Cognitive and psychiatric phenotypes of movement disorders in childrenHilla Ben-Pazi, MD

Movement Disorders Clinic,Neuropediatric Unit, Shaare Zedek Medical Center,Jerusalem, Israel

Haloperidol treatment

Insects crawlingHaloperidol –

discontinued

Psychosis:Heard voicesLocked doorsCouldn’t stay alone

Tonsillitis

Referred : hemichorea

Temper tantrums13 year old child

ADHD?

Lab: ^Antistreptolysin titer Mild aortic & mitral regurgitation

Basal Ganglia: Schematic slices

Rostral striatum

Striatal = Caudate+ Putamen

Caudal striatum

Basal ganglia- Dual processing Parallel and integrative processing of motor cognitive and psychiatric pathwaysParallelEmotional processingMotivationCognitive & executive

function Motor planningMotor execution

Haber, 2003

Integrative:Emotional -> cognitive -> motor

striatum

Substantia nigra- Midbrain

Opsoclonus-myoclonus syndrome (OMS)infantile autoimmune disorder Presentation:

Irritability Sleep disturbance Speech difficulty

Tate 2005; Papero 1995

Long term 13/14 speech

impairments Verbal fluency Articulation

50% understandable

Immuno-modulatory therapy

OMS Long term60-80% cognitive impairment

IQ 50 – 70 >50% special education

Psychiatric symptoms Irritability Emotional labiality 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

most disruptive:speechbehavior

Tourette syndrome and ticsrepeated, intermittent movements, briefly suppressible and usually associated with premonitory urge.

Emotional comorbidities extensively studied more disturbing than tics

ADHD (70%) ODD (30%) OCD (26%) Separation anxiety

(14%) Bipolar disorder (11%) Depression (2-

9%) PDD (5%) Schizophrenia (3%)

Compulsions Touching

Palms Counting

Obsessions Somatosensory Symmetry

Concern with appearance

Kurlan 2002; Miguel 1997

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

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

chelating th

erapy reverses symptoms

including psychiatric ill

ness

Machado 2006; Ullah, 2009

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

Restless Legs Syndrome is common (2%)90% ADHD

Correlates with RLS Not with sleep fragmentation

Anxiety / depression

Iron supplementation Improves RLS Not ADHD

Dopaminergic treatment Improves both RLS & ADHD

Chervin, 2002; Picchietti 2008; Miller , 2000Leg movements

Hyp

erac

tivi

ty

Psychiatric side effects – Mov dis drugsSide effect Mechanis

mDrug Mov Dis

Depression D depletion

Tetrabenazine

Chorea

Lethargy D antagonist

Haloperidol

Cog, Irritability

EnhancingGABA-A recp

Clonazepam Myoclonus

Hallucinations

β blocker Propranonol Tremor

Drowsiness α 2 agonist

Clonidine Tics

Anxiety Confusion

Anti Ach Trihexyphenidyl

Dystonia

Depression, Anxiety

D precursor

L-Dopa

Take home message

Assess psychiatric and cognitive

changes

Evaluate using validated tools

Treat according to disability

Monitor side effects