Smell testing is abnormal in 'lubag' or X-linked dystonia-parkinsonism: a pilot study.
We administered a culturally corrected University of Pennsylvania Smell Identification Test (ccUPSIT) consisting of 25 odor items to 20 patients with 'Lubag' or X-linked dystonia-parkinsonism and 20 control subjects matched by sex, age, educational background, smoking history, and geographical origin. The mean ccUPSIT score of Lubag patients (18 +/- 3.19) was statistically lower (P = 0.003) than controls (20.5 +/- 3.02). The smell scores did not correlate with phenotype, severity of dystonia, or duration of disease. Nine of 20 Lubag patients (45%) had ccUPSIT scores below the mean, with the lowest score being 11. This pilot study suggests that olfactory dysfunction may occur in Lubag patients.
Evidente
Virgilio Gerald H
VG
Department of Neurology, Mayo Clinic, 13400 E. Shea Boulevard, Scottsdale, AZ 85259, USA. evidente.virgilio@mayo.edu
Esteban
Raymund P
RP
Hernandez
Jose Luis
JL
Natividad
Filipinas F
FF
Advincula
Joel
J
Gwinn-Hardy
Katrina
K
Hardy
John
J
Singleton
Amanda
A
Singleton
Andrew
A
D000328:Adult; D004421:Dystonia / Q000175:diagnosis* / Q000235:genetics*; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis* / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009812:Odorants; D010300:Parkinson Disease / Q000175:diagnosis* / Q000235:genetics*; D010865:Pilot Projects; D012903:Smell / Q000235:genetics*