Highlights
- •Patients presented with a combination of all known presentations of NMOSD, including ON and myelitis/ symptomatic narcolepsy.
- •28 patients (77.8%) had longitudinal extensive transverse myelitis on MRI spine, whereas 27 patients (75%) had normal MRI brains.
- •In terms of treatment, 26 patients (72.2%) received pulse steroid while 10 patients (27.8%) received both pulse therapy and plasmapheresis.
- •29 patients were treated with azathioprine (80.6%), 4 patients were treated with rituximab (11.1%), and 2 (8.6%) were treated with mycophenolate mofetil (5.6%) as immunomodulatory treatment of NMOSD.
- •More than half of the patients were diagnosed within 12 months of the index event (range 0–144 months), and multiple sclerosis was the most common initial misdiagnosis.
Abstract
Background
The clinical and radiological characteristics of neuromyelitis optica spectrum disorder
(NMOSD) from Pakistan is unknown. Our study aimed to describe the clinical and radiological
features of NMOSD patients presenting to a Pakistani tertiary care center.
Materials and methods
This retrospective, observational study was conducted at the Neurology Department,
Pakistan Institute of Medical Sciences between January 2017 and September 2021 (56
months). The study included patients diagnosed with neuromyelitis optica spectrum
disorder (NMOSD) according to the 2015 International Panel for NMO Diagnosis (IPND)
criteria, with the exclusion of patients under 12 years of age and those who tested
positive for Myelin oligodendrocyte glycoprotein (MOG) IgG antibody. The patients
were divided into two groups based on clinical presentation and the presence of NMO-IgG
antibodies: NMO-IgG positive NMO (Seropositive NMO) and NMO-IgG negative (Seronegative
NMO). The clinical features of NMOSD were recorded, and data was analyzed using SPSS
version 26.0.
Results
Among 204 patients with suspected demyelination, multiple sclerosis was diagnosed
in 100 individuals (49.02%), while acute disseminated encephalomyelitis (ADEM), clinically
isolated syndrome (CIS), and neuromyelitis optica (NMO) were found in 5 patients each
(2.45%, 2.45%, and 17.65%, respectively). Out of 36 patients with NMO, 32 (88.89%)
tested positive for NMO-Ab, while the remaining 4 (11.11%) were seronegative for both
NMO and anti-MOG Abs. The mean age of NMO-positive patients who tested positive for
NMO antibodies was 31.03±10.12 years, compared to 27.95±2.5 years for NMO-negative
patients, though this difference was not statistically significant (p>0.05). Females were more commonly affected by NMO, accounting for 72.2% of the NMO-positive
group, and there was a significant difference in clinical phenotypes between the two
groups (p<0.05). The NMO-positive group predominantly had relapsing NMO presentation (75%),
and 72.8% of these patients showed longitudinally extensive transverse myelitis on
the MRI spine. Azathioprine was the most frequently administered treatment for positive
NMO patients (69.44%), followed by rituximab and MMF. The follow-up period for the
study participants lasted 24 months.
Conclusion
This is the first study on NMOSD cases in Pakistan. According to the present study,
NMOSD is most prevalent among women in their forties. Relapsing NMO was the most common
form of presentation. 89% of patients had antibodies against AQP4. 72.8% of patients
suffered from LETM during the course of their disease. There are some features of
our NMOSD cases that appear comparable with those around the world, despite some limitations
in testing and access to care. It is clear that the clinical and radiological spectrums
of patients with NMO and NMOSD in this cohort are similar. It is reasonable to suspect
NMO if demyelinating episodes are not characteristic of MS.
Keywords
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Article info
Publication history
Published online: March 23, 2023
Accepted:
March 21,
2023
Received in revised form:
March 14,
2023
Received:
January 20,
2023
Identification
Copyright
© 2023 Elsevier B.V. All rights reserved.
