Highlights
- •The relative frequency of Neuromyelitis optica spectrum disorders in patients from Ecuador was 16%
- •Neuromyelitis optica spectrum disorders was most frequent in females, with onset in the fourth decade of life.
- •The AQP4-IgG seropositive in our cohort was 81%.
- •The demographic, clinical and radiological spectrum of neuromyelitis optica was wide in Ecuadorian patients.
Abstract
Background
Neuromyelitis optica spectrum disorder (NMOSD) is a complex disease characterized
by a severe inflammation of the central nervous system (CNS). This disease typically
manifests with recurrent optic neuritis (ON) and acute transverse myelitis (ATM).
The clinical and radiological spectrum of NMOSD is little known in Latin America (LATAM)
and few reports have been published in the literature so far. In Ecuador, no reports
on NMOSD have been published. For this reason we aimed to assess the demographic,
clinical and imaging characteristics of patients with NMOSD from third level hospitals
from Ecuador.
Methods
This is a descriptive study in which we assessed medical reports of patients with
inflammatory demyelinating diseases who were attended in third level hospitals from
Ecuador in 2017. Then we applied the 2015 diagnostic criteria, those patients who
met the new NMOSD diagnostic criteria were selected and analyzed. Additionally, exploratory
sub-analyses were subsequently carried out.
Results
We identified 59 patients with NMOSD, the relative frequency of NMOSD was 15.9%. The
multiple sclerosis (MS) /NMOSD ratio was 5.2:1. Twenty four percent of patients were
newly defined as having NMOSD when 2015 criteria was applied. The median time to diagnoses
was shorter by the 2015 criteria than 2006 criteria (p<0.001). NMOSD was more prevalent
in women (female/male ratio 4.4:1). The disease onset was more frequent at the fourth
decade of life. The most common symptoms at the disease onset were ON and the association
of ON with ATM. The mean of expanded disability status scale (EDSS) was 4.8 (SD±1.8).
Concomitant autoimmune diseases were infrequent in this population (11.9%). The brain
magnetic resonance imaging (MRI) abnormalities were present in 25.7% of patients at
disease onset. Spinal cord MRI showed longitudinally extensive transverse myelitis
(LETM) in 91.5% of cases. Recurrent NMOSD was frequent in this cohort (88%). Positivity
for antibodies against aquaporin-4 (AQP4-IgG) which was measured through indirect
immunofluorescence assay (IIF) was identified in 81% of the patients tested. Patients
with seronegative AQP4-IgG had higher grade of disability than seropositive patients
(p<0.05). Ninety eight percent of patients received treatment with immunosuppressive
drugs. Three patients died due to gastric cancer (1 patient) and infectious diseases
(2 patients).
Conclusions
This is the first descriptive study in an Ecuadorian cohort of patients with NMOSD.
We show a wide epidemiological, clinical and radiological spectrum of NMOSD.
Keywords
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Article info
Publication history
Published online: May 27, 2020
Accepted:
May 16,
2020
Received in revised form:
May 13,
2020
Received:
March 9,
2020
Identification
Copyright
© 2020 Elsevier B.V. All rights reserved.
