1. Cognitive and psychiatric features of movement disorders in children N Nardocci Fondazione IRCCS Istituto Neurologico“C Besta” Milano

2. Limitation to the review Small number of the study including small number of participants Often studies not based on standardized cognitive and psychiatric assessment Definition of behavioural or psychiatric symptoms not uniform Difficulties in interpretating the cognitive and psychiatric manifestation as the result of the disease itself or as a consequence of therapy Findings are presented as prevalence without indicating the severity of disability In none the assessment include the impact on quality of life

4. Sydenham chorea: cognitive aspects Lower scores on WISC-R and impaired verbal fluency (Swedo FE et al 1993; Cunningham t al 2006) ADHD reported in up 60% of children with persistent chorea (Maia DP et al 2005) ADHD symptoms reported before the appearance of chorea (Ridel Kl et al 2010)

5. Sydenham chorea: psychiatric aspects Retrospective studies: 46% of patients decribed behavioural changes with emotional liability, depression, anxiety, irritability, age- regressed behaviour (Zomorrodi A et al 2006; Swedo et al 1993; Ridel et al 2010) Prospective studies: obsessive-compulsive symptoms (70-81%); OCD (17-21%). (Swedo el al 1993;Ashbar et al 1998)

7. Benign Hereditary chorea Low-average IQ reported in isolated cases No reports of cognitive decline (Schrag A et al 2000; Kleiner-Fisman G et al 2007) Adult onset psychosis reported in several families (Kleiner-Fismann G et al 2007)

8. Tourette syndrome ADHD (up to 70%) OC symptoms (up to 30%) OCD ( up to 26%) Separation anxiety (14%) Bipolar disorders (11%) Depression (2-9%) Schizophrenia (3%) Pervasive developmental disorders (5%) (Ganizadeh A et al 2009; Bund L et al 2009; Robertson 2006; Denckla MB 2006; Roessner V 2007

9. Opsoclonus-myoclonus syndrome Cognitive impairment, lower intellectual performances (60-80%) and behavioural problems (17-90%). (Hammer et al 1995; Tate et al 2006; Turkel et al 2006; De Grandis et al 2009) Behavioural disturbances: rage attacks, OC symptoms, Hyperactivity, Depression and ADHD (Tate et al 2006)

10. Wilson disease Psychiatric symptoms up 50% of adults before treatment. (Shanmugiah A et al 2008) Psychiatric symptoms seems to occur with a higher percentage (60%) in the adolescent- onset an may precede motor signs(Ullah M et al 2009)

16. Primary and DYT1 dystonia No data referring on cognitive and psychiatric aspects in children Anxiety or depression before the onset of motor sign in 23% among a group of young people with Primary dystonia (aged 3-28 yrs). (Koukouni V et al 2007) Depression has been identified in a series of asynptomatic DYT1 carrier (Heimann GA et al, 2004)

17. Dystonia plus syndromes Learning disability and depression have been reported in some families affected by DTY5 Dystonia (Hoffmann GF et al 2003; Hahn H et al 2001) OCD usually appearing after the motor symptoms depression and anxiety in myoclonus dystonia (DYT11) (Saunders- Pullmann et al 2002; Nardocci 2012)

18. Conclusions Existing informations suggest that non motor deficits differ according with age, severity and progression of the disease Non motor symptoms in children with MD may cause major disability The cognitive and psychiatric manifestations may result from the disease itself or may be a consequence of therapy Their recognition is mandatory and may facilitate management and the treatment requires clinical expertise ADHD has been reported as a major comorbidity in Tourette syndrome OCD is manifest in Sydenham chorea, Tourette syndrome and myoclonus dystonia Cognitive and behavioural problems are typical of opsoclonus-myoclonus syndrome Depression and anxiety are more frequent in genetic dystonia

22. DYT11 Dystonia (SCGE gene mutations) No evidence of cognitive defects in children OCD may appear after the onset of motor symptoms in childhood (Saunders-Pullmann et al 2002; Nardocci 2012)