Use of the frontal assessment battery in evaluating executive dysfunction in patients with Huntington’s disease

• Published in: Journal of neurology Vol. 256; no. 11; pp. 1809 - 1815
• Main Authors: Rodrigues, Guilherme Riccioppo, Souza, Carolina Pinto, Cetlin, Roberto Satler, de Oliveira, Daniel Sabino, Pena-Pereira, Marcio, Ujikawa, Liliana Tiemi, Marques, Wilson, Tumas, Vitor
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer-Verlag 01-11-2009; Springer; Springer Nature B.V
• Subjects: Adolescent; Adult; Age Factors; Aged; Alzheimer's disease; Analysis of Variance; Behavior; Biological and medical sciences; Brain damage; Cognition Disorders - diagnosis; Cognition Disorders - etiology; Cognitive ability; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Educational Status; Executive function; Executive Function - physiology; Female; Humans; Huntington Disease - complications; Huntingtons disease; Longitudinal Studies; Male; Medical sciences; Medicine; Medicine & Public Health; Mental Status Schedule; Middle Aged; Neurology; Neuropsychological Tests; Neuroradiology; Neurosciences; Original Communication; Regression Analysis; Reproducibility of Results; ROC Curve; Validity; Young Adult; Executive function; Huntington’s disease; Frontal assessment battery; Human; Nervous system diseases; Cognitive disorder; Huntington disease; Huntington's disease; Genetic disease; Cerebral disorder; Central nervous system disease; Frontal; Degenerative disease; Extrapyramidal syndrome
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-009-5197-0

The frontal assessment battery (FAB) is a bedside cognitive scale designed to measure executive functions. Huntington’s disease (HD) is a neurodegenerative disorder characterized by motor, behavioral, and cognitive dysfunction. The aim of this study was to check the validity of the FAB for the evaluation of cognitive impairment in patients with HD. Forty-one patients diagnosed with HD and 53 healthy controls matched by education, sex and age were evaluated with a validated Brazilian version of the UHDRS, the VFT, the SDMT, the SIT, the MMSE, and the FAB. The diagnosis of HD was made by DNA analysis. FAB scores were lower in patients than in the controls (
p
 < 0.001) and had significant correlations with the VFT (
r
 = 0.79;
p
 < 0.05), the SDMT (
r
 = 0.80;
p
 < 0.05), the SIT (
r
 = 0.72;
p
 < 0.05), the MMSE (
r
 = 0.83;
p
 < 0.05), the FCS (
r
 = 0.79;
p
 < 0.05) and the motor section of the UHDRS (
r
 = −0.80;
p
 < 0.05). The FAB differentiated between HD patients in the initial and later stages of the disease. The one-year longitudinal evaluation revealed a global trend toward a worsening in the second score of the FAB. The results demonstrate that the FAB presents good internal consistency and also convergent and discriminative validity; therefore it is a useful scale to assess executive functions and to evaluate cognitive impairment in patients with HD.