Andersen-Tawil syndrome diagnostic criteria

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Diagnostic Criteria

The diagnosis of Andersen-Tawil syndrome (ATS) is suspected in individuals with either A or B:


A. Two of the following three criteria:


  • Periodic paralysis


  • Symptomatic cardiac arrhythmias or electrocardiographic (ECG) evidence of enlarged U-waves, ventricular ectopy, or a prolonged QTc or QUc interval


  • Characteristic facies, dental anomalies, small hands and feet, and at least two of the following:


    • Low-set ears


    • Widely spaced eyes


    • Small mandible


    • Fifth-digit clinodactyly


    • Syndactyly


B. One of the above three in addition to at least one other family member who meets two of the three criteria


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