Very late onset neuromyelitis optica spectrum disorders.
BACKGROUND AND PURPOSE
Neuromyelitis optica spectrum disorder (NMOSD) often presents in the elderly with an insidious onset of symptoms and aggressive progression. There have been anecdotal cases of very late onset (VLO)-NMOSD, but case series reports are rare. The aim of this retrospective study was to clarify the clinical features of VLO-NMOSD.


METHODS
According to the age at onset, we classified patients with NMOSD into three subgroups: ≤49 years, early onset NMOSD (EO-NMOSD); 50-69 years, late onset NMOSD (LO-NMOSD); and ≥70 years, VLO-NMOSD. We evaluated the clinical characteristics, magnetic resonance imaging (MRI) findings, laboratory data, and immunotherapies of the groups.


RESULTS
Overall, 12 men and 64 women with a median (interquartile range) age at onset and duration of disease of 42.0 (29.0-55.8) years and 70.0 (16.3-143.0) months, respectively, were included. Eight (11%) patients had VLO-NMOSD, 22 (29%) had LO-NMOSD, and 46 (61%) had EO-NMOSD. Patients with EO-NMOSD had a significantly longer interval between episodes as well as time between the first symptom and diagnosis of NMOSD than did those with VLO-NMOSD and LO-NMOSD (p = 0.046). Optic neuritis and nerve lesions on MRI were significantly less frequent in patients with VLO-NMOSD than in those with LO-NMOSD and EO-NMOSD (p = 0.002 and p = 0.028, respectively). In contrast, patients with VLO-NMOSD had higher nadir Expanded Disability Status Scale and Nurick scale scores and a significantly longer spinal lesion length than did those with LO-NMOSD and EO-NMOSD (p = 0.029, p = 0.049, and p = 0.032, respectively).


CONCLUSIONS
Patients with VLO-NMOSD tend to develop severe myelitis with long cord lesions but not optic neuritis.
['Nakahara|Keiichi|K|0000-0002-8931-8861', 'Nakane|Shunya|S|0000-0003-1958-095X', 'Nagaishi|Akiko|A|', 'Narita|Tomoko|T|', 'Matsuo|Hidenori|H|', 'Ando|Yukio|Y|']
D017668:Age of Onset D000368:Aged D051401:Aquaporin 4 D005260:Female D006801:Humans D008279:Magnetic Resonance Imaging D008297:Male D008875:Middle Aged D009187:Myelitis D009471:Neuromyelitis Optica D009902:Optic Neuritis D012189:Retrospective Studies
neuromyelitis optica aging magnetic resonance imaging optic neuritis transverse myelitis