Andersen-Tawil syndrome (ATS): management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Andersen-Tawil syndrome (ATS): management. Ventricular arrhythmias, QT abnormalities, Large U waves. Fully referenced neurology checklist.

Electrocardiogram (ECG) and Holter

  • Ventricular arrhythmias
  • QT abnormalities
  • Large U waves

Muscle biopsy

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Neurological investigations

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Magnetic resonance imaging (MRI)

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Neurological treatments

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Cardiac treatments

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Acronym

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References

  1. Spillane J, Kullmann DM, Hanna MG. Genetic neurological channelopathies: molecular genetics and clinical phenotypes. JNNP 2016; 87:37-48. 
  2. Meola G, Hanna MG, Fontaine B. Diagnosis and new treatment in muscle channelopathies. JNNP 2009; 80:360-365.
  3. Rakowicz W, Hanna M. Muscle ion channel diseases. ACNR 2003; 3:14-16.
  4. Vivekanandam V, Männikkö R, Skorupinska I, et al. Andersen-Tawil syndrome: deep phenotyping reveals significant cardiac and neuromuscular morbidity. Brain 2022; 145:2108-2120.
  5. Delannoy E, Sacher F, Maury P, et al. Cardiac characteristics and long-term outcome in Andersen-Tawil syndrome patients related to KCNJ2 mutation. Europace 2013; 15:1805-1811.
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