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Löscher, WN; Wanschitz, J; Reiners, K; Quasthoff, S.
Morvan's syndrome: clinical, laboratory, and in vitro electrophysiological studies.
MUSCLE NERVE 2004 30: 157-163. Doi: 10.1002/mus.20081 (- Case Report)
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Co-Autor*innen der Med Uni Graz
Quasthoff Stefan
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Abstract:
Morvan's syndrome is a rare disorder characterized by neuromyotonia, hyperhidrosis, and central nervous system dysfunction. We report a patient with features of this syndrome, but who initially presented with breathing difficulties. Concentric needle electromyography showed an abundance of myokymic and neuromyotonic discharges. Exercise tests and repetitive nerve stimulation showed a decrement-increment response of compound muscle action potentials. Antibodies against voltage-gated potassium channels were not detected on repeated testing, but the presence of oligoclonal bands in the cerebrospinal fluid (CSF) suggested an autoimmune etiology. At follow-up over 3 years, no cancer was found. Electrophysiological in vitro studies of effects of patient serum and CSF on rat nerves provided no evidence of altered voltage-gated sodium or potassium conductances. We conclude that putative humoral factors do not block ion channels acutely but may cause channel dysfunction with chronic exposure.
Find related publications in this database (using NLM MeSH Indexing)
Action Potentials -
Adolescent -
Animals -
Autoantibodies - analysis
Electric Conductivity - analysis
Electrodiagnosis - analysis
Electromyography - analysis
Exercise Test - analysis
Humans - analysis
Male - analysis
Muscle, Skeletal - physiopathology
Myokymia - blood
Oligoclonal Bands - cerebrospinal fluid
Peroneal Nerve - physiopathology
Potassium Channels, Voltage-Gated - immunology
Rats - immunology
Sodium Channels - metabolism
Sural Nerve - physiopathology

Find related publications in this database (Keywords)
electrotonus
ion channel antibodies
Morvan's syndrome
neuromyotonia
repetitive nerve stimulation
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