Sporadic amyotrophic lateral sclerosis with seropositive neuromyelitis optica spectrum disorder: A case report
- Abstract
- Rationale: Neuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disorder of the central nervous system with
an autoantibody against aquaporin-4 protein (AQP4), and amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease.
We report a female patient with ALS who had asymptomatic AQP4 antibody at the diagnosis of ALS, and NMOSD occurred 4 years
later after the diagnosis of ALS.
Patient concerns: She was already bedridden and had tracheostomy because of ALS which was diagnosed at her age of 55. At
the time of her ALS diagnosis, she had no brain or spinal cord lesions, but was seropositive for AQP4 antibody. At her age of 59, newonset complete paralysis of all extremities and severe pain on the posterior neck and both shoulders occurred and visited the
hospital.
Diagnosis: Longitudinally extensive transverse myelitis was diagnosed, which was the onset attack of seropositive NMOSD. The
diagnosis was confirmed based on the international consensus diagnostic criteria for NMOSD with MR imaging, cerebrospinal fluid
exam and laboratory work-ups with AQP4 antibody test.
Interventions: High dose methylprednisolone was administered for 5 days. Plasma exchange as a further treatment was
recommended, but she and her family refused.
Outcomes: Her pain was relieved after steroid treatment, but there was no improvement of her leg weakness.
Lessons: This case is a rare combination of neuroinflammatory and neurodegenerative diseases. Considering the alterations of
blood-brain barrier along with the progression of ALS, it highlights that the consequence of ALS pathogenesis might affect the
development of NMOSD. And the careful follow-up is recommended even in patients with profound weakness, especially if those
who were at risk of developing certain neurological disorders.
- All Author(s)
- J. Y. Kim
; H. J. Oh
; Y. Kim
; J. M. Seok
- Issued Date
- 2021
- Type
- Article
- Keyword
- amyotrophic lateral sclerosis; anti-aquaporin-4 antibody; neuromyelitis optica spectrum disorder
- Publisher
- Lippincott Williams & Wilkins
- ISSN
- 0025-7974
; 1536-5964
- Citation Title
- Medicine
- Citation Volume
- 100
- Citation Number
- 16
- Citation Start Page
- e25580
- Citation End Page
- e25580
- Language(ISO)
- eng
- DOI
- 10.1097/md.0000000000025580
- URI
- http://schca-ir.schmc.ac.kr/handle/2022.oak/2451
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