Optic Neuritis as the Presenting Feature of Neuromyelitis Optica (NMO): Diagnosis and Management

AbstractAcquired demyelinating syndromes (ADS) present with acute or subacute monofocal or polyfocal neurologic deficits localizing to the central nervous system. The clinical features of distinct ADS have been carefully characterized including optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. These disorders may all be monophasic disorders. Alternatively, optic neuritis, partial transverse myelitis, and acute disseminated encephalomyelitis may be first presentations of a relapsing or polyphasic neuroinflammatory disorder, such as multiple sclerosis or neuromyelitis optica. The clinical features of these disorders and the differential diagnosis are discussed in this article.

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Optic Neuritis–Independent Retinal Atrophy in Neuromyelitis Optica Spectrum Disorder

Journal of Neuro-Ophthalmology ◽

10.1097/wno.0000000000001282 ◽

2021 ◽

Vol Publish Ahead of Print ◽

Author(s):

Angeliki G. Filippatou ◽

Eleni S. Vasileiou ◽

Yufan He ◽

Kathryn C. Fitzgerald ◽

Grigorios Kalaitzidis ◽

...

Keyword(s):

Optic Neuritis ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Retinal Atrophy

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Seronegative Neuromyelitis Optica: A Case Report of a Hispanic Male

Case Reports in Neurology ◽

10.1159/000446105 ◽

2016 ◽

Vol 8 (2) ◽

pp. 102-107 ◽

Cited By ~ 2

Author(s):

Nabeel Badri ◽

Mohamed Teleb ◽

Saad Syed ◽

Miraie Wardi ◽

Mateo Porres-Aguilar ◽

...

Keyword(s):

Optic Neuritis ◽

Neuromyelitis Optica ◽

Brain Mri ◽

Sudden Onset ◽

Interesting Case ◽

Imaging Studies ◽

Thoracic Spinal Cord ◽

Lower Extremity Weakness ◽

Thoracic Spinal ◽

Hispanic Male

Neuromyelitis optica (NMO) is a rare disease, common in white females and rarely reported in Hispanic males. It is usually associated with recurrent demyelinating spectrum that is autoimmune in nature. The diagnosis is usually confirmed by antibody biomarkers; however, they can be negative and lead to more dilemma in diagnosis. Furthermore, the course of disease and prognosis are different in seronegative as compared to seropositive NMO. Treatment is similar in both subgroups with new approaches under investigation for seronegative NMO patients. We present an interesting case of a 37-year-old Hispanic male who presented with sudden onset of lower extremity weakness, numbness, blurry vision, and urinary retention. Magnetic resonance imaging (MRI) of the thoracic spine showed multiphasic demyelinating process involving the thoracic spinal cord. His brain MRI also revealed changes suggesting optic neuritis. The patient met the criteria for diagnosis of NMO by having optic neuritis and myelitis by imaging studies despite having negative aquaporin-4 antibodies (AQP4-Ab). His condition improved after plasma exchange. NMO can be difficult to distinguish from acute multiple sclerosis in the early stages of the disease. Having AQP4-Ab testing is important for diagnosis with imaging studies; however, negative antibody results cannot exclude the diagnosis, but rather group it in seronegative subtype. Ongoing studies and research suggest that seronegative NMO might have a different pathophysiology, manifestation, and prognosis.

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Visual field characteristics in neuromyelitis optica in absence of and after one episode of optic neuritis

Clinical Ophthalmology ◽

10.2147/opth.s43894 ◽

2013 ◽

pp. 1145 ◽

Cited By ~ 13

Author(s):

Harold Merle ◽

Olindo ◽

Jeannin ◽

Angelique Donnio ◽

Richer ◽

...

Keyword(s):

Visual Field ◽

Optic Neuritis ◽

Neuromyelitis Optica

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First-ever optic neuritis: distinguishing subsequent neuromyelitis optica from multiple sclerosis

Neurological Sciences ◽

10.1007/s10072-014-1635-6 ◽

2014 ◽

Vol 35 (5) ◽

pp. 781-783 ◽

Cited By ~ 20

Author(s):

Young-Min Lim ◽

So Young Pyun ◽

Hyun Taek Lim ◽

In Hye Jeong ◽

Kwang-Kuk Kim

Keyword(s):

Multiple Sclerosis ◽

Optic Neuritis ◽

Neuromyelitis Optica

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Factors influencing intravenous methylprednisolone pulse therapy in Chinese patients with isolated optic neuritis associated with AQP4 antibody-seropositive neuromyelitis optica

Scientific Reports ◽

10.1038/s41598-021-01109-5 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Sitong Guo ◽

Hanqiu Jiang ◽

Libin Jiang ◽

Jingting Peng ◽

Hongjuan Liu ◽

...

Keyword(s):

Visual Acuity ◽

Optic Neuritis ◽

Neuromyelitis Optica ◽

Age At Onset ◽

Chinese Patients ◽

Intravenous Methylprednisolone ◽

Methylprednisolone Pulse ◽

Factors Influencing ◽

Intravenous Methylprednisolone Pulse ◽

Aqp4 Antibody

AbstractThis study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 female) diagnosed with NMO-ON in the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to September 2020. All patients with AQP4-antibody seropositivity had clinical manifestations of acute ON, excluding other diagnoses and received IVMP treatment at 500 mg/day or 1000 mg/day for 3 days. Primary outcome was the extent of improvement in logMAR visual acuity after IVMP treatment. The therapeutic influences of sex, age, baseline visual acuity, therapeutic intervals, and IVMP dose on acute NMO-ON were analysed. Chi-square tests, Mann–Whitney U-tests, Kruskal–Wallis tests, Spearman’s correlation coefficients, and multiple linear regression were used for statistical analysis. Age ranged between 7 and 80 years (median age, 44; interquartile range [IQR], 29–52) years. Among the 243 eyes, the median improvement in logMAR visual acuity was 0.3 (IQR, 0–0.9). Therapeutic efficacy of IVMP was significantly higher in female than in male patients (Z = 2.117, P = 0.034). The treatment effect gradually decreased with increase in age at onset (Rs = 0.157, P = 0.015), and visual improvement was significantly lower in patients aged > 50 years than in those ≤ 50 years (Z = 2.571, P = 0.010). When patients had low visual acuity at onset, improvements were more obvious (rho = − 0.317, P < 0.001); however, final visual acuity was still low (rho = 0.688, P < 0.001). Therapeutic effect was negatively correlated with therapeutic intervals (rho = 0.228, P = 0.001). Dosage of methylprednisolone (1000 mg/day or 500 mg/day) did not significantly influence treatment efficacy (Z = 0.951 P = 0.342). Therefore, IVMP therapy can improve visual acuity in the affected eyes of patients with AQP4 antibody-seropositive NMO-ON with similar effect at 500 mg/day and 1000 mg/day doses. Sex, age at onset, and therapeutic intervals may influence the efficacy of IVMP in patients with NMO-ON.

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