anapproachtoachildwithabnormalmovement-130507112735-phpapp01

8/9/2019 anapproachtoachildwithabnormalmovement-130507112735-phpapp01

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An Approach to a Childwith Abnormal Movement

Sunil Agrawal

1st year MDPediatrics


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Contents

• Introduction

• Pathophysiology

• Classifcation• istory

• !"amination

• Investigations• Management


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Introduction

# Dys$unction in the implementation o$appropriate targeting and velocity o$intended movements%

- dys$unction o$ posture,

# the presence o$ abnormal involuntarymovements%

#the per$ormance o$ normal#appearing

movements at inappropriate orunintended times.


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Introduction# Contd&

• Can be the primary or secondarymani$estation o$ numerous neurologicdisorders

• Classifcation can be di'cult

• Can resemble % sometimes di'cult todistnguish $rom each other


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Pathophysiology

• (he components typically implicated indisorders o$ movement are – the basal ganglia )caudate% putamen% globus

pallidus% subthalamic nucleus% substantia nigra* and

– $rontal corte"+

• (he accomplishment o$ smooth% coordinatedmovement re,uires a multi$aceted networ- o$

brain regions% including basal ganglia and$rontal corte"% but also thalamus% cerebellum%spinal cord% peripheral nerve% and muscle+


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Movement Disorder according to lesionMovement Disorder according to lesion

+ .esion in globus pallidus / athetosis+ .esion in globus pallidus / athetosis

+ .esion in the subthalamic nucleus /+ .esion in the subthalamic nucleus /

hemiballismushemiballismus

+ Multiple small lesion in putamen / s+chorea+ Multiple small lesion in putamen / s+chorea

+ .esion in caudate nucleus# huntington chorea+ .esion in caudate nucleus# huntington chorea

+ .esion in substantia nigra /par-inson0s disease+ .esion in substantia nigra /par-inson0s disease


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Fernandez alvarez, 2005

; 1< years

• Tics - 43%

• Dystonia- 23%

• Tremor- 16%• Myoclonus 6%

• Mixea- 4%

• Chorea- 3%• Hypokinetic 3%


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Defnitions


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(ics

• Spasmodic% involuntary% repetitive%stereotyped movements that arenonrhythmic% o$ten e"acerbated by stress

• May a?ect any group o$ muscle

• Classifcation# – (ransient tics o$ childhood ) > 1 year* @ to B o$ children / Most common

movement abnormality o$childhood

– Chronic tics )E 1 year* – (ourette syndrome


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Chorea

• FDanceG in 6ree-

• Irregular% rapid% uncontrolled%involuntary movements

• Horsen on rest% but remain orimprove with voluntary movement

• Incorporated into semipurpose$ul

acts to modi$y the movement• (one # normal


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Chorea#Contd++

• Causes#• Parain$ectious and autoimmune disorders#

– Syndenham0s chorea –

S.!

• Structural basal ganglia lesions# – ascular chorea in stro-e

– Mass lesions


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Chorea# causes Contd&

• 6enetic#

– untington0s disease

– Ata"ia telangiectasia

• In$ectious chorea#

– I encephalopathy

– Cysticercosis

– (o"oplasmosis

– Diphtheria

– Scarlet $ever

– iral encephalitis) Mumps% measles% varicella*


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• Metabolic or to"ic encephalopathies#

– ypo: hypernatremia

– ypocalcemia

– yperthyroidism

– ypoparathyroidism

– epatic: 3enal $ailure

– Carbon mono"ide% Manganese% mercury%OP poisoning


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• Drug induced chorea# – Dopamine receptor bloc-ing agents#

• Phenothiaines

– Antipar-insonian drugs#• .#dopa

• Dopamine agonists

• Anticholinergics

– Antiepileptic drugs#• Phenytoin

• Carbamaepine


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• Drug induced chorea# – Dopamine receptor bloc-ing agents#

• Phenothiaines

– Antipar-insonian drugs#• .#dopa

• Dopamine agonists

• Anticholinergics

– Antiepileptic drugs#• Phenytoin

• Carbamaepine


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Athetosis

• Distal writhing movements o$ e"tremities• Choreoathetosis• Also has rigidity

• Causes# – !"trapyramidal CP# asphy"ia% -ernicterus or

genetic metabolic disorder li-e glutaric aciduria

– CP due to prematurity – Post# in$ectious – Cirulatory arrest $or comple" cardiac surgery – Drugs li-e phenothiaines


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(remor

• 3hythmic oscillations o$ a part o$ the body around thecentral point

• 3est #• Intention #

Causes-

Physiological• !ssential tremor• Drugs#

– alproic acid – 2euroleptics – Ca?eine

• (rauma# head inJury• Metabolic disorder

# hypoglycemia% thyroto"icosis% neuroblastoma%

pheochromocytoma% Hilson disease


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Dystonia

• Syndrome o$ sustained musclecontractions% $re,uently causing twistingand repetitive movements or abnormalpostures

hallmar- # simultaneous contraction o$

agonist and antagonist muscle

• Kocal• Segmental• Multi$ocal• emi dystonia• 6eneralied


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Dystonia# Contd++

• Causes#

– Perinatal asphy"ia

– 8ernicterus

– 6eneralised primary dystonia

– Drugs

– Hilson disease# Dystonia most common

neurologic mani$estation

+Segmental# genetic% idiopathic or overuse


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5allismus

• Korm o$ chorea

• Movements more coarse and ballistic

• yper chorea

• !"tremity Lailing

• Causes

– Sydenham0s chorea

– Stro-e

– Cerebral tumours and

– (rauma


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Myoclonus

• ery brie$% abrupt% involuntary% non#suppressible% Jer-y contractioninvolving a single muscle or muscle

group# shoc- li-e

• Presence in normal )associated withsleep% e"ercise% an"iety* and

numerous pathologic situations% bothepileptic and nonepileptic

• Kocal % segmental or generalied


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Myoclonus

• Causes#

– Physiologic

– 5enign nocturnal myoclonus

– 5enign myoclonus o$ in$ancy – !ssential myoclonus

– !pileptic myoclonus) Nuvenile myoclonic epilepsy*

– Opsoclonus# myoclonus

– Post C2S inJury – 5asal ganglia disorders

– Drug induced


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Myoclonus

• Causes#

– Physiologic

– 5enign nocturnal myoclonus

– 5enign myoclonus o$ in$ancy – !ssential myoclonus

– !pileptic myoclonus) Nuvenile myoclonic epilepsy*

– Opsoclonus# myoclonus

– Post C2S inJury – 5asal ganglia disorders

– Drug induced


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Ata"ia

• Inability to ma-e smooth% accurate andcoordinated movements

• Due to disorder o$ cerebellum%sensory pathway inposterior column o$ spinal cord

#6eneralised or # primarily a?ect gait or hands and arms

acute or chronic Causes!

• Acute or 3ecurrent# – 5rain tumor – Drugs li-e alcohol% thallium% anticonvulsants – Postin$ectious: immune – (rauma

– ascular disorder


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Ata"ia# Contd++

• Chronic or Progressive Ata"ia# – 5rain tumors

– Congenital mal$ormations#

• Cerebellar aplasias• Dandy# Hal-er mal$ormation

• Chiari mal$ormation

– ereditary ata"ias


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ypo-inesia

• Par-insonism brady-inesia% rigidity%tremor or abnormal posture

• Is rare in childhood

• Causes#

– Post head trauma

– Post encephalitis

– 6enetic disorders# Nuvenile untingtonchorea% Hilson disease% ata"ia

telangiectasia


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Approach


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8ey ,uestions

• Is the pattern o$ movements normal or abnormal

• Is the number o$ movements e"cessive ordiminished

• Is the movement paro"ysmal )sudden onset ando?set*% continual )repeated again and again*% orcontinuous )without stop*

• as the movement disorder changed over time

• Do environmental stimuli or emotional statesmodulate the movement disorder


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8ey ,uestions# Contd++

• Can the movements be suppressed voluntarily

• Are there fndings on the e"amination

suggestive o$ $ocal neurologic defcit orsystemic disease

• Is there a $amily history o$ a similar or relatedcondition

• Does the movement disorder abate with sleep


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istory

• "#e at onset-• $ull term neonate Jitteriness In$ant myoclonus% athetosis% transient dystonia Older child chorea

• $ex- $emale Sydenham0s chorea%

thryroto"icosis

male tics% tremors


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istory# Contd++

nset&'urationAcute In$ection% trauma

Slowly progressive Hilson0sdisease% (ourette syndrome%Par-inson0s disease% ungtington0schorea


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istory# Contd++

• :o wa"ing and waning (ics

• "##ra)ate' with stress tremor% tics% (ourettesynd 6eneralised primarydystonia% 2octurnal myoclonus%

Syndenham0s chorea• *elie)in# $actors#

• 5ehavioral abnormalities chorea% Par-insonsdisease

• Diurnal variation# with sleep nocturnalmyoclonus


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istory# Contd++

• :o $ever in$ective origin

• poisoning

• Associated with signs o$ hepatic

$ailure# Hilson disease


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istory# Contd++

• Sydenham chorea

# Associated with hypotonia%emotionalability

#Other $eatures o$ rheumatic $ever

• Noint pain % rashes S.!

• Associated with presenile dementia#untington disease


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istory# Contd++

• :o heat intolerance% increasedappetite with weight loss% increasedstool $re,uency% palpitation#

thyroto"icosis

• Keatures o$ increased ICP# 5rain

tumors


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istory# Contd++

• :o inta-e o$ drugs

• Perinatal history#• Dystonia# Asphy"ia% Naundice

• Athetosis# Asphy"ia% Jaundice andprematurity

• Cardiac surgery# Choreoathetosis


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istory# Contd++

• Developmental history #delayed milestones

• Immuniation history polio% diptheria % pertusis

• Kamily history# untington disease)AD* Hilson0s disease

!ssential tremor• Consanguinuty


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On !"amination

• 6eneral loo-unconscious / C2S in$ection

• itals /• 3aised temp # In$ective• Pulse% 5P% Pattern o$ respiration ICSO.%

C2S in$ection% thyroto"icosis• Ant $ontanel


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• Icterus Hilsons disease

• !ye

• opsoclonusmyoclonus syndrome• blepharospasm tics• 8#K ring

• Mas- li-e $ace )Par-insonism*

• 3ash Meningococal% Oculocutaneous (elangiectasias


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• Koreshortened occiput# Chiarimal$ormation

• Prominent occiput# Dandy Hal-ermal$ormation


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• Syndenham chorea# Mil-maid0s grip

Choreic hand

Darting tongue Pronator sign


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C2S !"amination

+C$ $peech# vocal tics% dysarthria# chorea

CRANIAL NERVE

3rd and 6th nerve palsy / raised IC( Motor exam# dystonia% hypotonia rigidity% brady-inesiaPar-insons disease e"aggerated reLe" thyroto"icosis

ata"ic gait cerebellar lesion% ata"iatelangiectasis

(ip toe wal-ing#generalised primary dystonia


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C2S e"amination+ Contd&

• Presence o$ primitive reLe"es# cerebralpalsy

• Signs o$ meningeal irritation

• any cerebellar signs


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Other Systems

• Musculoskeletal examinaion #side o$ the body# hemichorea%

hemiballismus# which Joint:limb# ballismus% dystonia

# Joint tenderness

• C$ any murmurs

• "'ominalhepatosplenomegaly % ascites

• Thyroi'


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Investigations

. C/C leucocytosis in$ective raised !S3 S.! . /iochemical! 3K(% .K(%35S %!lectrolytes

R Throat culture • (ma#in#! C( SCA2 % M3I• 9S6% !CO


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Investigations

40lectrophysiolo#ical stu'iesQ !M6# dystoniaQ !!6Q !C6

• 0$pecial tests• Serological assay# ASO% antiDnase% A2A antiphospholipid Ab• Serum Cu:ceruloplasmin:@= hr urinary copper: liver

biopsy• (est $or metabolic disorder• (o"ins• Selective absence o$ IgA# Ata"ia telangiectasia

ROther testing $or rare disease# based in symptoms andclinical suspicion


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Principle o$ Management

• Symptomatic treatment

• (reatment o$ the cause

•Counselling


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Drug (reatment

• Dystonia !

– Diphenhydramine iv may reverse drug relateddystonia

– (rihe"yphenidyl% carbamaepine levodopa%bromocriptine% diaepam

– 5otilinum to"in inJection

– Deep brain stimulation $or generalied dystonia

– A trial o$ .#DOPA is indicated in all cases o$chronic dystonia+


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Drug (reatment

• Tics!

aloperidol% clonidine

•Chorea!Diaepam% valproic acid%phenothiaine% haloperidol

• Tremor! 5 bloc-ers% anticholinergics


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3e$erences

• 2elson (e"t boo- o$ pediatrics• 6hai %!ssential Pediatrics

• Movement Disorders in Children ##

Schlaggar and Min- @= )@* B ## Pediatricsin 3eview

• Clinical pediatric neurology%6erald+M+Kenichel Brd edition

• Pediatrics in 3eview ol+@= 2o+@ Kebruary@B


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(han- you

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