Hereditary paroxysmal ataxia with neuromyotonia

Authors

  • J. Vaamonde,

    1. Movement Disorders Unit and Neurophysiology Service, Department of Neurology, Clinica Universitatia, Pamplona, Spain
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  • J. Artieda,

    1. Movement Disorders Unit and Neurophysiology Service, Department of Neurology, Clinica Universitatia, Pamplona, Spain
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  • Dr. J. A. Obeso

    Corresponding author
    1. Movement Disorders Unit and Neurophysiology Service, Department of Neurology, Clinica Universitatia, Pamplona, Spain
    • Department of Neurology, Clinica Universitaria, Apdo 192, 31080 Pamplona, Spain
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Abstract

The clinical manifestations of a patient with hereditary paroxysmal ataxia and neuromyotonia are described. Generalized tremor, triggered by sudden movemnts, and spasms of hand and foot muscles were the main clinical findings. Electromyogram (EMG) and nerve blocking studies led to the diagnosis of neuromyotonia. Treatment with acetozolamide was of no therapeutic value, confirming previous observations about the difference in response of paroxysmal ataxia with and without neuromyotonia.

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