclinical features, and treatment of MNOS. Forty-nine patients with MNOS were included in this study. They were young males with a median onset age of 23 years. No tumors were observed in the patients, and 24 of them reported prodromal symptoms. The most common clinical presentations were psychiatric symptoms (35/49) and seizures (25/49). Abnormalities on magnetic resonance imaging involved the brainstem (11/49), cerebellum (9/49), and parietal lobe (9/49). Most patients mostly responded to immunotherapy and had a good long-term prognosis. However, the overall recurrence rate of MNOS was higher than that of monoantibody-positive diseases. The existence of concurrent NMDAR antibodies should be suspected in patients with MOG antibody-associated disease having psychiatric symptoms, seizures, movement disorders, or autonomic dysfunction. Similarly, serum MOG antibody testing should be performed when patients with anti-NMDAR encephalitis present with atypical clinical manifestations, such as visual impairment and limb weakness, and neuroradiological findings, such as optic nerve, spinal cord, or infratentorial involvement or meningeal enhancement). Early detection of the syndrome and prompt treatment can be beneficial for these patients, and maintenance immunosuppressive therapy is recommended due to the high overall recurrence rate of the syndrome.
... Given the significant differences in treatment and outcome, it is crucial to distinguish between MOGAD, NMOSD and MS. So far, there were quite a lot of studies (5)(6)(7)(8)(9) comparing MOGAD with MS and NMOSD in clinical features and imaging findings. In this paper, we conducted a retrospective study between MOGAD and MS, MOGAD and NMOSD from a clinical laboratory perspective. ...
Objectives
To analyze the differences in laboratory data between patients with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD).
Methods
The study included 26 MOGAD patients who visited Beijing Tiantan Hospital from 2018 to 2021. MS and NMOSD patients who visited the clinic during the same period were selected as controls. Relevant indicators were compared between the MOGAD group and the MS/NMOSD groups, and the diagnostic performance of meaningful markers was assessed.
Results
The MOGAD group showed a slight female preponderance of 57.7%, with an average onset age of 29.8 years. The absolute and relative counts of neutrophils were higher in the MOGAD group than in the MS group, while the proportion of lymphocytes was lower. The cerebrospinal fluid (CSF) IgG level, IgG index, 24-h IgG synthesis rate, and positive rate of oligoclonal bands (OCB) were lower in MOGAD patients than in the MS group. The area under ROC curve (AUC) was 0.939 when combining the relative lymphocyte count and IgG index. Compared to the NMOSD group, the MOGAD group had higher levels of serum complement C4 and lower levels of serum IgG. The AUC of serum C4 combined with FT4 was 0.783.
Conclusion
Statistically significant markers were observed in the laboratory data of MOGAD patients compared to MS/NMOSD patients. The relative lymphocyte count combined with IgG index had excellent diagnostic efficacy for MOGAD and MS, while serum C4 combined with FT4 had better diagnostic efficacy for MOGAD and NMOSD.
... 16,17 Some degree of permanent neurological disability has been reported in as many as 47% of adult MOGAD patients, 18 with up to 60% resulting from the onset attack. 19 While high-dose CS treatment is generally used as a first-line treatment for acute attacks, Jarius et al. reported complete or almost complete recovery in only 50% of 122 attacks treated with IVMP as a sole treatment, with the remaining having partial (44%) or no or almost no recovery (6%). When TPE or immunoadsorption (IA) was added as a second-line treatment, complete or almost complete recovery was observed in 40% of 25 attacks, partial recovery in 56%, and no or almost no recovery in 4%. ...
Background:
The potential therapeutic benefit of intravenous immunoglobulins (IVIGs) for acute attacks of myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is unknown.
Objective:
The objective was to describe the outcomes of IVIG treatment for acute MOGAD attacks.
Methods:
A retrospective observational study involving seven tertiary neuroimmunology centers. Data collection included patients' demographics, Expanded Disability Status Scale (EDSS), and visual acuity (VA) before the attack, at the nadir of the attack before IVIG treatment, and at follow-up visits ⩾3 months after treatment.
Results:
Thirty-nine patients were included, of which 21 (53.8%) were female. The median age was 23 years (range 5-74 years), and the median disease duration was 4 months (range 0-93 months). The most common type of attack treated with IVIG was isolated optic neuritis (ON) (unilateral n = 14, bilateral n = 5, associated with transverse myelitis (TM), n = 1), followed by acute disseminated encephalomyelitis (ADEM) (n = 8), multifocal (n = 7), TM (n = 3), brainstem (n = 1), and other encephalitis (n = 1). A significant improvement in both the EDSS and VA measures was observed at follow-up compared to the time of IVIG treatment initiation (p < 0.0001 for both outcome measures).
Conclusion:
IVIG may be an effective treatment option for acute MOGAD attacks. Further prospective studies are warranted to validate our results.
... 1 With the development of anti-MOG IgG antibody testing, positivity has been found in various nonspecific central nervous system diseases, and the clinical spectrum of MOGAD is expected to be broadened. 6,7 During myelitis attacks in MOGAD, MRI often demonstrates two sagittal extension patterns: 1) longitudinally extensive transverse myelitis (at least three vertebral segments) and/or 2) T2-weighted hyperintense short lesions. 8 Cervical spine MRI performed 6 years previously revealed increased signal intensity from the C4 to C7 levels without definite contrast enhancement in the sagittal and axial T2-weighted images (arrows) (E, F), and brain MRI revealed bilateral multifocal hyperintense lesions involving the bilateral thalamus (arrow) (H), left-side midbrain, and periaqueductal gray (arrow) (G) in the fluid-attenuated inversion-recovery images. ...
... The disease course can be relapsing (reported for 44-83% of patients) or monophasic, the latter subtype being more common in MOGAD than in other neurological demyelinating disorders, such as neuromyelitis optica spectrum disorder (NMOSD) or multiple sclerosis (MS) [3,5]. The clinical phenotypes of MOGAD are variable and include transverse myelitis (TM), optic neuritis (ON), acute disseminated encephalomyelitis (ADEM), NMOSD and cerebral cortical encephalitis, making diagnosis challenging [5][6][7][8][9][10][11][12][13]. ...
Introduction:
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare demyelinating disorder of the central nervous system. Despite increased recognition of MOGAD as a distinct disease and the availability of sensitive methods of MOG antibody testing, diagnostic challenges remain. We conducted a survey to explore the patient experience from the start of symptoms to final MOGAD diagnosis.
Methods:
A 23-question online survey (including multiple-choice and free-text responses) covering symptom history, healthcare interactions and impact of diagnosis was emailed to people living with MOGAD by The MOG Project patient advocacy group. People living with MOGAD could share the survey with their caregivers. Anonymised responses were analysed.
Results:
In total, 204 people living with MOGAD or their caregivers from 21 countries completed the survey; most respondents were from North America. Age of symptom onset ranged from 1 to 66 (median 28) years. Symptoms that prompted patients to seek medical care included blurred vision/loss of vision (58.2%), eye pain (35.8%) and difficulty walking (25.4%). Patients most frequently presented to emergency care physicians (38.7%) and primary care doctors (26.0%), with the MOGAD diagnosis most often made by general neurologists (40.4%) or neuro-immunologists (30.0%). Patients saw a median of four doctors before diagnosis, with 26.5% of patients seeing at least six doctors. Although 60.6% of patients received a MOGAD diagnosis within 6 months of experiencing initial health problems, 17.7% experienced a ≥ 5-year delay. More than half of patients (55.4%) received an alternative primary diagnosis before final MOGAD diagnosis. Most respondents (60.6%) reported receiving insufficient information/resources at the time of MOGAD diagnosis. Diagnostic delay was associated with long-term negative consequences for physical health.
Conclusion:
This survey provides unique insights from people living with MOGAD and their caregivers that could help address the challenges faced in the pathway to final MOGAD diagnosis.
... [13] The published MOGAD cohorts showed a relapsing course in 42-44% of patients. [15] Disability in MOGAD is accrued predominantly due to recurrent relapses. Hence, many clinicians in our center maintain patients on long-term immunosuppressive therapy. ...
Objectives
The aim of the study was to study the demographical, clinical, radiological features, and outcome of anti-myelin oligodendrocyte glycoprotein (MOG) antibody spectrum disorder and compare these features with patients negative for anti-MOG antibody. MOG antibody-associated disease (MOGAD) and aquaporin-4 (AQP4) antibody-related diseases are immunologically distinct pathologies. Our aim was to compare the clinical and radiological features of MOG antibody-related diseases with AQP4 antibody-related diseases and seronegative demyelinating diseases (Non-multiple sclerosis).
Materials and Methods
This was a prospective and cohort study conducted at an apex tertiary care institute in the northern part of India from Jan 2019 to May 2021. We compared clinical, laboratory, and radiological findings of patients with MOGAD, AQP4 antibody-related diseases, and seronegative demyelinating disease.
Results
There were a total of 103 patients – 41 patients of MOGAD, 37 patients of AQP4 antibody-related diseases and 25 seronegative demyelinating disease. Bilateral optic neuritis was the most frequent phenotype in patients with MOGAD (18/41) whereas myelitis was the most common phenotype in the AQP4 (30/37) and seronegative groups (13/25). Cortical, juxtacortical lesions, anterior segment optic neuritis, optic sheath enhancement, and conus involvement in myelitis were radiological findings that separated MOGAD from AQP4 related diseases. Nadir Expanded Disability Status Scale (EDSS) and visual acuity were similar across the groups. Last follow-up EDSS was significantly better in the MOG antibody group as compared to AQP4 antibody group (1 [0–8] vs. 3.5 [0–8]; P = 0.03). Encephalitis, myelitis, and seizures were more common in the younger population (<18 vs. >18 years) in MOGAD (9 vs. 2, P = 0.001; 9 vs. 7, P = 0.03; 6 vs. 0, P = 0.001).
Conclusion
We identified several clinical and radiological features that can help physicians to distinguish MOGAD from AQP4-immunoglobulin G+neuromyelitis optica spectrum disorder. Differentiation is vital as treatment response might vary among both groups.
... The clinical spectrum of acute disseminated encephalomyelitis (ADEM) is evolving particularly after the advent of myelin oligodendrocyte glycoprotein (MOG) IgG antibodies [1,2]. Herein, we describe three cases of ADEM to know the evolving spectrum of it. ...
... MOG-IgG positivity in association with monophasic and recurrent ADEM has been proven in multiple studies. Approximately 50% MOG-positive children present with ADEM compared to 10% adults [1]. Baumann et al. [6] compared MOG-positive and negative paediatric ADEM population and showed that MOG-positive patients had a better clinical outcome, despite more anatomical involvement. ...
Dear Sir, The clinical spectrum of acute disseminated encephalo-myelitis (ADEM) is evolving particularly after the advent of myelin oligodendrocyte glycoprotein (MOG) IgG antibodies [1, 2]. Herein, we describe three cases of ADEM to know the evolving spectrum of it. Case 1 A twenty-nine-year-old male presented with subacute onset weakness of all four limbs associated with urinary retention over 10 days and encephalopathy for the last 4 days. He had an acute febrile illness 4 weeks prior to this episode. Brain and spine MRI revealed a longitudinally extensive transverse myelitis (LETM) from C2 to C7 (Fig. 1a) and multiple T2/FLAIR hyperintensities in the bilateral sub-cortical, juxtacortical and cortical regions (Fig. 1b). CSF revealed lymphocytic pleocytosis (WBCs 191, lymphocytes 98%), with raised proteins (169 mg/dl) and normal sugar level. CSF workup for infectious causes was negative. ANA, ANCA and serum NMO antibody were negative. He was found positive for MOG-IgG antibody on cell-based assay. He was treated with IV MPS followed by 5 cycles of plasma exchange. He showed significant clinical improvement in sensorium and motor weakness. He had no relapses in the subsequent 2-year follow-up on azathioprine. Case 2 A twelve-year-old girl presented with recurrent vomiting, swaying to either side and encephalopathy for the last 10 days. She had a history of four episodes of optic neuritis in the last two and a half years and recovered completely in all episodes after receiving treatment. Brain MRI showed multiple fluffy T2/FLAIR hyperintensities in bilateral periventricular, subcortical and deep white matter of bilateral parietal lobes and temporal lobes, basifrontal lobes, right putamen, external capsule and cerebellar hemispheres (Fig. 1c and d). Focal areas of short segment T2 hyperintense lesion are noted in the spinal cord at the level of C4-C6, D2-D3 and D8-D10 segments. CSF analysis showed 20 cells all lymphocytes, protein 64 mg/dl and normal glucose. She tested positive for serum MOG-IgG antibody. She had complete symptom resolution after 5 days of IV MPS. Thereafter, she was started on azathioprine and continues to be asymptomatic one and half years into follow-up, without any further attacks. Case 3 A fifty-year-old male presented with a history of headache , vomiting and progressive worsening of sensorium over 2 weeks. The patient was irritable and drowsy, but had no focal deficits on examination. MRI brain showed confluent T2/FLAIR hyperintensities in bilateral subcor-tical fronto-parietal white matter and basal ganglia, pons and splenium of corpus callosum (Fig. 1e). A diagnosis of ADEM was made and he was treated with IV MPS with significant symptomatic improvement. However, 12 weeks later, he developed progressive vision loss in both eyes with visual acuity (VA) in both eyes limited to perception of light. Fundus examination revealed blurred disc margins
