Internal globus pallidotomy in dystonia secondary to Huntington's disease

INTRODUCTION AND METHOD: The prototypic motor feature of Huntington's disea se (HD) is chorea, but parkinsonism and involuntary movements such as dysto nia and myoclonus can also be present. Pallidotomy has been shown to be an effective treatment for medically refractory Parkinson's disease (PD). We p erformed bilateral microelectrode guided-stereocactic pallidotomies targete d at globus pallidum internus (GPi) to treat a 13-year-old patient diagnose d with Westphal variant of HD with intractable generalized dystonia and par kinsonism. RESULTS: Intraoperative microelectrode recordings of GPi cells showed a rel atively low firing rate, 29 +/- 14 fit, with most neurons showing pauses. A cutely, after surgery, limb dystonia mildly improved but trunk dystonia per sisted. Postoperative follow up 3 months later showed minimal clinical impr ovement in dystonic features with marked worsening of spasticity. CONCLUSION: In our case, bilateral pallidotomy produced modest palliative f unctional improvement in dystonic features. Cellular firing patterns were: markedly different than in PD and were similar to those found in dystonia.