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J Neurol. 2016 Jan;263(1):140-9. doi: 10.1007/s00415-015-7952-8. Epub 2015 Nov 3.

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

Author information

1
Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford, OX3 9DU, UK. maciej.jurynczyk@ndcn.ox.ac.uk.
2
Department of Neurology, Medical University of Lodz, Lodz, Poland. maciej.jurynczyk@ndcn.ox.ac.uk.
3
Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, 55905, MN, USA.
4
Department of Neurology, School of Medicine, Istanbul Bilim University, Istanbul, Turkey.
5
Department of Neurology, Vejle Hospital and Neurobiology, Institute of Molecular Medicine, University of Southern Denmark, Odense, Denmark.
6
Department of Neurology, Atkinson Morley's Wing, St George's Hospital, London, UK.
7
The Townsville Hospital, 100 Angus Smith Drive, Douglas Qld 4814, Douglas, Townsville, Australia.
8
Department of Neurology, Queens Hospital Rom Valley Way, Romford, RM7 0AG, UK.
9
Department of Neurology, University Hospital Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
10
Division of Clinical Neuroscience, Queens Medical Center, University of Nottingham, Derby Road, Nottingham, NG7 2UH, UK.
11
Neuroscience Department, Santa Maria Hospital, University of Lisbon, Lisbon, Portugal.
12
Department of Neurology, Institute of Clinical Research, Odense, Odense University Hospital, University of Southern Denmark, Odense, Denmark.
13
NMO Clinical Service, The Walton Centre, Liverpool, UK.
14
Department of Neurology, National Cancer Center, Seoul, South Korea.
15
Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany.
16
Neuromyelitis Optica Clinic, John Hopkins University, 1800 E. Orleans St., Baltimore, MD, 21287, USA.
17
Service de Neurologie A, Hopital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 69677, Lyon, France.
18
University College Dublin, St. Vincent's University Hospital, Elm Park, Dublin 4, Ireland.
19
Anne Rowling Regenerative Neurology Clinic, University of Edinburgh, Chancellor's Building, Edinburgh, EH16 4SB, UK.
20
Department of Neurology, Tohoku University School of Medicine, Sendai, 980-8574, Japan.
21
Nitte University, Mangalore, 575018, Karnataka, India.
22
Department of Neurology, NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany.
23
Department of Neurology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
24
Department of Neurology, Medical University of Lodz, Lodz, Poland.
25
Neurology Service, University Hospital of Strasbourg, Strasbourg, France.
26
Department of Neurology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.
27
Department of Neurology, Neurosurgery and Psychiatry, University of Medicine and Pharmacy Carol Davila, Bucharest, Romania.
28
Mayo Clinic Division of Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, 13400 E Shea BLVD, Scottsdale, 85259, AZ, USA.
29
Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford, OX3 9DU, UK.
30
Nuffield Department of Clinical Neurosciences, Level 3, West Wing, John Radcliffe Hospital, University of Oxford, Headley Way, Headington, Oxford, OX3 9DU, UK. jacqueline.palace@ndcn.ox.ac.uk.

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); Devic’s syndrome; Multiple sclerosis; Optic neuritis; Transverse myelitis

PMID:
26530512
PMCID:
PMC4816597
DOI:
10.1007/s00415-015-7952-8
[Indexed for MEDLINE]
Free PMC Article

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