הלה בן פזי

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