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Cognitive and psychiatric features of movement disorders in children

Cognitive and psychiatric features of movement disorders in children. N Nardocci Fondazione IRCCS Istituto Neurologico“ C Besta ” Milano. Limitation to the review. Small number of the study including small number of participants

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Cognitive and psychiatric features of movement disorders in children

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  1. Cognitive and psychiatricfeatures of movementdisorders in children N Nardocci Fondazione IRCCS Istituto Neurologico“CBesta” Milano

  2. Limitationto the review • Smallnumberof the studyincludingsmallnumberofparticipants • Oftenstudiesnotbased on standardized cognitive and psychiatricassessment • Definition of behavioural or psychiatricsymptomsnotuniform • Difficulties in interpretating the cognitive and psychiatricmanifestationas the resultof the diseaseitself or as a consequenceoftherapy • Findings are presentedasprevalencewithoutindicating the severityofdisability • In none the assessment include the impact on quality of life

  3. Sydenhamchorea: cognitive aspects • Lowerscores on WISC-R and impairedverbalfluency (Swedo FE et al 1993; Cunningham t al 2006) • ADHD reported in up 60% ofchildrenwithpersistentchorea (Maia DP et al 2005) • ADHD symptomsreportedbefore the appearanceofchorea (Ridel Kl et al 2010)

  4. Sydenhamchorea: psychiatricaspects • Retrospectivestudies: 46% ofpatientsdecribedbehaviouralchangeswithemotionalliability, depression, anxiety, irritability, age-regressedbehaviour (Zomorrodi A et al 2006; Swedoet al 1993; Ridelet al 2010) • Prospectivestudies: obsessive-compulsivesymptoms (70-81%); OCD (17-21%). (Swedoel al 1993;Ashbar et al 1998)

  5. BenignHereditarychorea • Low-average IQ reported in isolatedcases • No reportsof cognitive decline (Schrag A et al 2000; Kleiner-FismanGet al 2007) • Adultonsetpsychosisreported in severalfamilies (Kleiner-FismannGet al 2007)

  6. Tourettesyndrome • ADHD (up to 70%) • OC symptoms (up to 30%) • OCD ( up to 26%) • Separationanxiety (14%) • Bipolardisorders (11%) • Depression (2-9%) • Schizophrenia (3%) • Pervasive developmentaldisorders (5%) (Ganizadeh A et al 2009; BundLet al 2009; Robertson 2006; Denckla MB 2006; RoessnerV 2007

  7. Opsoclonus-myoclonussyndrome • Cognitive impairment, lowerintellectualperformances (60-80%) and behaviouralproblems (17-90%). (Hammeret al 1995; Tate et al 2006; Turkelet al 2006; De Grandiset al 2009) • Behaviouraldisturbances: rageattacks, OC symptoms, Hyperactivity, Depression and ADHD (Tate et al 2006)

  8. Wilson disease • Psychiatricsymptoms up 50% ofadultsbefore treatment. (Shanmugiah A et al 2008) • Psychiatricsymptomsseems to occur with a higherpercentage (60%) in the adolescent-onsetan may precede motor signs(Ullah M et al 2009)

  9. Primary and DYT1 dystonia • No data referring on cognitive and psychiatricaspects in children • Anxiety or depressionbefore the onsetof motor sign in 23% among a groupofyoung people withPrimarydystonia (aged 3-28 yrs). (KoukouniVet al 2007) • Depressionhasbeenidentified in a seriesofasynptomatic DYT1 carrier (Heimann GA et al, 2004)

  10. Dystonia plus syndromes • Learning disability and depressionhavebeenreported in some families affected by DTY5 Dystonia (Hoffmann GF et al 2003; Hahn H et al 2001) • OCD usuallyappearingafter the motor symptomsdepression and anxiety in myoclonusdystonia (DYT11) (Saunders-Pullmannet al 2002; Nardocci 2012)

  11. Conclusions • Existinginformationssuggestthat non motor deficitsdifferaccording with age, severity and progression of the disease • Non motor symptoms in childrenwith MD may cause major disability • The cognitive and psychiatricmanifestationsmayresultfrom the diseaseitself or maybe a consequenceoftherapy • Theirrecognitionismandatory and may facilitate management and the treatment requiresclinical expertise • ADHD hasbeenreportedas a major comorbidity in Tourettesyndrome • OCD ismanifest in Sydenhamchorea, Tourettesyndrome and myoclonusdystonia • Cognitive and behaviouralproblems are typical of opsoclonus-myoclonussyndrome • Depression and anxiety are more frequent in geneticdystonia

  12. DYT11 Dystonia (SCGE gene mutations) • No evidence of cognitive defects in children • OCD mayappearafter the onsetof motor symptoms in childhood (Saunders-Pullmannet al 2002; Nardocci 2012)

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