title: Treatment of phenylketonuria-associated tremor with deep brain stimulation: case report.
abstract: OBJECTIVE
  Phenylketonuria (PKU) is an inborn error of metabolism that causes severe neurological impairment, despite dietary treatment. We present a case of PKU-induced cerebellar tremor treated with deep brain stimulation. There have been no previously reported cases of a patient with a PKU tremor treated with deep brain stimulation.
  
  
  METHODS
  A 36-year-old male patient with PKU presented with signs of cerebellar disease including dysmetria, resting tremor, and intention tremor in the left upper extremity.
  
  
  METHODS
  A deep brain stimulation electrode was placed in the ventral intermediate nucleus of the right thalamus.
  
  
  CONCLUSIONS
  Immediately after surgery, the patient had nearly complete resolution of intention tremor in the left arm. His resting tremor in the left hand was also greatly improved. The 30-month follow-up examination revealed maintenance of the immediate postoperative improvement.
authors.affiliation: Department of Neurology, Louisiana State University Medical School, New Orleans, Louisiana 70112, USA. bpayne1@lsuhsc.edu
authors.forename: Mary Say
authors.initials: MS
authors.lastname: Payne
authors.forename: Benjamin Lyons
authors.initials: BL
authors.lastname: Brown
authors.forename: Jayaraman
authors.initials: J
authors.lastname: Rao
authors.forename: Bryan Rankin
authors.initials: BR
authors.lastname: Payne
mesh_terms: D000328:Adult; D046690:Deep Brain Stimulation; D006801:Humans; D008297:Male; D010661:Phenylketonurias / Q000150:complications*; D014202:Tremor / Q000209:etiology / Q000601:surgery / Q000628:therapy*