Tremor and Klinefelter's Syndrome

Marcie L Rabin; Shivam Om Mittal; Bahman Jabbari

doi:10.7916/D84M93KR

Tremor and Klinefelter's Syndrome

Tremor Other Hyperkinet Mov (N Y). 2015 Jun 15:5:304. doi: 10.7916/D84M93KR. eCollection 2015.

Authors

Marcie L Rabin¹, Shivam Om Mittal², Bahman Jabbari³

Affiliations

¹ Atlantic Neuroscience Institute, Summit, NJ, USA.
² Department of Neurology, Case Western Reserve University, Cleveland, OH, USA.
³ Department of Neurology, Yale University School of Medicine, New Haven, CT, USA.

Abstract

Background: Klinefelter's syndrome (KS) has been associated with tremor, but reports on tremor phenomenology and treatment are limited.

Case reports: Patient 1 is a 17-year-old male with a dystonic tremor treated with deep brain stimulation (DBS). Patient 2 is a 57-year-old male with a predominant left hand resting tremor and dystonic features.

Discussion: Our cases suggest that the tremor in patients with KS may be dystonic in nature. Patient 1 is also the third reported case of successful treatment with DBS. These cases have implications for elucidating the underlying neurobiological mechanism of tremor and identifying treatment options.

Keywords: 47, XXY; Klinefelter’s; Tremor; deep brain stimulation (DBS); dystonia; sex chromosome.