Neuropsychological profile of a Filipino gentleman with X-linked dystonia-parkinsonism: a case report of Lubag disease.
X-Linked Dystonia-Parkinsonism (XDP or "Lubag") is a progressive neurodegenerative disorder unique to the Island of Panay in the Philippines. Imaging and autopsy studies have suggested involvement of the caudate and putamen in late stages. Because the clinical presentation of patients with XDP resembles that of patients with Parkinson disease or dystonia, it is reasonable to predict the neuropsychological profile might be similar; however, the neuropsychological profile of a XDP patient has not previously been published. We present the neuropsychological findings of a 67-year-old gentleman with a 10-year history of XDP who presented with parkinsonian and dystonic symptoms. He was evaluated for suitability for deep brain stimulation surgery. Neuropsychological findings demonstrated diffuse impairment involving memory, visuospatial, language, and executive functioning.
Howe
Laura L S
LL
Clinical and Health Psychology, McKnight Brain Institute, University of Florida, Gainesville, FL, USA. lauralshowe@yahoo.com
Kellison
Ida L
IL
Fernandez
Hubert H
HH
Okun
Michael S
MS
Bowers
Dawn
D
D000368:Aged; D041321:Chromosomes, Human, X; D003072:Cognition Disorders / Q000209:etiology / Q000235:genetics / Q000523:psychology*; D020821:Dystonic Disorders / Q000150:complications / Q000235:genetics / Q000523:psychology*; D040181:Genetic Diseases, X-Linked / Q000150:complications / Q000235:genetics / Q000523:psychology*; D006801:Humans; D008297:Male; D009483:Neuropsychological Tests; D010300:Parkinson Disease / Q000150:complications / Q000235:genetics / Q000523:psychology*; D010679:Philippines