assess the natural progression of cognitive impairment in Huntington's disease (HD) and to reveal factors that may mask this
Although numerous cross-sectional studies reported cognitive deterioration
at different stages of the disease, progressive cognitive deterioration has been, up to now, difficult to demonstrate in neuropsychological
The authors assessed 22 patients in early stages of HD at yearly
intervals for 2 to 4 years (average, 31.2 +/- 10 months), using a comprehensive neuropsychological battery based on the Core
Assessment Program for
Intracerebral Transplantation in Huntington's Disease (CAPIT-HD).
The authors observed a significant decline in different cognitive
functions over time: these involved primarily attention and executive functions but also involved language comprehension,
and visuospatial immediate memory.
Episodic memory impairment that was already present at the time of enrollment
show significant decline. The authors found a significant retest effect at the second assessment in many tasks.
Many attention and executive tasks adequately assess the
progression of the disease at an early stage. For other functions, the overlapping of retest effects and disease progression
may confuse the results. High interindividual and intraindividual variability seem to be hallmarks of the disease.
Neurology 2001 Apr 24;56(8):1052-8 (ISSN: 0028-3878) Bachoud-Levi AC;
Maison P; Bartolomeo P; Boisse MF; Dalla Barba G;Ergis AM; Baudic S; Degos JD; Cesaro P; Peschanski M Departement de Neurosciences
Medicales, CHU Henri Mondor, Creteil, France. firstname.lastname@example.org