title: What is the impact of education on Huntington's disease?
abstract: Huntington's disease (HD) is a neurodegenerative disease caused by a cytosine adenosine guanine (CAG) expansion in the huntingtin gene. The length of the triplet repeat is the most important factor in determining age of onset and the severity of the disease, but substantial variability of these parameters is attributed to other factors. To investigate the relationship between the years of education and the age at onset and the severity of the phenotype in patients with HD, we applied multiple linear regression analysis to examine the impact of education on the age at onset and the severity of the clinical scores assessed by the Unified Huntington's Disease Rating Scale (UHDRS) of 891 patients with HD from the multinational observational study "Registry" conducted by the European Huntintgton's Disease Network. The model was adjusted for CAG repeat length and age at the time of assessment. Patients with lengthier education exhibited earlier estimated age at onset but less severe clinical scores (motor = -3.6, P = 0.006; cognitive = 27.0, P < 0.001; behavioral = -3.0, P < 0.001; and functional capacity = 1.1 points, P < 0.001) than those with shorter education, after controlling for age and number of CAG repeats. These differences persisted throughout all quartiles of disease severity. An earlier recognition of symptoms and manifestations among the more educated patients could explain the earlier estimated age at onset in this group. The link between better clinical UHDRS scores and higher education might reflect a beneficial effect of education or its covariates on the course of HD.
authors: Department of Neurology, Hospital Ramon y Cajal, Madrid, Spain.
authors: Jose Luis
authors: JL
authors: López-Sendón
authors: Ana
authors: A
authors: Royuela
authors: Patricia
authors: P
authors: Trigo
authors: Michael
authors: M
authors: Orth
authors: Herwig
authors: H
authors: Lange
authors: Ralf
authors: R
authors: Reilmann
authors: Jennifer
authors: J
authors: Keylock
authors: Hugh
authors: H
authors: Rickards
authors: Silvia
authors: S
authors: Piacentini
authors: Ferdinando
authors: F
authors: Squitieri
authors: Bernhard
authors: B
authors: Landwehrmeyer
authors: Marie-Noelle
authors: MN
authors: Witjes-Ane
authors: Caroline K
authors: CK
authors: Jurgens
authors: Raymund A C
authors: RA
authors: Roos
authors: Victor
authors: V
authors: Abraira
authors: Justo G
authors: JG
authors: de Yébenes
mesh_terms: D000328:Adult; D017668:Age of Onset; D003072:Cognition Disorders / Q000209:etiology; D004522:Educational Status; D005192:Family Health; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000235:genetics / Q000523:psychology; D008297:Male; D001523:Mental Disorders / Q000209:etiology; D008875:Middle Aged; D010641:Phenotype; D012044:Regression Analysis; D012720:Severity of Illness Index; D019680:Trinucleotide Repeat Expansion / Q000235:genetics*