Author Of 1 Presentation
HT06.02 - Presentation 02
Abstract
Abstract
Since the discovery of antibodies against aquaporin-4 (AQP4-IgG) in patients with Neuromyelitis Optica Spectrum Disorders (NMOSD), an increasing number of AQP4-IgG+ NMOSD patients were described having limited forms such as longitudinally extensive myelitis or recurrent and/or bilateral optic neuritis, area postrema attacks with persistent (> 48 hours) hiccups, nausea and vomiting and those patients with associated diencephalic, brainstem and cerebral lesions “typical” for NMOSD. According to the international consensus diagnostic criteria for NMOSD published on 2015, the diagnosis of NMOSD for AQP4-IgG positive cases is possible if there is a suggestive attack with involvement 1 of 6 core locations (optic nerve, spinal cord, area postrema of the dorsal medulla, brainstem, diencephalon or cerebrum). In seronegative patients, two or more core locations must be affected, with at least one of the attacks in the optic nerve, spinal cord or the area postrema, and additional magnetic resonance imaging (MRI) criteria should be fulfilled. The 2015 NMOSD diagnostic criteria recommends cell-based assays (CBA) to detected AQP4-IgG. However, the availability of diagnostic tests such as AQP4-IgG by CBA is still limited in low income countries, as well as long-term treatments recently approved for NMOSD. More recently, antibodies against the myelin oligodendrocyte glycoprotein (MOG-IgG) have been incorporated in the clinical practice in many developed countries as an important tool to differentiate patients with MOG-IgG associated Optic Neuritis, Encephalitis, and/or Myelitis (MONEM) from seronegative NMOSD and other demyelinating CNS disorders. Nevertheless, the availability of MOG-IgG CBAs is even more restricted than AQP4-IgG, as it is limited to research laboratories in many countries. The management of these patients require an international effort to support patients from countries without access to antibody testing for AQP4-IgG and MOG-IgG, as well as provide drugs to reduce the risk of further attacks and disability.
Presenter Of 1 Presentation
HT06.02 - Presentation 02
Abstract
Abstract
Since the discovery of antibodies against aquaporin-4 (AQP4-IgG) in patients with Neuromyelitis Optica Spectrum Disorders (NMOSD), an increasing number of AQP4-IgG+ NMOSD patients were described having limited forms such as longitudinally extensive myelitis or recurrent and/or bilateral optic neuritis, area postrema attacks with persistent (> 48 hours) hiccups, nausea and vomiting and those patients with associated diencephalic, brainstem and cerebral lesions “typical” for NMOSD. According to the international consensus diagnostic criteria for NMOSD published on 2015, the diagnosis of NMOSD for AQP4-IgG positive cases is possible if there is a suggestive attack with involvement 1 of 6 core locations (optic nerve, spinal cord, area postrema of the dorsal medulla, brainstem, diencephalon or cerebrum). In seronegative patients, two or more core locations must be affected, with at least one of the attacks in the optic nerve, spinal cord or the area postrema, and additional magnetic resonance imaging (MRI) criteria should be fulfilled. The 2015 NMOSD diagnostic criteria recommends cell-based assays (CBA) to detected AQP4-IgG. However, the availability of diagnostic tests such as AQP4-IgG by CBA is still limited in low income countries, as well as long-term treatments recently approved for NMOSD. More recently, antibodies against the myelin oligodendrocyte glycoprotein (MOG-IgG) have been incorporated in the clinical practice in many developed countries as an important tool to differentiate patients with MOG-IgG associated Optic Neuritis, Encephalitis, and/or Myelitis (MONEM) from seronegative NMOSD and other demyelinating CNS disorders. Nevertheless, the availability of MOG-IgG CBAs is even more restricted than AQP4-IgG, as it is limited to research laboratories in many countries. The management of these patients require an international effort to support patients from countries without access to antibody testing for AQP4-IgG and MOG-IgG, as well as provide drugs to reduce the risk of further attacks and disability.
Invited Speaker Of 1 Presentation
HT06.02 - Presentation 02
Abstract
Abstract
Since the discovery of antibodies against aquaporin-4 (AQP4-IgG) in patients with Neuromyelitis Optica Spectrum Disorders (NMOSD), an increasing number of AQP4-IgG+ NMOSD patients were described having limited forms such as longitudinally extensive myelitis or recurrent and/or bilateral optic neuritis, area postrema attacks with persistent (> 48 hours) hiccups, nausea and vomiting and those patients with associated diencephalic, brainstem and cerebral lesions “typical” for NMOSD. According to the international consensus diagnostic criteria for NMOSD published on 2015, the diagnosis of NMOSD for AQP4-IgG positive cases is possible if there is a suggestive attack with involvement 1 of 6 core locations (optic nerve, spinal cord, area postrema of the dorsal medulla, brainstem, diencephalon or cerebrum). In seronegative patients, two or more core locations must be affected, with at least one of the attacks in the optic nerve, spinal cord or the area postrema, and additional magnetic resonance imaging (MRI) criteria should be fulfilled. The 2015 NMOSD diagnostic criteria recommends cell-based assays (CBA) to detected AQP4-IgG. However, the availability of diagnostic tests such as AQP4-IgG by CBA is still limited in low income countries, as well as long-term treatments recently approved for NMOSD. More recently, antibodies against the myelin oligodendrocyte glycoprotein (MOG-IgG) have been incorporated in the clinical practice in many developed countries as an important tool to differentiate patients with MOG-IgG associated Optic Neuritis, Encephalitis, and/or Myelitis (MONEM) from seronegative NMOSD and other demyelinating CNS disorders. Nevertheless, the availability of MOG-IgG CBAs is even more restricted than AQP4-IgG, as it is limited to research laboratories in many countries. The management of these patients require an international effort to support patients from countries without access to antibody testing for AQP4-IgG and MOG-IgG, as well as provide drugs to reduce the risk of further attacks and disability.
Author Of 1 Presentation
TC06.03 - Presentation 03 (ID 606)
Abstract
Abstract
The myelin oligodendrocyte glycoprotein (MOG) is a protein expressed in the outermost surface of the myelin sheath of the central nervous system. Its localization makes it a target for autoantibodies. Over the last few years, serum autoantibodies recognizing MOG (MOG-IgG) have been associated to optic neuritis, myelitis, acute disseminated encephalomyelitis, cortical encephalitis, and some cases of aquaporin-4 (AQP4-IgG) negative neuromyelitis optica spectrum disorder (NMOSD). Pediatric cases represent a significant proportion of MOG-IgG+ patients; MOG-IgG+ cases may be even more frequent than AQP4-IgG+ NMOSD at this age group. Several groups have published case reports and observational studies reporting clinical features and treatments regimes used in clinical practice. Nevertheless, given the recent recognition of this condition, there is still no randomized controlled trial on acute phase and long-term treatments. Most of the MOG-IgG+ patients receive high- or low-dose corticosteroids in the acute phase, demonstrating a better response compared to AQP4-IgG+ NMOSD. Other treatments for acute phase are intravenous human immunoglobulin and plasma exchange. Some of the MOG-IgG+ patients may have a monophasic disease and become MOG-IgG negative after 6 months to 2 years, so it is controversial to start long-term attack-prevention treatments after the first MOG-IgG demyelinating episode. Strategies like long-term oral corticosteroids and/or azathioprine may not have a clear benefit of these approaches and expose patients to the risks of complications due to prolonged corticosteroid use or immunosuppression. On the other hand, long-term treatment is usually indicated for relapsing cases, especially in those with persistent MOG-IgG titers. Some MOG-IgG+ patients have been misdiagnosed and exposed to disease-modifying treatments for multiple sclerosis (MS). Few anecdotal reports indicated that these patients experienced more relapses under interferon-beta but it is unclear if other drugs may increase the number and/or promote severe relapses as previously observed in AQP4-IgG+ NMOSD. In clinical practice, common long-term attack-prevention treatments for MOG-IgG+ patients are immunosuppressive drugs such as azathioprine, mycophenolate mofetil and cyclophosphamide or B-cell depleting therapies like rituximab; however, the clinical response seems to be different from AQP4-IgG+ NMOSD. A recent cohort study of relapsing MOG-IgG+ pediatric patients showed efficacy of repeated intravenous human immunoglobulin in reducing the number of relapses compared to immunosuppressive treatments.
Presenter Of 1 Presentation
TC06.03 - Presentation 03 (ID 606)
Abstract
Abstract
The myelin oligodendrocyte glycoprotein (MOG) is a protein expressed in the outermost surface of the myelin sheath of the central nervous system. Its localization makes it a target for autoantibodies. Over the last few years, serum autoantibodies recognizing MOG (MOG-IgG) have been associated to optic neuritis, myelitis, acute disseminated encephalomyelitis, cortical encephalitis, and some cases of aquaporin-4 (AQP4-IgG) negative neuromyelitis optica spectrum disorder (NMOSD). Pediatric cases represent a significant proportion of MOG-IgG+ patients; MOG-IgG+ cases may be even more frequent than AQP4-IgG+ NMOSD at this age group. Several groups have published case reports and observational studies reporting clinical features and treatments regimes used in clinical practice. Nevertheless, given the recent recognition of this condition, there is still no randomized controlled trial on acute phase and long-term treatments. Most of the MOG-IgG+ patients receive high- or low-dose corticosteroids in the acute phase, demonstrating a better response compared to AQP4-IgG+ NMOSD. Other treatments for acute phase are intravenous human immunoglobulin and plasma exchange. Some of the MOG-IgG+ patients may have a monophasic disease and become MOG-IgG negative after 6 months to 2 years, so it is controversial to start long-term attack-prevention treatments after the first MOG-IgG demyelinating episode. Strategies like long-term oral corticosteroids and/or azathioprine may not have a clear benefit of these approaches and expose patients to the risks of complications due to prolonged corticosteroid use or immunosuppression. On the other hand, long-term treatment is usually indicated for relapsing cases, especially in those with persistent MOG-IgG titers. Some MOG-IgG+ patients have been misdiagnosed and exposed to disease-modifying treatments for multiple sclerosis (MS). Few anecdotal reports indicated that these patients experienced more relapses under interferon-beta but it is unclear if other drugs may increase the number and/or promote severe relapses as previously observed in AQP4-IgG+ NMOSD. In clinical practice, common long-term attack-prevention treatments for MOG-IgG+ patients are immunosuppressive drugs such as azathioprine, mycophenolate mofetil and cyclophosphamide or B-cell depleting therapies like rituximab; however, the clinical response seems to be different from AQP4-IgG+ NMOSD. A recent cohort study of relapsing MOG-IgG+ pediatric patients showed efficacy of repeated intravenous human immunoglobulin in reducing the number of relapses compared to immunosuppressive treatments.
Moderator Of 1 Session
Invited Speaker Of 1 Presentation
TC06.03 - Presentation 03 (ID 606)
Abstract
Abstract
The myelin oligodendrocyte glycoprotein (MOG) is a protein expressed in the outermost surface of the myelin sheath of the central nervous system. Its localization makes it a target for autoantibodies. Over the last few years, serum autoantibodies recognizing MOG (MOG-IgG) have been associated to optic neuritis, myelitis, acute disseminated encephalomyelitis, cortical encephalitis, and some cases of aquaporin-4 (AQP4-IgG) negative neuromyelitis optica spectrum disorder (NMOSD). Pediatric cases represent a significant proportion of MOG-IgG+ patients; MOG-IgG+ cases may be even more frequent than AQP4-IgG+ NMOSD at this age group. Several groups have published case reports and observational studies reporting clinical features and treatments regimes used in clinical practice. Nevertheless, given the recent recognition of this condition, there is still no randomized controlled trial on acute phase and long-term treatments. Most of the MOG-IgG+ patients receive high- or low-dose corticosteroids in the acute phase, demonstrating a better response compared to AQP4-IgG+ NMOSD. Other treatments for acute phase are intravenous human immunoglobulin and plasma exchange. Some of the MOG-IgG+ patients may have a monophasic disease and become MOG-IgG negative after 6 months to 2 years, so it is controversial to start long-term attack-prevention treatments after the first MOG-IgG demyelinating episode. Strategies like long-term oral corticosteroids and/or azathioprine may not have a clear benefit of these approaches and expose patients to the risks of complications due to prolonged corticosteroid use or immunosuppression. On the other hand, long-term treatment is usually indicated for relapsing cases, especially in those with persistent MOG-IgG titers. Some MOG-IgG+ patients have been misdiagnosed and exposed to disease-modifying treatments for multiple sclerosis (MS). Few anecdotal reports indicated that these patients experienced more relapses under interferon-beta but it is unclear if other drugs may increase the number and/or promote severe relapses as previously observed in AQP4-IgG+ NMOSD. In clinical practice, common long-term attack-prevention treatments for MOG-IgG+ patients are immunosuppressive drugs such as azathioprine, mycophenolate mofetil and cyclophosphamide or B-cell depleting therapies like rituximab; however, the clinical response seems to be different from AQP4-IgG+ NMOSD. A recent cohort study of relapsing MOG-IgG+ pediatric patients showed efficacy of repeated intravenous human immunoglobulin in reducing the number of relapses compared to immunosuppressive treatments.