Is Neuromyelitis Optica Distinct From Multiple Sclerosis?

Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

“…However, research performed over the past two decades has cast serious doubt on the traditional idea of MS being a homogeneous disease with a number of clinicoradiological “variants”:The demonstration of substantial histopathological as well as immunological differences among biopsied or autopsied patients with clinicoradiologically defined bona fide MS ('pattern 1 MS', characterized by T cell and macrophage infiltration, vs. 'pattern 2 MS', defined by additional antibody and complement deposition, suggesting a contribution of humoral mechanisms to disease pathology, vs. 'pattern 3 MS', characterized by distal oligodendrogliopathy with dysregulated myelin protein expression and oligodendrocyte apoptosis, still occuring on an inflammatory background) has reintroduced the idea of immunopathogenetic heterogeneity among patients with inflammatory CNS demyelination [8, 26, 27]. AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.MOG-IgG-positive encephalomyelitis (EM), which shares substantial clinical overlap with both MS and NMOSD, has also recently been shown to be an immunologically distinct entity in its own right [29]. The recent identification of novel autoantibodies against, for example, glial fibrillary acidic protein (GFAP) [30] or the flotillin-1/2 heterocomplex [31] in patients with CNS demyelination and reports on demyelination in patients with anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis [32] have the potential to further challenge the idea of MS as an immunologically homogeneous disease in the future.Finally, recent studies [33, 34] suggesting substantial differences between MS and BCS in regard to both radiological and histopathological presentation and immunopathology have meanwhile also cast doubt on the concept of BCS being a “variant of MS”.…”

“…AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.…”

Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

“…However, research performed over the past two decades has cast serious doubt on the traditional idea of MS being a homogeneous disease with a number of clinicoradiological “variants”:The demonstration of substantial histopathological as well as immunological differences among biopsied or autopsied patients with clinicoradiologically defined bona fide MS ('pattern 1 MS', characterized by T cell and macrophage infiltration, vs. 'pattern 2 MS', defined by additional antibody and complement deposition, suggesting a contribution of humoral mechanisms to disease pathology, vs. 'pattern 3 MS', characterized by distal oligodendrogliopathy with dysregulated myelin protein expression and oligodendrocyte apoptosis, still occuring on an inflammatory background) has reintroduced the idea of immunopathogenetic heterogeneity among patients with inflammatory CNS demyelination [8, 26, 27]. AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.MOG-IgG-positive encephalomyelitis (EM), which shares substantial clinical overlap with both MS and NMOSD, has also recently been shown to be an immunologically distinct entity in its own right [29]. The recent identification of novel autoantibodies against, for example, glial fibrillary acidic protein (GFAP) [30] or the flotillin-1/2 heterocomplex [31] in patients with CNS demyelination and reports on demyelination in patients with anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis [32] have the potential to further challenge the idea of MS as an immunologically homogeneous disease in the future.Finally, recent studies [33, 34] suggesting substantial differences between MS and BCS in regard to both radiological and histopathological presentation and immunopathology have meanwhile also cast doubt on the concept of BCS being a “variant of MS”.…”

“…AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.…”

Evaluation of pattern-reversal visual evoked potential in patients with neuromyelitis optica

“…The identification of VEP patterns in patients with NMO can help differentiate this condition from MS. A significant percentage of non-evoked visual responses, and reductions of P100 wave amplitudes with normal latency, were observed in this study; these features are not related to the findings of the VEP in MS. 20 The most common changes in the studies of VEP in MS, are: absolute increase in latency of P100 wave, changes in the morphology of this wave, and in few cases the absence of response. 18,19,[21][22][23] In the study by Matthews et al (1977) a total of 223 individuals were evaluated by VEP testing in order to assess the differences in the pattern of responses in patients with definite, probable, and possible MS, and healthy controls.…”

“…The identification of VEP patterns in patients with NMO can help differentiate this condition from MS. A significant percentage of non-evoked visual responses, and reductions of P100 wave amplitudes with normal latency, were observed in this study; these features are not related to the findings of the VEP in MS. 20…”

NMO in pediatric patients: brain involvement and clinical expression

“…The patients described in this study were children seen in two different cities in Venezuela: [1] the Neuropediatrics Service of the JM de los Rios Pediatric Hospital, in Caracas, and [2] The University Hospital in Maracaibo. Maracaibo and Caracas are the most populated cities in the country.…”

Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

“…However, research performed over the past two decades has cast serious doubt on the traditional idea of MS being a homogeneous disease with a number of clinicoradiological “variants”:The demonstration of substantial histopathological as well as immunological differences among biopsied or autopsied patients with clinicoradiologically defined bona fide MS ('pattern 1 MS', characterized by T cell and macrophage infiltration, vs. 'pattern 2 MS', defined by additional antibody and complement deposition, suggesting a contribution of humoral mechanisms to disease pathology, vs. 'pattern 3 MS', characterized by distal oligodendrogliopathy with dysregulated myelin protein expression and oligodendrocyte apoptosis, still occuring on an inflammatory background) has reintroduced the idea of immunopathogenetic heterogeneity among patients with inflammatory CNS demyelination [8, 26, 27]. AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.MOG-IgG-positive encephalomyelitis (EM), which shares substantial clinical overlap with both MS and NMOSD, has also recently been shown to be an immunologically distinct entity in its own right [29]. The recent identification of novel autoantibodies against, for example, glial fibrillary acidic protein (GFAP) [30] or the flotillin-1/2 heterocomplex [31] in patients with CNS demyelination and reports on demyelination in patients with anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis [32] have the potential to further challenge the idea of MS as an immunologically homogeneous disease in the future.Finally, recent studies [33, 34] suggesting substantial differences between MS and BCS in regard to both radiological and histopathological presentation and immunopathology have meanwhile also cast doubt on the concept of BCS being a “variant of MS”.…”

“…AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.…”

Evaluation of pattern-reversal visual evoked potential in patients with neuromyelitis optica

“…The identification of VEP patterns in patients with NMO can help differentiate this condition from MS. A significant percentage of non-evoked visual responses, and reductions of P100 wave amplitudes with normal latency, were observed in this study; these features are not related to the findings of the VEP in MS. 20 The most common changes in the studies of VEP in MS, are: absolute increase in latency of P100 wave, changes in the morphology of this wave, and in few cases the absence of response. 18,19,[21][22][23] In the study by Matthews et al (1977) a total of 223 individuals were evaluated by VEP testing in order to assess the differences in the pattern of responses in patients with definite, probable, and possible MS, and healthy controls.…”

“…The identification of VEP patterns in patients with NMO can help differentiate this condition from MS. A significant percentage of non-evoked visual responses, and reductions of P100 wave amplitudes with normal latency, were observed in this study; these features are not related to the findings of the VEP in MS. 20…”

NMO in pediatric patients: brain involvement and clinical expression

“…The patients described in this study were children seen in two different cities in Venezuela: [1] the Neuropediatrics Service of the JM de los Rios Pediatric Hospital, in Caracas, and [2] The University Hospital in Maracaibo. Maracaibo and Caracas are the most populated cities in the country.…”

Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

“…However, research performed over the past two decades has cast serious doubt on the traditional idea of MS being a homogeneous disease with a number of clinicoradiological “variants”:The demonstration of substantial histopathological as well as immunological differences among biopsied or autopsied patients with clinicoradiologically defined bona fide MS ('pattern 1 MS', characterized by T cell and macrophage infiltration, vs. 'pattern 2 MS', defined by additional antibody and complement deposition, suggesting a contribution of humoral mechanisms to disease pathology, vs. 'pattern 3 MS', characterized by distal oligodendrogliopathy with dysregulated myelin protein expression and oligodendrocyte apoptosis, still occuring on an inflammatory background) has reintroduced the idea of immunopathogenetic heterogeneity among patients with inflammatory CNS demyelination [8, 26, 27]. AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.MOG-IgG-positive encephalomyelitis (EM), which shares substantial clinical overlap with both MS and NMOSD, has also recently been shown to be an immunologically distinct entity in its own right [29]. The recent identification of novel autoantibodies against, for example, glial fibrillary acidic protein (GFAP) [30] or the flotillin-1/2 heterocomplex [31] in patients with CNS demyelination and reports on demyelination in patients with anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis [32] have the potential to further challenge the idea of MS as an immunologically homogeneous disease in the future.Finally, recent studies [33, 34] suggesting substantial differences between MS and BCS in regard to both radiological and histopathological presentation and immunopathology have meanwhile also cast doubt on the concept of BCS being a “variant of MS”.…”

“…AQP4-IgG-positive NMO has been convincingly shown to be distinct from MS with regard to pathogenesis, prognosis and optimum treatment and—after a short period of disbelief and debates between “lumpers and splitters” [28]—is now considered a disease entity in its own right by virtually all experts in the field.…”

Evaluation of pattern-reversal visual evoked potential in patients with neuromyelitis optica

“…The identification of VEP patterns in patients with NMO can help differentiate this condition from MS. A significant percentage of non-evoked visual responses, and reductions of P100 wave amplitudes with normal latency, were observed in this study; these features are not related to the findings of the VEP in MS. 20 The most common changes in the studies of VEP in MS, are: absolute increase in latency of P100 wave, changes in the morphology of this wave, and in few cases the absence of response. 18,19,[21][22][23] In the study by Matthews et al (1977) a total of 223 individuals were evaluated by VEP testing in order to assess the differences in the pattern of responses in patients with definite, probable, and possible MS, and healthy controls.…”

“…The identification of VEP patterns in patients with NMO can help differentiate this condition from MS. A significant percentage of non-evoked visual responses, and reductions of P100 wave amplitudes with normal latency, were observed in this study; these features are not related to the findings of the VEP in MS. 20…”

NMO in pediatric patients: brain involvement and clinical expression

“…The patients described in this study were children seen in two different cities in Venezuela: [1] the Neuropediatrics Service of the JM de los Rios Pediatric Hospital, in Caracas, and [2] The University Hospital in Maracaibo. Maracaibo and Caracas are the most populated cities in the country.…”