Neurophysiologic characterization of periodic paralysis episode in a patient with Andersen-Tawil syndrome

Primary period paralysis (PP) are rare disorders of skeletal muscle characterized by episodic attacks of muscle weakness. These episodes can be triggered by exercise and result from abnormal muscle membrane excitability. The diagnosis is based on clinical features, positive family history, serum potassium level during episodes and response to treatment. However the diagnosis is not always straightforward since some of these features may be absent (Venance et al., 2006). Post-exercise electrophysiological testing can be useful in guiding the diagnosis, as changes in compound motor action potential (CMAP) after exercise correlate with exercise induced weakness and ion channel mutation (Fournier et al., 2004).

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