This document discusses the cognitive and psychiatric phenotypes associated with several pediatric movement disorders. It provides examples of children who presented with psychiatric symptoms like irritability, temper tantrums, and psychosis but were ultimately diagnosed with physical conditions causing their movements disorders like hemichorea, opsoclonus-myoclonus syndrome, Tourette syndrome, Wilson's disease, and restless leg syndrome. Many movement disorders are associated with long-term cognitive and psychiatric comorbidities like low IQ, behavioral issues, anxiety, and depression. Proper diagnosis and treatment of the underlying physical condition can help address both motor symptoms and comorbid mental health issues.

1. Cognitive and psychiatric
phenotypes of movement
disorders in children
Hilla Ben-Pazi, MD
Movement Disorders Clinic,
Neuropediatric Unit,
Shaare Zedek Medical Center,
Jerusalem, Israel

2. 13 year old child Tonsillitis
Temper tantrums
ADHD?
Referred : hemichorea
Haloperidol treatment
Insects crawling
Haloperidol –
discontinued
Psychosis:
Heard voices
Locked doors
Lab: Couldn’t stay alone
 ^Antistreptolysin titer
 Mild aortic & mitral regurgitation

3. Basal Ganglia: Schematic slices
Caudal Rostral
striatum striatum
Striatal = Caudate+ Putamen

4. Basal ganglia- Dual processing
Parallel and integrative processing of
motor cognitive and psychiatric pathways
Parallel striatum
Emotional processing
Motivation
Cognitive & executive
function
Motor planning
Motor execution
Integrative:
Emotional -> cognitive -> Substantia nigra- Midbrain
motor
Haber, 2003

5. Opsoclonus-myoclonus syndrome (OMS)
infantile autoimmune disorder
 Presentation:
 Irritability
 Sleep disturbance
 Speech difficulty
 Long term
 13/14 speech impairments Immuno-modulatory
therapy
 Verbal fluency
 Articulation
 50% understandable
Tate 2005; Papero 1995

6. Long term OMS
60-80% cognitive impairment
 IQ 50 – 70
 >50% special education
 Psychiatric symptoms most disruptive:
 Irritability speech
 Emotional labiality behavior
 ODD (65%)
 Obsessive or compulsive symptoms (58%)
 Poor affective regulation (10/17)
Behavioral problems and low cognitive function
- inconsistent correlation
- poor regardless of early treatment
Tate 2005; Papero 1995; Hayward 2001

7. Tourette syndrome and tics
repeated, intermittent movements, briefly suppressible and
usually associated with premonitory urge.
Emotional comorbidities
 extensively studied  Compulsions
 more disturbing than tics  Touching
 ADHD (70%)  Palms
 ODD (30%)  Counting
 OCD (26%)
 Obsessions
 Separation anxiety (14%)
 Somatosensory
 Bipolar disorder (11%)
 Depression (2-9%)  Symmetry
 PDD (5%)  Concern with appearance
 Schizophrenia (3%)
Kurlan 2002; Miguel 1997

8. 17 y/o girl deterioration in school
performance
 General weakness
 Psychiatric evaluation
 Psychological problems
 She couldn’t write
 Tremor, difficulty eating
 Referred to a neurologist
 Bradykinesia, rigidity
 No Kayser- Fliesher ring
 ^ urinary Cu
 Low ceruloplasmin
 Liver Bx-> ^Cu accumulation
T2 Hyperintensity- Caudate & Putamen

9. Wilson disease malfunction of the copper-
transporting adenosine triphosphatase
Cognitive impairments are common
 time of onset is not certain
Psychiatric symptoms
 30-50% of adults prior to diagnosis
 >50% of children (n=96)
 Depression & suicidal ideation
 Anxiety
 Bipolar affective disorder
 Excessive talkativeness
 Apathy
 Multiplicity of signs -> different neuroanatomical sites
Machado 2006; Ullah, 2009

10. Restless Legs Syndrome
characterized by a desire to relieve leg discomfort
by movement at night
 A 6-year-old girl was referred for ADHD
 At night, she would fall off her bed without waking
 Polysomnogram: intermittent leg movements
 Iron supplementation
 improved sleep
 not in ADHD
 -> methylphenidate

11. Restless Legs Syndrome is common (2%)
90% ADHD
 Correlates with RLS
 Not with sleep fragmentation
 Anxiety / depression
Hyperactivity
 Iron supplementation
 Improves RLS
 Not ADHD
 Dopaminergic treatment
 Improves both RLS & ADHD
Leg movements
Chervin, 2002; Picchietti 2008; Miller , 2000

12. Psychiatric side effects – Mov dis drugs
Mov Dis Drug Mechanism Side effect
Chorea Tetrabenazine D depletion Depression
Haloperidol D antagonist Lethargy
Myoclonus Clonazepam Enhancing  Cog,
GABA-A recp Irritability
Tremor Propranonol β blocker Hallucinations
Tics Clonidine α 2 agonist Drowsiness
Dystonia Trihexyphenidyl Anti Ach Anxiety
Confusion
L-Dopa D precursor Depression,
Anxiety

13. Take home message