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Neuropathology Of Neuro-Ophthalmic Disorders
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Generally, the ON is associated with MS, neuromyelitis optica (NMO) spectrum disorder and myelin oligodendrocyte glycoprotein autoantibodies (MOG-IgG) disorder. The ON is seen approximately in 70% cases of MS, and it is the presenting symptom in 25% cases.13 Patches of chronic demyelination develop in this condition anywhere in the optic pathway. The ON in NMO may occur either alone or it may be accompanied by transverse myelitis. The myelitis may occur within 1–2 weeks to 6 months. Approximately 80% of the affected patients are seropositive for AQP4-IgG.14 Non-whites (Asians) and women are more frequently affected by this disease compared to white population. A high risk of recurrence has been seen in this condition, and the prognosis of visual recovery is generally poor. The ON associated with MOG-IgG commonly presents as acute disseminated encephalomyelitis (ADEM) or recurrent ON. It is generally severe, painful and associated with optic disc edema, but fortunately it is steroid responsive. The differential diagnosis of retrobulbar ON may mimic compressive optic neuropathy, paraneoplastic optic neuropathy, autoimmune optic neuropathy, etc., and therefore, these need to be excluded.
Cranial Neuropathies II, III, IV, and VI
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Tanyatuth Padungkiatsagul, Heather E. Moss
Optic nerve inflammation can also be caused by infectious agents such as syphilis, Lyme, or tuberculosis. Rarely, it is associated with rheumatologic disease such as lupus. Prolonged inflammation of the optic nerve in the absence of a connective tissue disease despite extensive rheumatologic or vasculitic work-up is termed autoimmune optic neuropathy.
Anti-Neutrophil Cytoplasmic Antibody-Associated Ocular Manifestations in Japan: A Review of 18 Patients
Published in Ocular Immunology and Inflammation, 2021
Masaru Miyanaga, Hiroshi Takase, Kyoko Ohno-Matsui
Several reports have described scleritis as the most common ocular manifestation in patients with AAV.4,5 The frequencies of ocular manifestations vary in each phenotype of AAV. Scleritis is more frequently reported in the GPA phenotype than in the MPA or EGPA phenotype. The frequency of scleritis in GPA is reportedly between 41.9% and 75.0%.4,5 Conversely, 24.0% patients with MPA and 3.3% patients with EGPA experience scleritis.5 The frequency of optic nerve involvement is reportedly 0.8% in patients with GPA, 8.0% in patients with MPA,5 and 17.0% in patients with EGPA.18 In contrast, several case studies have reported optic nerve involvement in cases of hypertrophic pachymeningitis with MPO-ANCA positivity.17,20 MPO-ANCA associated with optic nerve involvement may be an autoimmune optic neuropathy.21 In our study, the frequency of optic nerve involvement (50%) was higher than that reported previously. This may be related to the patient population in this study, since patients in our study comprised those with AAV such as GPA as well as those positive for serum ANCAs not categorized into any known phenotypes.