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MOVEMENT MOVEMENT DISORDERSDISORDERS
IN CHILDRENIN CHILDRENSeptember 2010September 2010
Gogi Kumar MDChild NeurologistDayton Children’s HospitalWright State University
Movement disorders in Movement disorders in childrenchildren
Can be paroxysmal: Dystonia, tics, tremorsCan be paroxysmal: Dystonia, tics, tremors
No single diagnostic testNo single diagnostic test
Clinical description is importantClinical description is important
Often confused with seizures, malingering, Often confused with seizures, malingering, cerebral palsycerebral palsy
Treatment can be effective in a small Treatment can be effective in a small percentagepercentage
Type of abnormal Type of abnormal movementsmovements
Chorea Chorea
AthetosisAthetosis
DystoniaDystonia
MyoclonusMyoclonus
TicsTics
TremorsTremors
StereotypiesStereotypies
Movement disorders in Movement disorders in childrenchildren
The The most commonmost common types of abnormal types of abnormal movements in children movements in children
Tics Tics
Drug induced Drug induced
Dystonia related to CPDystonia related to CP
Sydenham’s choreaSydenham’s chorea
Essential or task specific tremorEssential or task specific tremor
Psychogenic tremorPsychogenic tremor
Movement disorders in Movement disorders in childrenchildren
Percentage in a series of 684 patients under 18 yrs of age
Movement disorders in children, Fernandez-Alvarez, Aicardi
Tics are different !!!Tics are different !!!
Can be reproduced voluntarilyCan be reproduced voluntarily
Patient has partial controlPatient has partial control
Do not interfere with voluntary activityDo not interfere with voluntary activity
Tics predominate in facial muscles, proximal Tics predominate in facial muscles, proximal limbslimbs
May persist in sleep May persist in sleep
ChoreaChorea
Chorea is a rapid, Chorea is a rapid, purposeless, irregular, jerky purposeless, irregular, jerky movement that seems to movement that seems to flow randomly from one part flow randomly from one part of the body to another.of the body to another.
EtiologyEtiology
Genetic choreasGenetic choreas
Huntington’s diseaseHuntington’s disease
NeuroacanthocytosisNeuroacanthocytosis
Ataxia telangiectasiaAtaxia telangiectasia
Spinocerebellar ataxiasSpinocerebellar ataxias
Paroxysmal kinosegenic choreoathetosisParoxysmal kinosegenic choreoathetosis
Etiology of choreaEtiology of chorea
Parainfectious and autoimmune disordersParainfectious and autoimmune disorders
Sydenham’s choreaSydenham’s chorea
SLESLE
Wilson’s diseaseWilson’s disease
Chorea gravidarumChorea gravidarum
Antiphospholipid antibody syndromeAntiphospholipid antibody syndrome
Post infectious or post vaccinal Post infectious or post vaccinal encephalitisencephalitis
Etiology of choreaEtiology of chorea
Athetotic Cerebral PalsyAthetotic Cerebral Palsy
Bilirubin encephalopathyBilirubin encephalopathy
Basal ganglia tumorsBasal ganglia tumors
Metabolic disorders.Metabolic disorders.
Sydenham’s ChoreaSydenham’s Chorea
Most common causeMost common cause of chorea in children of chorea in children
Typical age is 8-9yrs with female Typical age is 8-9yrs with female preponderance.preponderance.
4-8 weeks after GABHS pharyngitis.4-8 weeks after GABHS pharyngitis.
20 – 35% may have hemichorea.20 – 35% may have hemichorea.
Hypotonia, emotional lability, ADHD, tics, Hypotonia, emotional lability, ADHD, tics, OCD symptoms may accompany it.OCD symptoms may accompany it.
60-80%pts have cardiac involvement.60-80%pts have cardiac involvement.
Sydenham’s choreaSydenham’s chorea
Self limited disorder with remission within 6-9 Self limited disorder with remission within 6-9 months.months.
Recurrence is reported in 20-50% of patientsRecurrence is reported in 20-50% of patients
Inv: ASO titres, throat culture, cardiac Inv: ASO titres, throat culture, cardiac examination.examination.
Treatment: Sodium Valproate, Carbamazepine, Treatment: Sodium Valproate, Carbamazepine, Pimozide.Pimozide.
Tetrabenazine, TiaprideTetrabenazine, Tiapride
SSRIs for OCD symptoms.SSRIs for OCD symptoms.
Rheumatic fever prophylaxis.Rheumatic fever prophylaxis.
Patient with possible Sydenham’s chorea
History including FH and drug use,physical examination
Atypical history or hemichorea
Typical history
Imaging MRI
ESR,CRP,Echocardiogram,ECG
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
Sydenham’s chorea
Other cause
Other cause
No other cause
Oosterveer DM et al. Sydenham’s chorea: a practical overview of the current literature. Pediatr Neurol 2010;43:1-6.
Drug induced choreaDrug induced chorea Dopamine receptor blocking agentsDopamine receptor blocking agents
Dopamine agonistsDopamine agonists
SSRIsSSRIs
TCAsTCAs
OCPsOCPs
Lithium.Lithium.
Investigations and TreatmentInvestigations and Treatment
MRI to rule out basal ganglia lesion.MRI to rule out basal ganglia lesion. Rheumatological markers.Rheumatological markers. Specific Gene testing according to the Specific Gene testing according to the
presence of other features.presence of other features. Treatment:Treatment: Antidopaminergic drugs are Antidopaminergic drugs are
the mainstay of treatment.the mainstay of treatment. Risperidone olanzapine, tetrabenazine, Risperidone olanzapine, tetrabenazine,
haloperidol.haloperidol. Amantidine, Gabapentin, Amantidine, Gabapentin, Carbamazepine, Carbamazepine,
Valproic acid.Valproic acid.
PANDASPANDAS
PANDASen Thriving PANDASen Thriving
ExtinctExtinct
EndangeredEndangered
PANDASPANDAS(pediatric autoimmune neuropsychiatric disorders (pediatric autoimmune neuropsychiatric disorders
associated with streptococcal infections)associated with streptococcal infections)
Presence of obsessive-compulsive disorder Presence of obsessive-compulsive disorder or tic disorder or tic disorder
Onset between age 3 years and beginning of Onset between age 3 years and beginning of puberty puberty
Onset or exacerbation of signs are Onset or exacerbation of signs are temporally related to group A b-hemolytic temporally related to group A b-hemolytic streptococcal infection. streptococcal infection.
Abnormal results of neurological Abnormal results of neurological examination (hyperactivity/choreiform examination (hyperactivity/choreiform movements or tics during an exarcerbation) movements or tics during an exarcerbation)
Dystonia and AthetosisDystonia and Athetosis
Dystonia is a syndrome of Dystonia is a syndrome of sustained muscle sustained muscle contractions causing abnormal postures or contractions causing abnormal postures or twisting and repetitive movements.twisting and repetitive movements.
Athetotic movements are Athetotic movements are complex, complex, wormlike, irregular, non propositional and wormlike, irregular, non propositional and predominate over postural anomalies and predominate over postural anomalies and on the distal parts of limbs and face.on the distal parts of limbs and face.
Common types of Common types of DystoniaDystonia
Benign paroxysmal torticollis in infancyBenign paroxysmal torticollis in infancy Dopa responsive dystoniaDopa responsive dystonia Idiopathic torsion dystoniaIdiopathic torsion dystonia Transient idiopathic dystonia of InfancyTransient idiopathic dystonia of Infancy Secondary dystonia:Secondary dystonia:
1.Structural brain damage :Hemidystonia1.Structural brain damage :Hemidystonia
2. Metabolic dystonia: Glutaric aciduria2. Metabolic dystonia: Glutaric aciduria
Wilson’s disease, Lesch Nyan syndromeWilson’s disease, Lesch Nyan syndrome
Homocystinuria.Homocystinuria.
Idiopathic Torsion Dystonia Idiopathic Torsion Dystonia DYT1DYT1
Onset is before 15 yrs of ageOnset is before 15 yrs of age
Onset in 1 limb, foot inversion is commonOnset in 1 limb, foot inversion is common
Initially it appears in only certain postures.Initially it appears in only certain postures.
Course is progressive for 5-10 yrs and then Course is progressive for 5-10 yrs and then becomes stationary.becomes stationary.
N shaped progressionN shaped progression
Tremors, swallowing difficulties,Tremors, swallowing difficulties,
myoclonus can be present.myoclonus can be present.
Idiopathic Torsion Dystonia Idiopathic Torsion Dystonia DYT1DYT1
Investigations:Investigations:
Blood count and search for acanthocytesBlood count and search for acanthocytes
MRIMRI
Copper and ceruloplasminCopper and ceruloplasmin
Slit lamp examinationSlit lamp examination
Lysosomal enzymes Lysosomal enzymes (hexosaminidase,galactosidase)(hexosaminidase,galactosidase)
ERGERG
Organic acids in urine.Organic acids in urine.
Dopa responsive dystonia Dopa responsive dystonia (DYT5)(DYT5)
First 12 yrs of life (median age of onset 4.5 and 6 First 12 yrs of life (median age of onset 4.5 and 6 yrs)yrs)
Females to male 2.5:1Females to male 2.5:1
Fatiguability, clumsiness of gait, dystonic posture Fatiguability, clumsiness of gait, dystonic posture limited to one foot .limited to one foot .
Severity of the dystonia worse during the day and Severity of the dystonia worse during the day and improves with sleep.improves with sleep.
Postural tremor / Hyperactive reflexes present.Postural tremor / Hyperactive reflexes present.
Marked and prolonged effect of L-Dopa Marked and prolonged effect of L-Dopa (5-30mg/kg/day)(5-30mg/kg/day)
Mutations of DYT-5 gene GTP cyclohydrolase1 geneMutations of DYT-5 gene GTP cyclohydrolase1 gene
Transient idiopathic dystonia Transient idiopathic dystonia of infancyof infancy
Onset before 5 months of ageOnset before 5 months of age
Abnormal posture limited to one hand.Abnormal posture limited to one hand.
Arm is abducted and the forearm pronated Arm is abducted and the forearm pronated with flexion at the wristwith flexion at the wrist
May be permanent but can be paroxysmalMay be permanent but can be paroxysmal
Dystonia disappears when the infant Dystonia disappears when the infant performs propositive movements.performs propositive movements.
Transient idiopathic dystonia of Transient idiopathic dystonia of infancyinfancy
Treatment of DystoniaTreatment of Dystonia
A trial of low dose Levo Dopa (62.5mg-A trial of low dose Levo Dopa (62.5mg-100mg) 2-3 times daily is warranted100mg) 2-3 times daily is warranted
High doses of Benzhexol (Artane) Start with High doses of Benzhexol (Artane) Start with 4-5mg/day and increase to 80 mg/day4-5mg/day and increase to 80 mg/day
Tetrabenazine and Pimozide/HaloperidolTetrabenazine and Pimozide/Haloperidol
BaclofenBaclofen
Clonazepam and DiazepamClonazepam and Diazepam
BotoxBotox
Deep Brain stimulationDeep Brain stimulation
Tremor in childrenTremor in children
10-20% of pediatric movement disorders.10-20% of pediatric movement disorders.
Action tremor most common.Action tremor most common.
Rest tremor uncommon, bad prognosis.Rest tremor uncommon, bad prognosis.
Task specific tremor .Task specific tremor .
Can occur in conjunction with dystonia/ Can occur in conjunction with dystonia/ myoclonus.myoclonus.
BenignPhysiologicalShuddering attacks
JitterinessSpasmus nutansHereditary
Essential tremorFragile X premutationStrokes or Brain LesionsThalamus, midbrain, or cerebellumPeripheral neuropathiesMitochondrial encephalopathiesMetabolicHypoglycemiaB12 deficiencyInborn errors of metabolismEndocrineHyperthyroidismHyperadrenergic states
Drugs, ToxinsValproatePhenytoinCarbamazepineLamictalGabapentinLithiumTricyclic antidepressantsBronchodilatorsCaffeineAmphetaminesThyroxineNeurolepticsCyclosporinAmiodaroneSerotonin reuptake inhibitorsCocaineMercuryManganese
Psychogenic
Etiology of Tremors
Essential tremorEssential tremor Adapted from Bain and colleaguesAdapted from Bain and colleagues
Core criteriaCore criteria (features that must be present):(features that must be present):
Bilateral action tremor of the hands and forearms (but not Bilateral action tremor of the hands and forearms (but not rest tremor);rest tremor);
or isolated head tremor with no signs of dystoniaor isolated head tremor with no signs of dystonia
No other neurological signs, with the exception of the No other neurological signs, with the exception of the cogwheel phenomenoncogwheel phenomenon
Secondary criteria (features that are supportive, but not Secondary criteria (features that are supportive, but not necessarynecessary):):
Long duration of symptoms (over 3 years)Long duration of symptoms (over 3 years)
Positive family historyPositive family history
Responsiveness to alcoholResponsiveness to alcohol
Epidemiology of ETEpidemiology of ET
Adults: Prevalence of 0.24%- 4.8%, no Adults: Prevalence of 0.24%- 4.8%, no reliable data in children.reliable data in children.
15-30% of Adults report onset in childhood.15-30% of Adults report onset in childhood.
M:F 3:1 in pediatrics.M:F 3:1 in pediatrics.
Tremor onset at a mean age of between 3-Tremor onset at a mean age of between 3-11 yrs.11 yrs.
Differential diagnosis of ETDifferential diagnosis of ET
Wilson’s disease and hyperthyroidismWilson’s disease and hyperthyroidism should always be excluded.should always be excluded.
If tremors are associated with dystonia, If tremors are associated with dystonia, myoclonus,ataxia,seizures,weakness or it is myoclonus,ataxia,seizures,weakness or it is unilateral unilateral suspect other causes.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
Monitor for progression
Propranolol as needed
Initiate maintenance therapy with propranolol 0.5–1 mg/kg/day inthree divided dosestitrated up to4 mg/kg/day
Poor response
Second-line agents:primidone, topiramate,gabapentin,benzodiazepines,botulinum toxinDeep brain
stimulation
Pediatr Drugs 2009; 11 (5)
Shudder attacksShudder attacks
http://video.yahoo.com/watch/2666541?fr=yvmtfhttp://video.yahoo.com/watch/2666541?fr=yvmtf
Start in the first yr of life.Start in the first yr of life.
Brief bursts 5-15 sec of rapid tremor of head Brief bursts 5-15 sec of rapid tremor of head and armand arm
No change in level of consciousnessNo change in level of consciousness
EEG normalEEG normal
Precede essential tremorPrecede essential tremor
Propranolol may be effective.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 AicardiFerrara 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: 589–602Sanger 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): 46–51Martino D et al,The PANDAS subgroup of tic disorders and childhood-onset obsessive–
compulsive disorder Journal of Psychosomatic Research 67 (2009) 547–557Kirsch 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
Thank You !