Characteristics Of Non-motor Symptoms And Changes After Deep Brain Stimulation In Simplex Generalized Dystonia

Part 1 Non-motor symptoms in Chinese patients with isolated generalized dystoniaBackground:Previous studies have indicated that non-motor symptoms are primary problems in focal dystonia,but limited data are available about non-motor problems and their correlation with motor severity in generalized dystonia(GD).Methods:In the present study,we performed a case-control study and enrolled isolated inherited or idiopathic GD patients and age-,sex-and education-matched healthy controls(HC).Clinical characteristics,motor symptoms,non-motor problems,including psychiatric co-morbidity,sleep problems,fatigue,cognitive function and quality of life(QoL)were assessed in both groups using various rating scales and assessments.Results:Thirty-three patients with GD and 33 controls were enrolled for evaluation of NMS,among them 20 patients with GD and match controls underwent a battery of cognitive function tests.Significant higher scores on depression and anxiety(p<0.001)were shown in GD compared with HC,whereas the frequency of obsessive-compulsive disorders approached that of HC(p=0.238).Patients with GD also had significantly higher Pittsburg Sleep Quality Index(PSQI)and fatigue scores than HC,whereas no difference was observed in excessive daytime somnolence.In GD,QoL was more impaired,with statistically lower scores in both physical and mental components.Psychiatric rating scales did not correlate to motor severity or disease duration but might influence quality of sleep and fatigue.Subgroup analysis suggests non-motor manifestations differ with different etiologies in GD.Compared with HC,GD patients behaved poorer mainly in MoCA,executive function/attention,spatial ability,some tests in memory and similarities while other cognitive function did not differ between patients and HC.Some of cognitive impairment was associated with psychiatric problems and motor severity,but medication and etiologies of dystonia were likely not to influence cognitive function.Conclusion:This study suggests that psychiatric problems in GD are likely to be primary determinants not correlated to motor severity,which may also affect quality of sleep and fatigue.Impaired cognitive function may be a manifestation of GD but seems to be,to some extent,associated with motor severity and psychiatric problems.Part 2 Non-motor effects of pallidal deep brain stimulation in Chinese patients with isolated generalized dystoniaBackground:The effects and safety of pallidal deep brain stimulation(DBS)surgery on the motor aspects of dystonia have been well described,however,research investigating its impact on non-motor symptoms(NMS)is relatively lacking,especially in generalized dystonia(GD).Methods:A cohort of 12 patients with GD underwent various rating scales and assessments on clinical characteristics,BFMDRS,psychiatric co-morbidity,sleep problems,fatigue and quality of life(QoL)at baseline and 3-month after Gpi-DBS.Cognitive function changes were also assessed in 9 patients.Baseline pre-DBS measures were compared to scores 3 months after DBS.Results:There was a significant improvement of dystonia measured by BFMDRS.Patients with GD demonstrated stable performance on psychiatric disorders,sleep and most of tests in cognitive function,with statistically significant improvements noted in fatigue,PCS,GH,MoCA and FAB.Changes in PCS correlated with improvements of BFMDRS.Conclusion:Most of NMS remained stable in patients with GD in a short follow-up of Gpi-DBS.Fatigue,some tests in cognitive function,and some domains of quality of life,especially physical components improved after DBS.Improvement of PCS might be secondary to motor improvement.