Gewählte Publikation:
SHR
Neuro
Krebs
Kardio
Lipid
Stoffw
Microb
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)
Web of Science
PubMed
FullText
FullText_MUG
- Co-Autor*innen der Med Uni Graz
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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.
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Action Potentials -
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Adolescent -
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Animals -
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Autoantibodies - analysis
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Electric Conductivity - analysis
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Electrodiagnosis - analysis
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Electromyography - analysis
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Exercise Test - analysis
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Humans - analysis
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Male - analysis
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Muscle, Skeletal - physiopathology
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Myokymia - blood
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Oligoclonal Bands - cerebrospinal fluid
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Peroneal Nerve - physiopathology
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Potassium Channels, Voltage-Gated - immunology
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Rats - immunology
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Sodium Channels - metabolism
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Sural Nerve - physiopathology
- Find related publications in this database (Keywords)
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electrotonus
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ion channel antibodies
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Morvan's syndrome
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neuromyotonia
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repetitive nerve stimulation