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Hereditary neuromyotonia: a mouse model associated with deficiency or increased gene dosage of the PMP22 gene
  1. KLAUS V TOYKA,
  2. JÜRGEN ZIELASEK,
  3. KENNETH RICKER
  1. Department of Neurology, Julius-Maximilians-Universität, Würzburg, Germany
  2. Institute of Cell Biology, Swiss Federal Institute of Technology (ETH), Zürich, Switzerland
  1. Professor Klaus V Toyka, Department of Neurology, Julius-Maximilians-University, Josef-Schneider-Strasse 11, D-97080 Würzburg, Germany. Telephone (+) 931-201-5751; fax (+) 931-201-3546.
  1. KATRIN ADLKOFER,
  2. UELI SUTER
  1. Department of Neurology, Julius-Maximilians-Universität, Würzburg, Germany
  2. Institute of Cell Biology, Swiss Federal Institute of Technology (ETH), Zürich, Switzerland
  1. Professor Klaus V Toyka, Department of Neurology, Julius-Maximilians-University, Josef-Schneider-Strasse 11, D-97080 Würzburg, Germany. Telephone (+) 931-201-5751; fax (+) 931-201-3546.

https://doi.org/10.1136/jnnp.63.6.812

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    EMG recordings from a 14 month old mouse homozygously deficient for the expression of the peripheral myelin protein PMP22. The mouse was anaesthetised with Hypnorm (Janssen, Beerse, Belgium) and investigated using a Tönnies electromyograph with a concentric needle electrode (Medelec DFC25, 0.3 mm diameter, recording area 0.019 mm2). (A) Multiplet discharges in the small foot muscles. (B) Representative recording of myokymic discharges and doublets from another mouse (PMP22 -/-). There was abundant myokymic motor activity, but no voluntary limb movements during these recordings.

    Neuromyotonia is characterised by increased muscle stiffness caused by hyperactivity of motor units. Clinical characteristics are increased stiffness and cramping on intended muscle contraction, slowed …

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