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
• 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
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)
Sydenham chorea: psychiatric aspects
• Retrospective studies: 46% of patients
decribed behavioural changes with emotional
liability, depression, anxiety, irritability, ageregressed 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)
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)
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
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)
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 adolescentonset an may precede motor signs(Ullah M et
al 2009)
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)
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) (SaundersPullmann et al 2002; Nardocci 2012)
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
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)