Figure 3.
Correlations between dystonia and cognition. Dystonia was rated with the BFM dystonia rating scale, with mild motor deficits scored as mild expressions of dystonia. Scores for patients with LND come from our previous study (Jinnah et al., 2006) while those for variants come from Tables 1 and 2. Cognition was assessed with IQ, which was taken from the results of clinical diagnostic testing or previous publications. Patient subgroups are LND (circles), HPRT-related neurological dysfunction (squares), and HPRT-related hyperuricaemia (triangles). Patients with LND and HPRT-related neurological dysfunction were distinguished by the presence of self-injurious behaviour. The HPRT-related hyperuricaemia group was defined as clinically insignificant motor dysfunction with a BFM score of 5 or less. There was a significant negative correlation between BFM and IQ scores (Spearman rho = −0.64, P < 0.001). This correlation remained after controlling for age (Spearman rho = −0.63, P < 0.001). There was no significant correlation between BFM and age (Spearman rho = 0.20, P = 0.21).