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Neurophysiological characteristics of MuSK antibody positive Myasthenia Gravis mice: Focal denervation and hypersensitivity to acetylcholinesterase inhibitors
Uppsala universitet, Medicinska och farmaceutiska vetenskapsområdet, Medicinska fakulteten, Institutionen för neurovetenskap, Klinisk neurofysiologi.
2012 (engelsk)Inngår i: Journal of the Neurological Sciences, ISSN 0022-510X, E-ISSN 1878-5883, Vol. 316, nr 1-2, s. 150-157Artikkel i tidsskrift (Fagfellevurdert) 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.

sted, utgiver, år, opplag, sider
2012. Vol. 316, nr 1-2, s. 150-157
Emneord [en]
Neuromuscular transmission, MuSK, Myasthenia, EMG, Acetylcholinesterase inhibitors, Denervation
HSV kategori
Forskningsprogram
Klinisk neurofysiologi
Identifikatorer
URN: urn:nbn:se:uu:diva-167278DOI: 10.1016/j.jns.2011.12.016ISI: 000303177900028PubMedID: 22251934OAI: oai:DiVA.org:uu-167278DiVA, id: diva2:482989
Tilgjengelig fra: 2012-01-24 Laget: 2012-01-24 Sist oppdatert: 2017-12-08bibliografisk kontrollert

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