An Approach to a Child

with Abnormal Movement

Sunil Agrawal
1st year MD
Pediatrics

Contents

Introduction
Pathophysiology
Classification
History
Examination
Investigations
Management

Introduction
- Dysfunction in the implementation of
appropriate targeting and velocity of
intended movements,
- dysfunction of posture,
- the presence of abnormal involuntary
movements,
-the performance of normal-appearing
movements at inappropriate or
unintended times.

Introduction- Contd
Can be the primary or secondary
manifestation of numerous neurologic
disorders
Classification can be difficult
Can resemble , sometimes difficult to
distnguish from each other

Pathophysiology
The components typically implicated in
disorders of movement are
the basal ganglia (caudate, putamen, globus
pallidus, subthalamic nucleus, substantia nigra) and
frontal cortex.

The accomplishment of smooth, coordinated

movement requires a multifaceted network of
brain regions, including basal ganglia and
frontal cortex, but also thalamus, cerebellum,
spinal cord, peripheral nerve, and muscle.

Movement Disorder according to lesion

. Lesion in globus pallidus athetosis
. Lesion in the subthalamic nucleus
hemiballismus
. Multiple small lesion in putamen s.chorea
. Lesion in caudate nucleus- huntington chorea
. Lesion in substantia nigra parkinsons disease

MOVEMENT DISORDERS

PYRAMIDAL
SYMPTOMS

BASAL GANGLIA
DISORDERS

CERBELLAR
DISORDERS

SPASTICITY

ATAXIA

HYPOKINESIAS

AKINESI
A

HYPERKINESIAS

MOTOR-SENSORY
BEHAVIOUR

RIGIDITY

TREMOR

COMPULSION
DYSTONI
A

MYOCLONU
S

CHOREA/
ATHETOSI
S

MANNERISM
TICS/
STERIOTYPIE
S

Fernandez alvarez, 2005

684 patient< 18 years

Tics DystoniaTremorMyoclonus
MixeaChoreaHypokinetic

43%
23%
16%
6%
4%
3%
3%

Biochemistry - neurotransmitters
Dopamine
Serotonin
GABA

Receptor

Acetylcholine
Histamine
Glutamate

Substance P

Hypokinetic - dopamine decreased - treat with dopamine

replacement or anticholinergic drugs
Hyperkinetic - dopamine increased and acetylcholine decreased
- treat with a dopamine antagonist or cholinergic drug

Definitions

Tics
Spasmodic, involuntary, repetitive,
stereotyped movements that are
nonrhythmic, often exacerbated by stress
May affect any group of muscle
Classification Transient tics of childhood ( < 1 year)
25 to 30% of children Most common
movement
abnormality of
childhood
Chronic tics (> 1 year)
Tourette syndrome

Chorea
Dance in Greek
Irregular, rapid, uncontrolled,
involuntary movements
Worsen on rest, but remain or
improve with voluntary movement
Incorporated into semipurposeful
acts to modify the movement
Tone - normal

Chorea-Contd..
Causes Parainfectious and autoimmune disorders Syndenhams chorea
SLE

Structural basal ganglia lesions Vascular chorea in stroke

Mass lesions

Chorea- causes Contd

Genetic Huntingtons disease
Ataxia telangiectasia

Infectious chorea

HIV encephalopathy
Cysticercosis
Toxoplasmosis
Diphtheria
Scarlet fever
Viral encephalitis( Mumps, measles, varicella)

Chorea- causes Contd

Metabolic or toxic encephalopathies Hypo/ hypernatremia
Hypocalcemia
Hyperthyroidism
Hypoparathyroidism
Hepatic/ Renal failure
Carbon monoxide, Manganese, mercury,
OP poisoning

Chorea- causes Contd

Drug induced chorea Dopamine receptor blocking agents Phenothiazines

Antiparkinsonian drugs L-dopa

Dopamine agonists
Anticholinergics

Antiepileptic drugs Phenytoin

Carbamazepine

Chorea- causes Contd

Drug induced chorea Dopamine receptor blocking agents Phenothiazines

Antiparkinsonian drugs L-dopa

Dopamine agonists
Anticholinergics

Antiepileptic drugs Phenytoin

Carbamazepine

Athetosis
Distal writhing movements of extremities
Choreoathetosis
Also has rigidity
Causes Extrapyramidal CP- asphyxia, kernicterus or
genetic metabolic disorder like glutaric aciduria
CP due to prematurity
Post- infectious
Cirulatory arrest for complex cardiac surgery
Drugs like phenothiazines

Tremor
Rhythmic oscillations of a part of the body around the
central point
Rest Intention CausesPhysiological
Essential tremor
Drugs-

Valproic acid
Neuroleptics
Caffeine

Trauma- head injury

Metabolic disorder
- hypoglycemia, thyrotoxicosis, neuroblastoma,
pheochromocytoma, Wilson disease

Dystonia
Syndrome of sustained muscle
contractions, frequently causing twisting
and repetitive movements or abnormal
postures
hallmark - simultaneous contraction of
agonist and antagonist muscle

Focal
Segmental
Multifocal
Hemi dystonia
Generalized

Dystonia- Contd..
Causes Perinatal asphyxia
Kernicterus
Generalised primary dystonia
Drugs
Wilson disease- Dystonia most common
neurologic manifestation
.Segmental- genetic, idiopathic or overuse

Ballismus

Form of chorea
Movements more coarse and ballistic
Hyper chorea
Extremity flailing
Causes
Sydenhams chorea
Stroke
Cerebral tumours and
Trauma

Myoclonus
Very brief, abrupt, involuntary, nonsuppressible, jerky contraction
involving a single muscle or muscle
group- "shock like"
Presence in normal (associated with
sleep, exercise, anxiety) and
numerous pathologic situations, both
epileptic and nonepileptic
Focal , segmental or generalized

Myoclonus
Causes

Physiologic
Benign nocturnal myoclonus
Benign myoclonus of infancy
Essential myoclonus
Epileptic myoclonus( Juvenile myoclonic epilepsy)
Opsoclonus- myoclonus
Post CNS injury
Basal ganglia disorders
Drug induced

Myoclonus
Causes

Physiologic
Benign nocturnal myoclonus
Benign myoclonus of infancy
Essential myoclonus
Epileptic myoclonus( Juvenile myoclonic epilepsy)
Opsoclonus- myoclonus
Post CNS injury
Basal ganglia disorders
Drug induced

Ataxia
Inability to make smooth, accurate and
coordinated movements
Due to disorder of cerebellum,sensory pathway in
posterior column of spinal cord
-Generalised or
- primarily affect gait or hands and arms

acute or chronic
Causes:
Acute or Recurrent

Brain tumor
Drugs like alcohol, thallium, anticonvulsants
Postinfectious/ immune
Trauma
Vascular disorder

Ataxia- Contd..
Chronic or Progressive Ataxia Brain tumors
Congenital malformations Cerebellar aplasias
Dandy- Walker malformation
Chiari malformation

Hereditary ataxias

Hypokinesia
Parkinsonism : bradykinesia, rigidity,
tremor or abnormal posture
Is rare in childhood
Causes Post head trauma
Post encephalitis
Genetic disorders- Juvenile Huntington
chorea, Wilson disease, ataxia
telangiectasia

Approach

Key questions
Is the pattern of movements normal or abnormal?
Is the number of movements excessive or
diminished?
Is the movement paroxysmal (sudden onset and
offset), continual (repeated again and again), or
continuous (without stop)?
Has the movement disorder changed over time?
Do environmental stimuli or emotional states
modulate the movement disorder?

Key questions- Contd..

Can the movements be suppressed voluntarily?
Are there findings on the examination
suggestive of focal neurologic deficit or
systemic disease?
Is there a family history of a similar or related
condition?
Does the movement disorder abate with sleep?

History
Age at onset full term neonate : jitteriness
Infant : myoclonus, athetosis, transient dystonia
Older child : chorea

Sexfemale: Sydenhams chorea,

thryrotoxicosis
male : tics, tremors

History- Contd..
Onset/duration
Acute : Infection, trauma
Slowly progressive : Wilsons
disease, Tourette syndrome,
Parkinsons disease, Hungtingtons
chorea

History- Contd..
Type of movement-

rapid jerky: chorea

slow movement : athetosis
sustained: dystonia

Involvement of body parts:

distal limb : athetosis

all body parts : chorea
hand : writercramp ,focal dystonia

Presence of movements in sleep :

seizure disorder
nocturnal myoclonus

History- Contd..
H/o waxing and waning: Tics
Aggravated with stress: tremor, tics, Tourette
synd
Generalised primary
dystonia, Nocturnal
myoclonus,
Syndenhams chorea
Relieving factors Behavioral abnormalities: chorea, Parkinsons
disease
Diurnal variation- with sleep : nocturnal
myoclonus

History- Contd..
H/o fever : infective origin
poisoning
Associated with signs of hepatic
failure- Wilson disease

History- Contd..
Sydenham chorea
- Associated with hypotonia,
emotionalability
-Other features of rheumatic fever

Joint pain , rashes : SLE

Associated with presenile dementiaHuntington disease

History- Contd..
H/o heat intolerance, increased
appetite with weight loss, increased
stool frequency, palpitationthyrotoxicosis
Features of increased ICP- Brain
tumors

History- Contd..
H/o intake of drugs
Perinatal history

Dystonia- Asphyxia, Jaundice

Athetosis- Asphyxia, jaundice and
prematurity

Cardiac surgery- Choreoathetosis

History- Contd..
Developmental history:
-delayed milestones
Immunization history :
polio, diptheria , pertusis
Family history- Huntington disease(AD)
Wilsons disease
Essential tremor
Consanguinuty

On Examination
General look
unconscious CNS infection

Vitals :
Raised temp - Infective
Pulse, BP, Pattern of respiration : ICSOL,
CNS infection, thyrotoxicosis
Ant fontanel

 Icterus : Wilsons disease

Eye :

opsoclonusmyoclonus syndrome
blepharospasm : tics
K-F ring

Mask like face (Parkinsonism)

Rash: Meningococal, Oculocutaneous
Telangiectasias

 Foreshortened occiput- Chiari

malformation
Prominent occiput- Dandy Walker
malformation

 Syndenham chorea- Milkmaids grip

Choreic hand
Darting tongue
Pronator sign

CNS Examination
GCS
Speech- vocal tics, dysarthria- chorea
CRANIAL NERVE
3rd and 6th nerve palsy raised ICT
Motor exam dystonia, hypotonia
rigidity, bradykinesia:Parkinsons disease
exaggerated reflex : thyrotoxicosis
ataxic gait : cerebellar lesion, ataxia
telangiectasis
Tip toe walking-generalised primary dystonia

CNS examination. Contd

Presence of primitive reflexes- cerebral
palsy
Signs of meningeal irritation?
any cerebellar signs?

Other Systems
Musculoskeletal examinaion
-side of the body- hemichorea,
hemiballismus
- which joint/limb- ballismus, dystonia
- joint tenderness

C.V.S: any murmurs

Abdominal:
hepatosplenomegaly , ascites
Thyroid

Investigations
* CBC

leucocytosis : infective
raised ESR : SLE

* Biochemical:

RFT, LFT,RBS ,Electrolytes

* Throat culture
Imaging:

CT SCAN , MRI
USG, ECHO

Investigations
4)Electrophysiological studies
EMG- dystonia
EEG
ECG

5)Special tests
Serological assay- ASO, antiDnase, ANA
antiphospholipid Ab
Serum Cu/ceruloplasmin/24 hr urinary copper/ liver
biopsy
Test for metabolic disorder
Toxins
Selective absence of IgA- Ataxia telangiectasia

*Other testing for rare disease- based in symptoms and

clinical suspicion

Principle of Management
Symptomatic treatment
Treatment of the cause
Counselling

Drug Treatment
Dystonia :
Diphenhydramine iv may reverse drug related
dystonia
Trihexyphenidyl, carbamazepine levodopa,
bromocriptine, diazepam
Botilinum toxin injection
Deep brain stimulation for generalized dystonia
A trial of L-DOPA is indicated in all cases of
chronic dystonia.

Drug Treatment
Tics:
Haloperidol, clonidine
Chorea:
Diazepam, valproic acid,
phenothiazine, haloperidol
Tremor:
B blockers, anticholinergics

References
Nelson Text book of pediatrics
Ghai ,Essential Pediatrics
Movement Disorders in Children -Schlaggar and Mink 24 (2) 39 -- Pediatrics
in Review
Clinical pediatric neurology,
Gerald.M.Fenichel 3rd edition
Pediatrics in Review Vol.24 No.2 February
2003

Thank you