Transcript MOVEMENT DISORDERS - The Children`s Medical Center of Dayton

MOVEMENT
DISORDERS
IN CHILDREN
September 2010
Gogi Kumar MD
Child Neurologist
Dayton Children’s Hospital
Wright State University
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
Type of abnormal movements
 Chorea
 Athetosis
 Dystonia
 Myoclonus
 Tics
 Tremors
 Stereotypies
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
Movement disorders in children
Percentage in a series of 684 patients under 18 yrs of age
Movement disorders in children,
Fernandez-Alvarez, Aicardi
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
Chorea
Chorea is a rapid, purposeless,
irregular, jerky movement that seems
to flow randomly from one part of the
body to another.
Etiology
Genetic choreas
Huntington’s disease
Neuroacanthocytosis
Ataxia telangiectasia
Spinocerebellar ataxias
Paroxysmal kinosegenic choreoathetosis
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
Etiology of chorea
 Athetotic Cerebral Palsy
 Bilirubin encephalopathy
 Basal ganglia tumors
 Metabolic disorders.
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.
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.
Patient with possible
Sydenham’s chorea
History including FH and drug
use,physical examination
Typical history
Atypical history
or hemichorea
Imaging MRI
No other
cause
ESR,CRP,Echocard
iogram,ECG
Jones criterion not met
Other
cause
Jones criterion met
ASO/Anti Dnase B,Throat culture, Rapid
antigen test,GABHS, RBC, TFT,Renal
and hepatic function,ceruloplasmin,RF
antinuclear antibodies
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.
Drug induced chorea
 Dopamine receptor blocking agents
 Dopamine agonists
 SSRIs
 TCAs
 OCPs
 Lithium.
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.
PANDAS
 PANDASen

Thriving
Extinct


Endangered
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)
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.
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.
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.
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.
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
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.
Transient idiopathic dystonia of infancy
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
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.
Etiology of Tremors
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
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
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.
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.
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
Treat underlying cause of
tremor
Assess for disability and determine
whether treatment is needed.
Determine whether daily therapy is
required
Propranolol as
needed
Pediatr Drugs 2009; 11 (5)
Initiate maintenance
therapy with propranolol
0.5–1 mg/kg/day in
three divided doses
titrated up to
4 mg/kg/day
Deep brain
stimulation
Monitor for
progression
Poor response
Second-line agents:
primidone,
topiramate,
gabapentin,
benzodiazepines,
botulinum toxin
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.
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):293-307.
Keller S, Dure L. Tremor in childhood,Semin Pediatr Neurol 2009 ;16: 60-70.
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
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