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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

Cognitive and psychiatricfeatures of movementdisorders in children

N Nardocci

Fondazione IRCCS Istituto Neurologico“CBesta”


limitation to the review
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
sydenham chorea cognitive aspects
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)
sydenham chorea psychiatric aspects
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)
benign hereditary chorea
  • 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)
tourette syndrome
  • 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

opsoclonus myoclonus syndrome
  • 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)
wilson disease
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)
primary and dyt1 dystonia
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)
dystonia plus syndromes
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)
  • 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
dyt11 dystonia scge gene mutations
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)