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Seronegative neuromyelitis optica spectrum disorder: severe polysymptomatic presentation with successful treatment response.
BMJ Case Rep 2019; 12(3)BC

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.

Authors+Show Affiliations

Intermediate Care Unit, Department of Internal Medicine, Centro Hospitalar Universitário de São João, Porto, Portugal.Department of Neuroradiology, Centro Hospitalar Universitário de São João, Porto, Portugal.Department of Neurology, Centro Hospitalar Universitário de São João, Porto, Portugal. Neurology and Neurosurgery Unit of Clinical Neurosciences and Mental Health Department, Faculty of Medicine, University of Porto, Porto, Portugal.Intermediate Care Unit, Department of Internal Medicine, Centro Hospitalar Universitário de São João, Porto, Portugal. Cardiovascular Research Centre, University of Porto Medical School, Porto, Portugal.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

30878968

Citation

Pires da Rosa, Gilberto, et al. "Seronegative Neuromyelitis Optica Spectrum Disorder: Severe Polysymptomatic Presentation With Successful Treatment Response." BMJ Case Reports, vol. 12, no. 3, 2019.
Pires da Rosa G, Costa F, Guimarães J, et al. Seronegative neuromyelitis optica spectrum disorder: severe polysymptomatic presentation with successful treatment response. BMJ Case Rep. 2019;12(3).
Pires da Rosa, G., Costa, F., Guimarães, J., & Friões, F. (2019). Seronegative neuromyelitis optica spectrum disorder: severe polysymptomatic presentation with successful treatment response. BMJ Case Reports, 12(3), doi:10.1136/bcr-2018-228553.
Pires da Rosa G, et al. Seronegative Neuromyelitis Optica Spectrum Disorder: Severe Polysymptomatic Presentation With Successful Treatment Response. BMJ Case Rep. 2019 Mar 16;12(3) PubMed PMID: 30878968.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Seronegative neuromyelitis optica spectrum disorder: severe polysymptomatic presentation with successful treatment response. AU - Pires da Rosa,Gilberto, AU - Costa,Francisca, AU - Guimarães,Joana, AU - Friões,Fernando, Y1 - 2019/03/16/ PY - 2019/3/18/entrez PY - 2019/3/18/pubmed PY - 2019/6/22/medline KW - brain stem / cerebellum KW - immunology KW - neuroimaging KW - neurology KW - spinal cord JF - BMJ case reports JO - BMJ Case Rep VL - 12 IS - 3 N2 - 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. SN - 1757-790X UR - https://www.unboundmedicine.com/medline/citation/30878968/Seronegative_neuromyelitis_optica_spectrum_disorder:_severe_polysymptomatic_presentation_with_successful_treatment_response L2 - http://casereports.bmj.com/cgi/pmidlookup?view=long&pmid=30878968 DB - PRIME DP - Unbound Medicine ER -