Rubral Tremor Associated with Klinefelter Syndrome; a Case Report and Literature Review

Abstract

Introduction: Previous case series suggested an association between Klinefelter syndrome (KS) and tremor, but most reported cases had ET-like tremor. Also, previous studies did not suggest any suitable treatments for the tremor. Case Presentation: The current report presented a case of KS that his rubral tremor led to the KS diagnosis at the age of 43. Regular anti-tremor medication was immediately started along with testosterone therapy; none of which were effective on the tremor. Next, a table of KS patients was provided with a tremor to elucidate the type of tremor and medical treatments in the patients. The result of the literature review of the most effective therapy for tremor in such patients showed that primidone, propranolol, gabapentin, and levetiracetam were not always effective, while deep brain stimulation of the ventral intermediate nucleus showed promising results. Conclusion: Since tremor in KS has an unknown origin, it may not be responsive to regular anti-tremor medication; therefore, deep brain stimulation (DBS) of the ventral intermediate (VIM) may be the best therapy for such patients