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Seronegative neuromyelitis optica spectrum disorder: severe polysymptomatic presentation with successful treatment response
- Source :
- BMJ Case Rep
- Publication Year :
- 2019
- Publisher :
- BMJ Publishing Group, 2019.
-
Abstract
- We report the case of a 50-year-old caucasian man presenting with lumbar pain, bilateral ataxia, central facial palsy, ophthalmoparesis and urinary retention. Cerebral MRI hinted a hyperintensity of the medulla oblongata and cervical medulla suggestive of myelitis. Cerebrospinal fluid displayed lymphocytic pleocytosis and elevated protein concentration. Without the possibility to rule out an infectious or inflammatory aetiology, antibiotics and corticosteroids were started. Nevertheless, neurological status deteriorated with loss of muscle strength and left eye amaurosis. A neuroaxis MRI exhibited encephalomyelitis with signal abnormalities involving the pons, medulla oblongata, left optic nerve and cervicodorsal medulla. Although negative for aquaporin-4-IgG antibodies, the patient fulfilled criteria for seronegative neuromyelitis optica spectrum disorder with the presence of multiple core clinical characteristics. Through early institution of corticosteroids, plasma exchange and rituximab, good functional recovery was achieved (Expanded Disability Status Scale score of 2). However, left eye amaurosis persisted despite salvage therapy with intravenous immunoglobulin.
- Subjects :
- 0301 basic medicine
Male
medicine.medical_specialty
Ataxia
Lymphocytic pleocytosis
Myelitis
Gastroenterology
Ophthalmoparesis
03 medical and health sciences
Amaurosis
0302 clinical medicine
Rare Disease
Internal medicine
Pons
medicine
Humans
Immunologic Factors
Medulla
Autoantibodies
Medulla Oblongata
Neuromyelitis optica
Plasma Exchange
business.industry
Neuromyelitis Optica
Optic Nerve
General Medicine
Middle Aged
medicine.disease
Magnetic Resonance Imaging
Hyperintensity
030104 developmental biology
Treatment Outcome
Immunoglobulin G
medicine.symptom
business
Rituximab
Tomography, X-Ray Computed
030217 neurology & neurosurgery
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Journal :
- BMJ Case Rep
- Accession number :
- edsair.doi.dedup.....dc87f071ace47cba39a25108c6c2fbca