Motor end-plate analysis to diagnose immune-mediated myasthenia gravis in seronegative patients.

Autor: Nagaoka A; Department of Clinical Neuroscience, Unit of Clinical Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki City, Japan. Electronic address: a-naga@nagasaki-u.ac.jp., Tsujino A; Department of Clinical Neuroscience, Unit of Clinical Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki City, Japan. Electronic address: akrtjn@nagasaki-u.ac.jp., Shiraishi H; Department of Neurology and Strokology, Nagasaki University Hospital, Nagasaki City, Japan., Kanamoto T; Department of Neurology and Strokology, Nagasaki University Hospital, Nagasaki City, Japan., Shima T; Department of Neurology and Strokology, Nagasaki University Hospital, Nagasaki City, Japan. Electronic address: tomoakishima0915@nagasaki-u.ac.jp., Yoshimura S; Department of Neurology and Strokology, Nagasaki University Hospital, Nagasaki City, Japan. Electronic address: shun-yoshimura@nagasaki-u.ac.jp., Miyazaki T; Department of Neurology and Strokology, Nagasaki University Hospital, Nagasaki City, Japan. Electronic address: tmiyazaki@nagasaki-u.ac.jp., Tateishi Y; Department of Neurology and Strokology, Nagasaki University Hospital, Nagasaki City, Japan. Electronic address: ytate@nagasaki-u.ac.jp., Tsujihata M; Nagasaki Kita Hospital, Nagasaki City, Japan. Electronic address: kita_m_tsuji@mx22.tiki.ne.jp., Motomura M; Medical Electronic Course, Nagasaki Institute of Applied Science, Nagasaki City, Japan. Electronic address: MOTOMURA_Masakatsu@NiAS.ac.jp., Maxwell S; Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital, Oxford, UK. Electronic address: susan.maxwell@ndcn.ox.ac.uk., Higuchi O; Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan. Electronic address: osmhgc_nkmc@nagasaki-u.ac.jp., Beeson D; Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital, Oxford, UK. Electronic address: David.beeson@ndcn.ox.ac.uk., Vincent A; Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital, Oxford, UK. Electronic address: angela.vincent@ndcn.ox.ac.uk.
Jazyk: angličtina
Zdroj: Journal of the neurological sciences [J Neurol Sci] 2022 Dec 15; Vol. 443, pp. 120494. Date of Electronic Publication: 2022 Nov 14.
DOI: 10.1016/j.jns.2022.120494
Abstrakt: This study aimed to evaluate the diagnostic usefulness of motor end-plate (MEP) analysis along with clustered acetylcholine receptor (AChR) antibody (Ab) assays in patients with myasthenia-like symptoms but negative routine AChR and muscle-specific kinase (MuSK) Ab tests. MEP analysis of muscle biopsies of the biceps brachii was performed in 20 patients to try to differentiate between those with or without immune-mediated myasthenia gravis (MG). Using a quantitative method, complement C3 deposition and AChR densities in MEPs were examined. Independently, cell-based assays were used to detect serum clustered-AChR Abs. Only five of 20 patients had complement deposition at MEPs; four of these patients had reduced AChR densities similar to those in patients with typical AChR Ab positive MG, and distinct from those in the remaining 15 patients. Two of the four serum samples from these patients had clustered-AChR Abs. All complement-positive patients were considered as having immune-mediated MG and improved with appropriate treatments; although one patient presented with MG 3 years later, the remaining patients had other diagnoses during over 10 years of follow-up. These results suggest the usefulness of MEP analysis of muscle biopsies in diagnosing immune-mediated MG in seronegative patients with myasthenia-like symptoms but, due to the invasiveness of the muscle biopsy procedure, clustered AChR Abs should, if possible, be tested first.
Competing Interests: Declaration of Competing Interest None.
(Copyright © 2022 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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