Genotypic Differences in Networks Supporting Regional Predictors of Speech Rate in Spinocerebellar Ataxia:...

Disordered speech production, dysarthria, is a common characteristic of the spinocerebellar ataxias (SCAs). Although dysarthric features differ across SCAs, a previous analysis revealed that a combination of regional cerebral blood flow (rCBF) in the left inferior frontal region and the right caudate predicted syllable rate, a pattern reported in normal speakers. This study examined the relationships between primary predictor brain regions and other areas of the brain in three SCA groups. The regions associated with the primary predictors are considered as elements of secondary networks since they are associated with regional speech predictors rather than directly with speech performance. Speech and rCBF data from 9 SCA1, 8 SCA5, and 5 SCA6 individuals were analyzed. Partial correlations were used to identify brain regions associated with the primary predictors. Secondary networks differed across SCA genotypes. SCA1 and SCA6 demonstrated both positive and negative associations between primary and secondary areas, whereas the associations in the SCA5 genotype were only positive. The SCA5 associations were also largely bilaterally symmetrical. Both SCA1 and SCA5 demonstrated secondary associations with the right caudate, whereas the SCA6 group had no such associations. These results demonstrate that although primary aspects of a brain network may remain functional, pathophysiological processes associated with different SCA genotypes may express themselves in alterations of broader, secondary brain networks. These secondary networks may reflect generic functional associations with the primary predictor regions, compensatory activity in the presence of an SCA, SCA pathology, or some combination of these factors. Impact statement This study demonstrates that although the primary predictors of speech rate in the brain are shared in normal speakers and three genotypes of ataxia, the genotypes differ from each other in broader activity patterns associated with the primary predictors. One implication is that although basic neural circuitry may remain functional for some period of time in progressive neurological disorders, abnormal relationships may exist in the broader neurological context in which they operate. These results also serve as a reminder that patterns of brain activity reflect involvement in the external world as well as the brain's response to itself.