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Internal globus pallidotomy in dystonia secondary to Huntington's disease
Author(s) -
Cubo Esther,
Shan Kathleen M.,
Penn Richard D.,
Kroin Jeffrey S.
Publication year - 2000
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/1531-8257(200011)15:6<1248::aid-mds1029>3.0.co;2-q
Subject(s) - pallidotomy , dystonia , parkinsonism , chorea , myoclonus , medicine , refractory (planetary science) , spasticity , rhizotomy , neurological disorder , movement disorders , globus pallidus , parkinson's disease , psychology , deep brain stimulation , central nervous system disease , neuroscience , anesthesia , surgery , basal ganglia , disease , central nervous system , biology , astrobiology , spinal cord
AND METHOD The prototypic motor feature of Huntington's disease (HD) is chorea, but parkinsonism and involuntary movements such as dystonia and myoclonus can also be present. Pallidotomy has been shown to be an effective treatment for medically refractory Parkinson's disease (PD). We performed bilateral microelectrode guided‐stereotactic pallidotomies targeted at globus pallidum internus (GPi) to treat a 13‐year‐old patient diagnosed with Westphal variant of HD with intractable generalized dystonia and parkinsonism. RESULTS Intraoperative microelectrode recordings of GPi cells showed a relatively low firing rate, 29 + 14 Hz, with most neurons showing pauses. Acutely, after surgery, limb dystonia mildly improved but trunk dystonia persisted. Postoperative follow up 3 months later showed minimal clinical improvement in dystonic features with marked worsening of spasticity. CONCLUSION In our case, bilateral pallidotomy produced modest palliative functional improvement in dystonic features. Cellular firing patterns were markedly different than in PD and were similar to those found in dystonia.