Cardiac arrhythmia in Wilson’s disease: An oversighted and overlooked entity!

• Main Authors: Bhupender Kumar Bajaj, Ankur Wadhwa, Richa Singh, Saurabh Gupta
• Format: Article
• Language: English
• Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2016-04-01
• Series: Journal of Neurosciences in Rural Practice
• Subjects: autonomic function; cardiac arrhythmia; heart block; hepatolenticular degeneration; wilson’s disease
• Online Access: http://www.thieme-connect.de/DOI/DOI?10.4103/0976-3147.186982

Wilson’s disease is a multisystem disorder which manifests with hepatic, neurological, musculoskeletal, hematological, renal, and cardiac symptoms. The hepatic and neurological manifestations often overshadow the other system involvement including cardiac symptoms and signs, which may prove fatal. We report a case of a young female who presented with progressive parkinsonian features and dystonia for around 4 months followed 2 months later by the complaint of episodes of light-headedness. She was diagnosed to have Wilson’s disease based on the presence of Kayser–Fleischer ring and laboratory parameters of copper metabolism. Electrocardiography of the patient incidentally revealed 2nd degree Mobitz type-1 atrioventricular block explaining her episodes of light-headedness. She was started on penicillamine and trihexyphenidyl. The heart block improved spontaneously. Cardiac autonomic function tests including blood pressure response to standing and heart rate response to standing were observed to be normal. We review the literature on cardiac manifestations of Wilson’s disease and emphasize that patients with Wilson’s disease should be assessed for cardiac arrhythmia and cardiac dysfunction as these may have therapeutic and prognostic implications.