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Neurophysiological characteristics of MuSK antibody positive Myasthenia Gravis mice: Focal denervation and hypersensitivity to acetylcholinesterase inhibitors
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
2012 (English)In: Journal of the Neurological Sciences, ISSN 0022-510X, E-ISSN 1878-5883, Vol. 316, no 1-2, 150-157 p.Article in journal (Refereed) Published
Abstract [en]

Myasthenia Gravis (MG) patients with antibodies against the muscle specific tyrosine kinase (MuSK+) typically present with focal fatigue and atrophy of the facial and bulbar muscles, along with unbeneficial reactions upon administration of acetylcholinesterase inhibitors (AChEIs). This study addresses the neurophysiological characteristics in facial versus limb muscles, before and after intraperitoneal injection of AChEIs, in mice immunized with MuSK. We performed in-vivo neurophysiological examinations in the masseter and gastrocnemius muscles of mice with MuSK+experimental autoimmune MG (EAMG) and in healthy control mice before and after administration of AChEIs. Abnormal spontaneous activity (fibrillations) was observed in the masseter muscle of MuSK+mice. Furthermore, 94% of MuSK-immunized mice displayed so called extra discharges (EDs) upon administration of a therapeutic AChEI dose, in contrast to 22% of the control mice, indicating neuromuscular hyperactivity. These findings support functional denervation in the masseter muscle and neuromuscular hypersensitivity already at a standard dose of AChEIs in MuSK+EAMG.

Place, publisher, year, edition, pages
2012. Vol. 316, no 1-2, 150-157 p.
Keyword [en]
Neuromuscular transmission, MuSK, Myasthenia, EMG, Acetylcholinesterase inhibitors, Denervation
National Category
Clinical Laboratory Medicine
Research subject
Clinical Neurophysiology
URN: urn:nbn:se:uu:diva-167278DOI: 10.1016/j.jns.2011.12.016ISI: 000303177900028PubMedID: 22251934OAI: oai:DiVA.org:uu-167278DiVA: diva2:482989
Available from: 2012-01-24 Created: 2012-01-24 Last updated: 2012-05-28Bibliographically approved

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Rostedt Punga, Anna
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