Seronegative neuromyelitis optica spectrum disorder: Severe polysymptomatic presentation with successful treatment response

• Type: Article in International Scientific Journal
• Date: 2019

Authors

Pires Da Rosa, G (Author) Other; Costa, F (Author) Other; Guimarães, J (Author) Other; Friões F (Author) FMUP

Journal

Title: BMJ Case Reports

Vol. 12

ISSN: 1757-790X

Publisher: BMJ PUBLISHING GROUP

Indexing

Scopus - 1 Citation

Other information

Authenticus ID: P-00Q-C1M

DOI: 10.1136/bcr-2018-228553

Abstract (EN): 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. © 2019 BMJ Publishing Group Limited.

Language: English

Type (Professor's evaluation): Scientific