Selective Procedural Memory Impairment but Preserved Declarative Memory in Spinocerebellar Ataxia Type 3

Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, is an autosomal dominant neurodegenerative disorder that affects mainly the cerebellum and less other brain areas. While the ataxic/motor features of the disease have been well described, the cognitive consequences of the degeneration require additional testing. The aim of this study was to evaluate learning abilities in SCA3. We tested 13 SCA3 patients and 14 age-matched healthy controls, all of Yemenite origin, on a neuropsychological battery of procedural and declarative memory tests. SCA3 patients demonstrated impaired sequence learning on the procedural Serial Reaction Time test (SRTt) but normal learning on the procedural Weather Prediction Probabilistic Classification test (WPPCt). SCA3 patients showed normal learning on the declarative Rey Auditory Verbal Learning test (Rey-AVLt). The correlations between the learning measures of the SRTt, WPPCt, and Rey-AVLt tests in SCA3 and controls separately were not significant. These results imply that the cerebellar degeneration in SCA3 causes selective impairment in procedural sequence learning while the procedural probabilistic learning and declarative memory were mostly preserved. These findings support the assumption that procedural learning is not a homogeneous function and could be dissociated in cerebellar neurodegenerative disease.