FMR1 alleles in Parkinson's disease: relation to cognitive decline and hallucinations, a longitudinal study.
Carriers of expanded alleles of the fragile X mental retardation (FMR1) gene may display parkinsonism, cognitive decline, and behavioral changes. The authors screened 2 male groups of patients affected with Parkinson's disease (PD) (n = 137). One group (n = 56) was followed longitudinally for up to 12 years. Length of CGG repeats in PD patients was compared with healthy controls (n = 310). In addition, the association of the number of CGG repeats with cognitive decline or hallucinations was studied in the longitudinally followed PD group. The authors found no repeats in the premutation range (55-200 CGG repeats) and no significant difference in the proportion of intermediate-size (41-54 CGG repeats) carriers between the PD and the control groups. Using linear regression, the number of CGG repeats was not related to motor or cognitive progression. However, the marked cognitive decline in 2 patients carrying intermediate-size alleles points to a possible association. More studies with larger PD samples are warranted.
Kurz
Martin Wilhelm
MW
Department of Neurology, Heinrich-Heine-University, Düsseldorf, Germany. kurzmartin@gmx.net
Schlitter
Anna Melissa
AM
Klenk
Yvonne
Y
Mueller
Thomas
T
Larsen
Jan Petter
JP
Aarsland
Dag
D
Dekomien
Gabriele
G
D000328:Adult; D000368:Aged; D000483:Alleles; D003072:Cognition Disorders / Q000175:diagnosis / Q000235:genetics*; D004252:DNA Mutational Analysis; D003704:Dementia / Q000175:diagnosis / Q000235:genetics*; D051860:Fragile X Messenger Ribonucleoprotein 1 / Q000235:genetics*; D006580:Genetic Carrier Screening; D006212:Hallucinations / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D010300:Parkinson Disease / Q000175:diagnosis / Q000235:genetics*; D013223:Statistics as Topic; D018911:Trinucleotide Repeats
D051860:Fragile X Messenger Ribonucleoprotein 1; C497683:FMR1 protein, human