1. MOVEMENT DISORDERS IN CHILDREN
September 2010
Gogi Kumar MD
Child Neurologist
Dayton Children’s Hospital
Wright State University

2. Movement disorders in children
Can be paroxysmal: Dystonia, tics, tremors
No single diagnostic test
Clinical description is important
Often confused with seizures, malingering, cerebral palsy
Treatment can be effective in a small percentage

3. Type of abnormal movements
Chorea
Athetosis
Dystonia
Myoclonus
Tics
Tremors
Stereotypies

4. Movement disorders in children
The most common types of abnormal movements in children
Tics
Drug induced
Dystonia related to CP
Sydenham’s chorea
Essential or task specific tremor
Psychogenic tremor

5. Movement disorders in children
Percentage in a series of 684 patients under 18 yrs of age
Movement disorders in children,
Fernandez-Alvarez, Aicardi

6. Tics are different !!! Can be reproduced voluntarily
Patient has partial control
Do not interfere with voluntary activity
Tics predominate in facial muscles, proximal limbs
May persist in sleep

7. Chorea
Chorea is a rapid, purposeless, irregular, jerky movement that seems to flow randomly from one part of the body to another.
Motor persistence.
Milkmaid’s grip :Maintaining a continuous hand grip.
Darting tongue: Difficulty holding there tongues out.
Motor impersistence

8. Etiology Genetic choreas Huntington’s disease Neuroacanthocytosis
Ataxia telangiectasia
Spinocerebellar ataxias
Paroxysmal kinosegenic choreoathetosis

9. Etiology of chorea
Parainfectious and autoimmune disorders Sydenham’s chorea SLE Wilson’s disease Chorea gravidarum Antiphospholipid antibody syndrome Post infectious or post vaccinal encephalitis

10. Etiology of chorea Athetotic Cerebral Palsy Bilirubin encephalopathy
Basal ganglia tumors
Metabolic disorders.

11. Sydenham’s Chorea Most common cause of chorea in children
Typical age is 8-9yrs with female preponderance.
4-8 weeks after GABHS pharyngitis.
20 – 35% may have hemichorea.
Hypotonia, emotional lability, ADHD, tics, OCD symptoms may accompany it.
60-80%pts have cardiac involvement.

12. Sydenham’s chorea
Self limited disorder with remission within 6-9 months.
Recurrence is reported in 20-50% of patients
Inv: ASO titres, throat culture, cardiac examination.
Treatment: Sodium Valproate, Carbamazepine, Pimozide.
Tetrabenazine, Tiapride
SSRIs for OCD symptoms.
Rheumatic fever prophylaxis.

13. Patient with possible Sydenham’s chorea
History including FH and drug use,physical examination
Typical history
Atypical history or hemichorea
ESR,CRP,Echocardiogram,ECG
No other cause
Imaging MRI
Jones criterion not met
Jones criterion met
ASO/Anti Dnase B,Throat culture, Rapid antigen test,GABHS, RBC, TFT,Renal and hepatic function,ceruloplasmin,RF antinuclear antibodies
Other cause
Sydenham’s chorea
Other cause
Oosterveer DM et al. Sydenham’s chorea: a practical overview of the current literature. Pediatr Neurol 2010;43:1-6.

14. Drug induced chorea Dopamine receptor blocking agents
Dopamine agonists
SSRIs
TCAs
OCPs
Lithium.

15. Investigations and Treatment
MRI to rule out basal ganglia lesion.
Rheumatological markers.
Specific Gene testing according to the presence of other features.
Treatment: Antidopaminergic drugs are the mainstay of treatment.
Risperidone olanzapine, tetrabenazine, haloperidol.
Amantidine, Gabapentin, Carbamazepine, Valproic acid.

16. PANDAS
PANDASen Thriving
Extinct
Endangered

17. Presence of obsessive-compulsive disorder or tic disorder
PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections)
Presence of obsessive-compulsive disorder or tic disorder
Onset between age 3 years and beginning of puberty
Onset or exacerbation of signs are temporally related to group A b-hemolytic streptococcal infection.
Abnormal results of neurological examination (hyperactivity/choreiform movements or tics during an exarcerbation)

18. Dystonia and Athetosis
Dystonia is a syndrome of sustained muscle contractions causing abnormal postures or twisting and repetitive movements.
Athetotic movements are complex, wormlike, irregular, non propositional and predominate over postural anomalies and on the distal parts of limbs and face.

19. Common types of Dystonia
Benign paroxysmal torticollis in infancy
Dopa responsive dystonia
Idiopathic torsion dystonia
Transient idiopathic dystonia of Infancy
Secondary dystonia:
1.Structural brain damage :Hemidystonia
2. Metabolic dystonia: Glutaric aciduria
Wilson’s disease, Lesch Nyan syndrome
Homocystinuria.

20. Idiopathic Torsion Dystonia DYT1
Onset is before 15 yrs of age
Onset in 1 limb, foot inversion is common
Initially it appears in only certain postures.
Course is progressive for 5-10 yrs and then becomes stationary.
N shaped progression
Tremors, swallowing difficulties,
myoclonus can be present.

21. Idiopathic Torsion Dystonia DYT1
Investigations:
Blood count and search for acanthocytes
MRI
Copper and ceruloplasmin
Slit lamp examination
Lysosomal enzymes (hexosaminidase,galactosidase)
ERG
Organic acids in urine.

22. Dopa responsive dystonia (DYT5)
First 12 yrs of life (median age of onset 4.5 and 6 yrs)
Females to male 2.5:1
Fatiguability, clumsiness of gait, dystonic posture limited to one foot .
Severity of the dystonia worse during the day and improves with sleep.
Postural tremor / Hyperactive reflexes present.
Marked and prolonged effect of L-Dopa (5-30mg/kg/day)
Mutations of DYT-5 gene GTP cyclohydrolase1 gene

23. Transient idiopathic dystonia of infancy
Onset before 5 months of age
Abnormal posture limited to one hand.
Arm is abducted and the forearm pronated with flexion at the wrist
May be permanent but can be paroxysmal
Dystonia disappears when the infant performs propositive movements.

24. Transient idiopathic dystonia of infancy

25. Treatment of Dystonia
A trial of low dose Levo Dopa (62.5mg-100mg) 2-3 times daily is warranted
High doses of Benzhexol (Artane) Start with 4-5mg/day and increase to 80 mg/day
Tetrabenazine and Pimozide/Haloperidol
Baclofen
Clonazepam and Diazepam
Botox
Deep Brain stimulation

26. Tremor in children 10-20% of pediatric movement disorders.
Action tremor most common.
Rest tremor uncommon, bad prognosis.
Task specific tremor .
Can occur in conjunction with dystonia/ myoclonus.

27. Etiology of Tremors Jitteriness Essential tremor Benign Physiological
Shuddering attacks
Jitteriness
Spasmus nutans
Hereditary
Essential tremor
Fragile X premutation
Strokes or Brain Lesions
Thalamus, midbrain, or cerebellum
Peripheral neuropathies
Mitochondrial encephalopathies
Metabolic
Hypoglycemia
B12 deficiency
Inborn errors of metabolism
Endocrine
Hyperthyroidism
Hyperadrenergic states
Drugs, Toxins
Valproate
Phenytoin
Carbamazepine
Lamictal
Gabapentin
Lithium
Tricyclic antidepressants
Bronchodilators
Caffeine
Amphetamines
Thyroxine
Neuroleptics
Cyclosporin
Amiodarone
Serotonin reuptake inhibitors
Cocaine
Mercury
Manganese
Psychogenic

28. Essential tremor Adapted from Bain and colleagues
Core criteria (features that must be present):
Bilateral action tremor of the hands and forearms (but not rest tremor);
or isolated head tremor with no signs of dystonia
No other neurological signs, with the exception of the cogwheel phenomenon
Secondary criteria (features that are supportive, but not necessary):
Long duration of symptoms (over 3 years)
Positive family history
Responsiveness to alcohol

29. Epidemiology of ET
Adults: Prevalence of 0.24%- 4.8%, no reliable data in children.
15-30% of Adults report onset in childhood.
M:F 3:1 in pediatrics.
Tremor onset at a mean age of between 3-11 yrs.

30. Differential diagnosis of ET
Wilson’s disease and hyperthyroidism should always be excluded.
If tremors are associated with dystonia, myoclonus,ataxia,seizures,weakness or it is unilateral suspect other causes.

31. Detailed medical history and examination
Postural and kinetic tremor
Detailed medical history and examination
Findings suggest other than essential tremor
History and physical examination suggests essential tremor.
Targeted diagnostic testing
Diagnosis of essential tremor
Assess for disability and determine whether treatment is needed.
Treat underlying cause of tremor
Monitor for progression
Determine whether daily therapy is required
Poor response
Initiate maintenance therapy with propranolol 0.5–1 mg/kg/day in
three divided doses
titrated up to
4 mg/kg/day
Propranolol as needed
Second-line agents:
primidone, topiramate,
gabapentin,
benzodiazepines,
botulinum toxin
Pediatr Drugs 2009; 11 (5)
Deep brain stimulation

32. Shudder attacks http://video.yahoo.com/watch/2666541?fr=yvmtf
Start in the first yr of life.
Brief bursts 5-15 sec of rapid tremor of head and arm
No change in level of consciousness
EEG normal
Precede essential tremor
Propranolol may be effective.

33. References
Pediatric Neurology ,Principles and Practice. Swaiman, Ashwal and Ferriero, Fourth Edition.
Movement Disorders in Children. Emilo Fernandez-Alvarez and Jean Aicardi
Ferrara J,Jankovic J .Epidemiology and Management of Essential Tremor in children, Pediatric Drugs 2009;11(5):
Keller S, Dure L. Tremor in childhood,Semin Pediatr Neurol ;16:
Cardoso F et al. Seminars on Chorea, Lancet Neurol 2006; 5: 589–602
Sanger TD et al, Definition and Classification of Hyperkinetic
Movements in Childhood, Movement Disorders Vol. 25, No. 11, 2010, pp. 1538–1549
Gonzalez-Alegre P, The Inherited dystonias Semin Neurol 2007;27:151–158
E. Ferna´ndez-Alvarez. Dystonia : The Paediatric Perspective European Journal of Neurology 2010, 17 (Suppl. 1): 46–51
Martino D et al,The PANDAS subgroup of tic disorders and childhood-onset obsessive–compulsive disorder Journal of Psychosomatic Research 67 (2009) 547–557
Kirsch DB, Mink JW. Psychogenic movement disorder in
children. Pediatr Neurol 2004;30:1-6.
Oosterveer DM, Overweg-Plandsoen WCT, Roos RAC. Sydenham’s chorea: a practical overview of the current lit- erature. Pediatr Neurol 2010;43:1-6.
Jancovic Joseph, Treatment of Dystonia Lancet Neurol 2006; 5: 864–72

34. Thank You !