title: Tremor associated with Klinefelter syndrome--a case series and review of the literature.
abstract: BACKGROUND
  Previous case series suggested a link between Klinefelter syndrome (KS) and essential tremor (ET) or an ET-like syndrome.
  
  
  METHODS
  We investigated three KS-patients with tremor including tremor-analyzes and discuss our data in context to findings from a literature review. The clinical outcome after deep brain stimulation (DBS) is also reviewed.
  
  
  RESULTS
  Tremor in KS is predominantly a postural and kinetic tremor that resembles ET. Our patients were further characterized by absent family history for tremor in first degree relatives, lack of subjective alcohol responsiveness inquired by history, and tremor onset in childhood. One of our patients and two cases from literature improved after DBS of the ventral intermediate nucleus (VIM) of the thalamus.
  
  
  CONCLUSIONS
  Tremor in KS shares several features with ET. If other characteristics such as family history, alcohol responsiveness, and age at tremor onset may serve as discriminating factors from ET, needs to be further investigated. First observations suggest that VIM-DBS may be efficacious.
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria.
authors.forename: Mariella
authors.initials: M
authors.lastname: Koegl-Wallner
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria.
authors.forename: Petra
authors.initials: P
authors.lastname: Katschnig-Winter
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria.
authors.forename: Tamara
authors.initials: T
authors.lastname: Pendl
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria.
authors.forename: Barbara
authors.initials: B
authors.lastname: Melisch
authors.affiliation: Department of Neurosurgery, Medical University of Graz, Graz, Austria.
authors.forename: Martin
authors.initials: M
authors.lastname: Trummer
authors.affiliation: Department of Neurosurgery, Medical University of Graz, Graz, Austria.
authors.forename: Etienne
authors.initials: E
authors.lastname: Holl
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Werner
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria.
authors.forename: Reinhold
authors.initials: R
authors.lastname: Schmidt
authors.affiliation: Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, 8036 Graz, Austria. Electronic address: petra.schwingenschuh@medunigraz.at.
authors.forename: Petra
authors.initials: P
authors.lastname: Schwingenschuh
mesh_terms: D000328:Adult; D006801:Humans; D007713:Klinefelter Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D014202:Tremor / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D055815:Young Adult
keywords: DBS; Deep brain stimulation; Essential tremor; Klinefelter syndrome; Tremor analysis