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Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes

Item Type:Article
Title:Status of diagnostic approaches to AQP4-IgG seronegative NMO and NMO/MS overlap syndromes
Creators Name:Juryńczyk, M. and Weinshenker, B. and Akman-Demir, G. and Asgari, N. and Barnes, D. and Boggild, M. and Chaudhuri, A. and D'hooghe, M. and Evangelou, N. and Geraldes, R. and Illes, Z. and Jacob, A. and Kim, H.J. and Kleiter, I. and Levy, M. and Marignier, R. and McGuigan, C. and Murray, K. and Nakashima, I. and Pandit, L. and Paul, F. and Pittock, S. and Selmaj, K. and de Sèze, J. and Siva, A. and Tanasescu, R. and Vukusic, S. and Wingerchuk, D. and Wren, D. and Leite, I. and Palace, J.
Abstract:Distinguishing aquaporin-4 IgG(AQP4-IgG)-negative neuromyelitis optica spectrum disorders (NMOSD) from opticospinal predominant multiple sclerosis (MS) is a clinical challenge with important treatment implications. The objective of the study was to examine whether expert clinicians diagnose and treat NMO/MS overlapping patients in a similar way. 12 AQP4-IgG-negative patients were selected to cover the range of clinical scenarios encountered in an NMO clinic. 27 NMO and MS experts reviewed their clinical vignettes, including relevant imaging and laboratory tests. Diagnoses were categorized into four groups (NMO, MS, indeterminate, other) and management into three groups (MS drugs, immunosuppression, no treatment). The mean proportion of agreement for the diagnosis was low (p o = 0.51) and ranged from 0.25 to 0.73 for individual patients. The majority opinion was divided between NMOSD versus: MS (nine cases), monophasic longitudinally extensive transverse myelitis (LETM) (1), acute disseminated encephalomyelitis (ADEM) (1) and recurrent isolated optic neuritis (RION) (1). Typical NMO features (e.g., LETM) influenced the diagnosis more than features more consistent with MS (e.g., short TM). Agreement on the treatment of patients was higher (p o = 0.64) than that on the diagnosis with immunosuppression being the most common choice not only in patients with the diagnosis of NMO (98 %) but also in those indeterminate between NMO and MS (74 %). The diagnosis in AQP4-IgG-negative NMO/MS overlap syndromes is challenging and diverse. The classification of such patients currently requires new diagnostic categories, which incorporate lesser degrees of diagnostic confidence. Long-term follow-up may identify early features or biomarkers, which can more accurately distinguish the underlying disorder.
Keywords:All Demyelinating Disease (CNS), Multiple Sclerosis, Devic's Syndrome, Optic Neuritis, Transverse Myelitis
Source:Journal of Neurology
ISSN:0340-5354
Publisher:Springer (Germany)
Volume:263
Number:1
Page Range:140-149
Date:January 2016
Official Publication:https://doi.org/10.1007/s00415-015-7952-8
PubMed:View item in PubMed

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