Late Onset of Neuromyelitis Optica Spectrum Disorders

Fragoso, Yára Dadalti; Ruocco, Heloisa Helena; Dias, Ronaldo Maciel; Cabeca, H. L. S.; Gonçalves, Ricardo; Sousa, Nise Alessandra de Carvalho; Spessotto, Caroline Vieira; Tauil, Carlos Bernardo; Alves-Leon, Soniza Vieira; Gomes, Sidney; Gonçalves, Marcus Vinícius Magno; Machado, Suzana C. Nunes; Anacleto, Andrea; Correa, Eber Castro; Pimentel, Maria Lúcia Vellutini; Santos, Gutemberg Augusto Cruz dos

doi:10.1007/s40120-019-0143-2

Cited by 17 publications

(12 citation statements)

References 26 publications

(29 reference statements)

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“…5,[7][8][9][10][11][12][13] In our LO-NMOSD-ON cohort, the mean age at ON onset was approximately 65 years, which was slightly higher than the ages reported in previous studies (56-59.4 years). 5,[7][8][9][10][11][12][13] However, the mean age at ON onset was 32.6 years in our EO-NMOSD-ON cohort, which is comparable with the typical age at NMOSD onset in previous studies (32-41 years). [2][3][4] Moreover, our previously published article, which studied prognostic factors for visual outcomes following the first episode of ON in each affected eye in overall age group of NMOSD-ON patients, showed that a median age at NMOSD-ON onset was 36 years (range 4-84 years).…”

Section: Demographic Datacontrasting

confidence: 71%

“…6 Few studies have investigated the epidemiological and clinical characteristics of LO-NMOSD. 5,[7][8][9][10][11][12][13] Some have compared these aspects with early-onset NMOSD (EO-NMOSD; age at NMOSD onset < 50 years). 5,[10][11][12][13] To the best of our knowledge, no studies have been performed to specifically compare clinical characteristics and long-term visual outcomes between patients with late-onset neuromyelitis optica spectrum disorder-related optic neuritis (LO-NMOSD-ON) and those with early-onset neuromyelitis optica spectrum disorder-related optic neuritis (EO-NMOSD-ON).…”

Section: Introductionmentioning

confidence: 99%

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Comparison of Early- and Late-Onset NMOSD-Related Optic Neuritis in Thai Patients: Clinical Characteristics and Long-Term Visual Outcomes

Thongmee¹,

Narongkhananukul²,

Padungkiatsagul³

et al. 2021

OPTH

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Objective To compare demographic data, clinical and radiological characteristics, treatments, and long-term visual outcomes between patients with late-onset neuromyelitis optica spectrum disorder-related optic neuritis (LO-NMOSD-ON) (age at onset ≥ 50 years) and patients with early-onset neuromyelitis optica spectrum disorder-related optic neuritis (EO-NMOSD-ON) (age at onset < 50 years). Patients and Methods This retrospective study included 47 patients (69 eyes) who were diagnosed with neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) over a 12-year period. There were 14 patients (21 eyes) and 33 patients (48 eyes) in the LO-NMOSD-ON and EO-NMOSD-ON groups, respectively. Results LO-NMOSD-ON–affected eyes exhibited significantly worse median nadir visual acuity (VA) at optic neuritis (ON) onset, compared with EO-NMOSD-ON–affected eyes (2.7 logMAR (range 2.6–2.9 logMAR) vs 1.95 logMAR (range 0.4–2.9 logMAR); p = 0.03). Similarly, 100% of LO-NMOSD-ON–affected eyes demonstrated a nadir VA of worse than or equal to 1.0 logMAR, compared with 62.5% of EO-NMOSD-ON–affected eyes ( p = 0.03). LO-NMOSD-ON–affected eyes had a worse median final VA, compared with EO-NMOSD-ON–affected eyes (1.3 logMAR (range 0–2.9 logMAR) vs 0.3 logMAR (range 0–2.9 logMAR); adjusted p = 0.037). LO-NMOSD-ON–affected eyes more frequently exhibited a final VA of worse than or equal to 1.0 logMAR, compared with EO-NMOSD-ON–affected eyes (57.1% vs 27.0%; adjusted p = 0.039). A positive correlation was observed between age at ON onset of each eye and the final VA (logMAR) (Spearman r = 0.34, p = 0.0075). The remaining parameters did not significantly differ between the two groups. Conclusion Patients with LO-NMOSD-ON had significantly worse nadir VA at ON onset and significantly worse final VA, relative to patients with EO-NMOSD-ON. Age at ON onset of each eye was positively correlated with final VA (logMAR). Despite the difference in common age at onset, NMOSD-ON should be included in the differential diagnosis of late-onset acute to subacute optic neuropathy, along with ischemic optic neuropathy.

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Section: Demographic Datacontrasting

confidence: 71%

Section: Introductionmentioning

confidence: 99%

Comparison of Early- and Late-Onset NMOSD-Related Optic Neuritis in Thai Patients: Clinical Characteristics and Long-Term Visual Outcomes

Thongmee¹,

Narongkhananukul²,

Padungkiatsagul³

et al. 2021

OPTH

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“…A retrospective study that studied late onset NMOSD utilized a total of 37 patients. Of the 37 patients analysed, 22 were whites and 15 were Africans (Fragoso et al, 2019). From the above discussed results, ethnic differences seem to be important in determining the age of onset.…”

Section: Age Of Onsetmentioning

confidence: 86%

Neuromyelitis optica spectrum disorder: an overview

Ramakrishnan

Nagarajan

2020

Acta Neurobiologiae Experimentalis

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Neuromyelitis optica also known as Devic's syndrome is a rare autoimmune disorder that predominantly targets the optic nerves and the spinal cord. It is a debilitating disorder that damages a person's health. Initially it was considered as a variant of multiple sclerosis (MS). But, in 2004, a water channel protein associated antibody was found to be responsible for the disease. This helped in distinguishing the disease from multiple sclerosis. Multiple molecular mechanisms like complement dependent cytotoxicity, antibody-dependent cellular cytotoxicity etc. contribute to the disease. Certain environmental and genetic factors have been identified as risk factors of the disease. Initially, the disease was thought to affect only the optic nerves and the spinal cord. But certain regions of the brain have also been found to be attacked during the course of the disease. A small proportion of the patients have been found to be seronegative for the AQP4-IgG. Recently, the term neuromyelitis optica spectrum disorder has been framed to include all the features of the disease. The disease remains incurable despite the availability of various treatment modalities. This review presents critical information obtained from prior studies regarding the disease and also raise several questions to understand the research gaps in this field.

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“…Several studies have described the importance of the age at the onset of NMOSD; therefore, clinical differences might exist between late-onset (age at disease onset, >50 years) and early-onset NMOSD (age at disease onset, <50 years) NMOSD. Late-onset NMOSD may often be comorbid with age-related conditions compared with early-onset NMOSD [ 6 ]. Moreover, elderly patients have a tendency for a rapid worsening of their condition and poor response to immunomodulatory therapies [ 5 , 7 ].…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…In rare instances, NMOSD has been reported in elderly persons (over 50 years of age), and the clinical entity of late-onset NMOSD has been mentioned [ 3 ]. Compared with early-onset NNOSD (below 50 years of age), late-onset NMOSD may exhibit characteristic features, especially those associated with aging or other non-neurological issues [ 4 , 5 , 6 , 7 , 8 ]. Here, we report a case of very-late-onset NMOSD in an elderly patient (76 years of age) with breast cancer and Parkinson disease who exhibited an unusual disease course.…”

Section: Introductionmentioning

confidence: 99%

Very-Late-Onset Neuromyelitis Optica Spectrum Disorder in a Patient with Breast Cancer and Parkinson Disease

et al. 2021

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Anti-aquaporin-4 (anti-AQP-4) antibody-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder resulting in severe, recurrent optic neuritis, transverse myelitis, brain stem syndrome, and other types of neurological involvement. Its median age of onset has been reported to be around 40 years. We report herein a case of very-late-onset NMOSD (76 years of age) and try to promote its awareness as a type of neurological deterioration in elder patients. A 76-year-old woman suffering from Parkinson disease was admitted to our hospital because of consciousness disturbance. Cranial magnetic resonance imaging revealed the presence of fluid-attenuated inversion recovery high-signal-intensity lesions in the right peri- and intralateral ventricle. Part of this lesion and the meninges showed gadolinium enhancement. Physical examination revealed the presence of a tumor in the right breast, which was later diagnosed as invasive ductal carcinoma. In addition, laboratory examinations led to the detection of anti-AQP-4 antibodies in her serum; consequently, the patient was diagnosed as having NMOSD. She received initial pulsed steroid therapy, followed by right mastectomy. Although the patient’s consciousness improved significantly, she developed abrupt-onset bilateral leg weakness and multiple longitudinal spinal cord lesions. Additional steroid therapy ameliorated the patient’s leg weakness and reduced the swelling of the spinal cord.

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Late Onset of Neuromyelitis Optica Spectrum Disorders

Cited by 17 publications

References 26 publications

Comparison of Early- and Late-Onset NMOSD-Related Optic Neuritis in Thai Patients: Clinical Characteristics and Long-Term Visual Outcomes

Comparison of Early- and Late-Onset NMOSD-Related Optic Neuritis in Thai Patients: Clinical Characteristics and Long-Term Visual Outcomes

Neuromyelitis optica spectrum disorder: an overview

Very-Late-Onset Neuromyelitis Optica Spectrum Disorder in a Patient with Breast Cancer and Parkinson Disease

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