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Cholinergic neuromuscular hyperactivity in patients with myasthenia gravis seropositive for MuSK antibody
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology. (Erik Stålberg)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology. (Erik Stålberg)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology. (Erik Stålberg)
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology. (Erik Stålberg)
2006 (English)In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 34, no 1, 111-115 p.Article in journal (Refereed) Published
Abstract [en]

A 75-year-old man with severe oculobulbar myasthenia gravis (MG) treated with acetylcholine esterase inhibitors (AChEIs) was found to have muscle-specific tyrosine kinase (MuSK) antibodies. Neurophysiological examination displayed extra repetitive discharges after the compound motor action potential (CMAP) at low-frequency stimulation, possibly triggered by AChEI. This indicates an abnormal sensitivity to acetylcholine in patients with MuSK antibodies and may be a useful indicator of the adverse effect of AChEI treatment in these patients. Muscle Nerve, 2006

Place, publisher, year, edition, pages
2006. Vol. 34, no 1, 111-115 p.
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-95261DOI: 10.1002/mus.20515PubMedID: 16453324OAI: oai:DiVA.org:uu-95261DiVA: diva2:169409
Available from: 2006-12-15 Created: 2006-12-15 Last updated: 2017-12-14Bibliographically approved
In thesis
1. MuSK Antibody(+) Versus AChR Antibody(+) Myasthenia Gravis: Clinical, Neurophysiological and Morphological Aspects
Open this publication in new window or tab >>MuSK Antibody(+) Versus AChR Antibody(+) Myasthenia Gravis: Clinical, Neurophysiological and Morphological Aspects
2007 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Myasthenia gravis (MG) is an autoimmune neuromuscular disorder that causes fluctuating muscle weakness. MG may be divided into an ocular form and a generalized form based on the involved muscles. Treatment differs between these different MG forms. The majority (80%) of patients with generalized MG are seropositive for antibodies against the acetylcholine receptor (AChR-Ab). Recently a new antibody was detected against muscle specific tyrosine kinase (MuSK) in about 40% of patients who are AChR-Ab seronegative. A few patients with MuSK-Abs have muscular atrophies, as well as electrophysiological myopathy.

In this thesis we have characterized MuSK-Ab seropositive [MuSK(+)] patients using clinical parameters, including health-related quality of life (hrQoL), neurophysiology and muscle morphology, and compared them to patients with and without AChR-Abs. The question concerned which factors contribute to their muscle weakness. Additionally, we wanted to determine if single-fiber electromyography (SFEMG) in a limb muscle has any predictive value for generalization of ocular MG.

Our results suggest that MuSK(+) patients more often have a myopathic electromyography pattern, although this pattern is found also in other immunological subtypes of MG. The myopathic pattern may be associated with the frequently found mitochondrial abnormalities. However, disturbed neuromuscular transmission is the primary cause of muscle weakness in the majority of MuSK(+) patients, as well as in AChR-Ab seropositive patients. The disease-specific hrQoL MG questionnaire was successfully validated into Swedish and these scores correlated with disturbed neuromuscular transmission in a proximal arm muscle. Abnormal SFEMG findings occur also in muscles outside of the facial area in ocular MG, although this is not predictive of subsequent generalization.

MuSK (+) patients have little or no beneficial effect of acetylcholine esterase inhibitors (AChEI). On the contrary AChEI may produce profound adverse effects. We present the hypothesis that this effect of AChEI is due to abnormal receptor morphology in MuSK(+) patients.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2007. 73 p.
Series
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 211
Keyword
Neurosciences, myasthenia gravis, MuSK antibody, AChR antibody, myopathy, single-fiber EMG, mitochondria, Swedish MG questionnaire, quality of life, Neurovetenskap
Identifiers
urn:nbn:se:uu:diva-7408 (URN)91-554-6752-0 (ISBN)
Public defence
2007-02-02, Grönwallssalen, Akademiska sjukhuset, ingång 70, 75185 Uppsala, 13:15
Opponent
Supervisors
Available from: 2006-12-15 Created: 2006-12-15Bibliographically approved

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Rostedt Punga, AnnaFlink, RolandAskmark, HåkanStålberg, Erik

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