• Mary Say Payne, Benjamin Lyons Brown, Jayaraman Rao, and Bryan Rankin Payne.
• Department of Neurology, Louisiana State University Medical School, New Orleans, Louisiana 70112, USA. bpayne1@lsuhsc.edu
• Neurosurgery. 2005 Apr 1; 56 (4): E868; discussion E868.

Objective And ImportancePhenylketonuria (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.Clinical PresentationA 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.InterventionA deep brain stimulation electrode was placed in the ventral intermediate nucleus of the right thalamus.ConclusionImmediately 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.

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