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First case of X‐linked dystonia‐parkinsonism (“Lubag”) to demonstrate a response to bilateral pallidal stimulation
Author(s) -
Evidente Virgilio Gerald H.,
Lyons Mark K.,
Wheeler Mark,
Hillman Renee,
Helepolelei Luann,
Beynen Froukje,
Nolte Dagmar,
Müller Ulrich,
Starr Philip A.
Publication year - 2007
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/mds.21420
Subject(s) - dystonia , deep brain stimulation , parkinsonism , refractory (planetary science) , rating scale , medicine , stimulation , anesthesia , psychology , surgery , parkinson's disease , disease , psychiatry , developmental psychology , astrobiology , physics
Abstract “Lubag” or X‐linked dystonia‐parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45‐year‐old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation (DBS) surgery. The patient started to exhibit improvement on initial programming, most notably of his severe jaw‐opening dystonia. At 1‐year follow‐up, his Burke‐Fahn‐Marsden dystonia score and motor Unified Parkinson's Disease Rating Scale score were improved by 71% and 62%, respectively, with the stimulators on compared to stimulators off state. Bilateral pallidal DBS may be a viable option for Lubag patients with medically refractory symptoms. © 2007 Movement Disorder Society