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Published on: April 14, 2014
Neuromyelitis optica and opticospinal multiple sclerosis: Mechanisms and pathogenesis
1Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.
Summary
Opticospinal MS (OSMS) in Asians shares features with neuromyelitis optica (NMO). While anti-aquaporin-4 (AQP4) antibodies are implicated in NMO, OSMS pathogenesis appears heterogeneous, involving both antibody-related and unrelated mechanisms.
Area of Science:
- Neuroimmunology
- Central Nervous System Diseases
Background:
- Multiple sclerosis (MS) is a CNS demyelinating disease.
- Neuromyelitis optica (NMO) selectively affects optic nerves and spinal cord.
- Opticospinal MS (OSMS) in Asians resembles NMO and involves opticospinal pathways.
Purpose of the Study:
- To investigate the relationship between OSMS and NMO.
- To explore the role of NMO-IgG/anti-aquaporin-4 (AQP4) antibodies in OSMS pathogenesis.
- To elucidate the heterogeneous mechanisms underlying OSMS and NMO.
Main Methods:
- Analysis of NMO-IgG/anti-AQP4 antibody presence in OSMS patients.
- Pathological examination of NMO and MS lesions.
- Assessment of astrocyte death and myelin basic protein (MBP) staining.
- Evaluation of cerebrospinal fluid (CSF) cytokine levels.
- Investigation of T cell responses to myelin proteins.
Main Results:
- OSMS in Asians shows features similar to NMO.
- Anti-AQP4 antibodies are present in a subset of OSMS patients.
- Pathology reveals variable AQP4 loss and preserved myelin in NMO lesions.
- Elevated pro-inflammatory cytokines (IL-17, IL-8, IFNγ, G-CSF) found in OSMS CSF.
- Evidence of epitope spreading in T cells reactive to myelin proteins.
Conclusions:
- OSMS in Asians may be distinct from NMO, with heterogeneous mechanisms.
- Pathogenesis involves both anti-AQP4 antibody-dependent and -independent pathways.
- Myelin-autoreactive T cells (Th17/Th1) may contribute to CNS inflammation in OSMS.
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