Late-onset neuromyelitis optica spectrum disorder: A case series from Iran

ObjectiveTo describe the clinical features of late-onset (≥50 years) neuromyelitis optica spectrum disorder (LO-NMOSD), to compare the outcome with that of early-onset (EO-NMOSD), and to identify predictors of disability.MethodsA retrospective, multicenter study of 238 patients with NMOSD identified by the 2015 criteria. Clinical and immunologic features of patients with LO-NMOSD were compared with those with EO-NMOSD. All patients were evaluated for aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) antibodies.ResultsSixty-nine (29%) patients had LO-NMOSD. Demographic features, initial disease presentation, annualized relapse rate, and frequency of AQP4-IgG and MOG-IgG did not differ between patients with LO-NMOSD and EO-NMOSD. Among patients with AQP4-IgG or double seronegativity, those with LO-NMOSD had a higher risk to require a cane to walk (hazard ratio [HR], 2.10, 95% CI 1.3–3.54, p = 0.003 for AQP4-IgG, and HR, 13.0, 95% CI 2.8–59.7, p = 0.001, for double seronegative). No differences in outcome were observed between patients with MOG-IgG and LO-NMOSD or EO-NMOSD. Older age at onset (for every 10-year increase, HR 1.63, 95% CI 1.35–1.92 p < 0.001) in NMOSD, and higher disability after the first attack (HR 1.68, 95% CI 1.32–2.14, p < 0.001), and double seronegativity (HR 3.74, 95% CI 1.03–13.6, p = 0.045) in LO-NMOSD were the main independent predictors of worse outcome.ConclusionsPatients with LO-NMOSD have similar clinical presentation but worse outcome than EO-NMOSD when they are double seronegative or AQP4-IgG positive. Serostatus and residual disability after first attack are the main predictors of LO-NMOSD outcome.

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Two Cases of Very-Late-Onset Neuromyelitis Optica Spectrum Disorder (NMOSD) in Patients over the Age of 80

Case Reports in Neurology ◽

10.1159/000505231 ◽

2020 ◽

Vol 12 (1) ◽

pp. 13-17 ◽

Cited By ~ 1

Author(s):

Shunya Fujiwara ◽

Yasuhiro Manabe ◽

Ryuta Morihara ◽

Taijun Yunoki ◽

Syoichiro Kono ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Clinical Course ◽

Late Onset ◽

Transverse Myelitis ◽

Spectrum Disorder ◽

High Dose ◽

Neuromyelitis Optica Spectrum Disorder ◽

Initial Manifestation ◽

High Dose Methylprednisolone ◽

Aqp4 Antibody

We report two cases of very-late-onset neuromyelitis optica spectrum disorder (NMOSD) in patients over the age of 80 with transverse myelopathy as the initial manifestation. In both cases, the patients presented with paraplegia and sensory, bladder, and rectal disturbances. Thoracic magnetic resonance imaging showed longitudinal high-intensity signals on a T2-weighted image. The patients received high-dose methylprednisolone. Their serum was positive for anti-AQP4 antibody (cell-based assay) during the clinical course. They were diagnosed with NMOSD and treated with immunoadsorption, plasmapheresis, and followed up with daily prednisolone. Very-late-onset NMOSD in patients over the age of 80 has only rarely been reported. The present cases suggest that NMOSD should be considered for elderly patients presenting with transverse myelitis. Early diagnosis and treatment are important.

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Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000343 ◽

2017 ◽

Vol 4 (4) ◽

pp. e343 ◽

Cited By ~ 30

Author(s):

Nasrin Asgari ◽

Eoin P. Flanagan ◽

Kazuo Fujihara ◽

Ho Jin Kim ◽

Hanne P. Skejoe ◽

...

Keyword(s):

Spinal Cord ◽

Neuromyelitis Optica ◽

Area Postrema ◽

Case Series ◽

Systemic Infection ◽

Median Number ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Retrospective Case ◽

Fluid Attenuated Inversion Recovery

Objective:To describe leptomeningeal blood-barrier impairment reflected by MRI gadolinium-enhanced lesions in patients with aquaporin-4 immunoglobulin G (AQP4-IgG)–positive neuromyelitis optica spectrum disorder (NMOSD).Methods:A retrospective case series of 11 AQP4-IgG–positive NMOSD patients with leptomeningeal enhancement (LME) were collected from 5 centers. External neuroradiologists, blinded to the clinical details, evaluated MRIs.Results:LME was demonstrated on postcontrast T1-weighted and fluid-attenuated inversion recovery images as a sign of leptomeningeal blood-barrier disruption and transient leakage of contrast agent into the subarachnoid space in 11 patients, 6 in the brain and 6 in the spinal cord. The patterns of LME were linear or extensive and were accompanied by periependymal enhancement in 5 cases and intraparenchymal enhancement in all cases. The location of LME in the spinal cord was adjacent to intraparenchymal contrast enhancement with involvement of a median number of 12 (range 5–17) vertebral segments. At the time of LME on MRI, all patients had a clinical attack such as encephalopathy (36%) and/or myelopathy (70%) with median interval between symptom onset and LME of 12 days (range 2–30). LME occurred in association with an initial area postrema attack (44%), signs of systemic infection (33%), or AQP4-IgG in CSF (22%) followed by clinical progression. LME was found at initial clinical presentation in 5 cases and at clinical relapses leading to a diagnosis of NMOSD in 6 cases.Conclusion:This study suggests that altered leptomeningeal blood barrier may be accompanied by intraparenchymal blood-brain barrier breakdown in patients with AQP4-IgG–positive NMOSD during relapses.

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Pediatric Neuromyelitis Optica Spectrum Disorder: Case Series and Literature Review

Life ◽

10.3390/life12010019 ◽

2021 ◽

Vol 12 (1) ◽

pp. 19

Author(s):

Michela Ada Noris Ferilli ◽

Roberto Paparella ◽

Ilaria Morandini ◽

Laura Papetti ◽

Lorenzo Figà Talamanca ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Pediatric Patients ◽

Demyelinating Disease ◽

Area Postrema ◽

Case Series ◽

Myelin Oligodendrocyte Glycoprotein ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Periaqueductal Gray Matter ◽

Aquaporin 4 Antibody

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events that primarily involve optic nerves and the spinal cord, but also affect other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. The aquaporin-4 antibody (AQP4-IgG) is specific for NMOSD. Recently, myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients. NMOSD is rare among children and adolescents, but early diagnosis is important to start adequate therapy. In this report, we present cases of seven pediatric patients with NMOSD and we review the clinical and neuroimaging characteristics, diagnosis, and treatment of NMOSD in children.

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