Abnormal movements in children
Dr E Lubbe
Prof I Smuts
Dept Paediatrics
PAH

• Paediatrics and Child Health
Goovadia and
Wittenberg
• Rudolph`s Fundamentals of
Paediatrics
Rudolph et all
• Movement Disorders in
Children
Fernandez-Alverez and
Aicardi

Cerebellum
•Control of movement patterns
•Motor learning
•Judge speed, force and direction
•Coordinator of information
•Receives information from
•Muscle spindles
•Labyrinth, eyes, parietal cortex
•Joints
•Pressure receptors
+
Extra pyramidal system
•Basal ganglia
•Thalamus
•Subthalamic nuclei
•Substantia nigra
•Red nucleus
•Brainstem reticular formation
Fluent movement
Start and stop of movement

Ach
Dopamine

• Tics
• Tremors
• Chorea
• Dystonias
• Stereotypies
• Myoclonus
• Ataxia

• Abnormalities in structural and biochemical function of the nuclear masses of the basal ganglia
• Not under voluntary control
• Patient usually can’t stop them
• Without apparent purpose
• Aggravated by physical, emotional and mental stress

SLOW
Abn movement
FAST
Dystonias
Athetosis
Rhythmic
Tremor
Stereotyped
(involuntary)
Non-stereotyped
Not
Rhythmic
Tics
Myoclonus
Chorea

(complex stereotyped movement)
• Most common movement disorder in children
• Motor: eye blinking, shoulder shrug..
Vocal: squeaking, cough, sniffing…
Sensory: sensation ‘clothes not right’..
• Can be supressed ; relief when expressed again
• Lessens in sleep
• Aggravated by stress / anxiety

• Tics many times a day nearly every day
• Significant impairment or marked distress
• Onset < 18 yr
• Not due to drug or illness

Transient Chronic Tourette’s
Single/multiple motor AND/OR vocal
Single/multiple motor OR vocal
Multiple motor
AND single/multiple vocal
=/ > 4 weeks
< 1 year
> 1 year
Not tic free >
3 months
> 1 year
Not tic free >
3 months

• Tourettes Syndrome:
- combination motor and vocal tics
- present > 1 year
- onset before 18 years
not only present during use of psychotropic drugs
• Vocal tics can include echolalia, palilalia and coprolalia but rarer than led to believe in lay press
(present in about 20%)
• One third of cases asymptomatic by 17 years

• Clinical
• Clues:
– Previous “normal” phenomena (throat clearing, eye blinking)
– Family history tics / OCD
– Features ADHD
– Urge
– Awareness occurrence
– ABILITY TO SUPPRESS
– No functional disability
– Can persist in sleep

• Diff dx:
• Chorea
• Myoclonus
• Stereotypies
• Compulsions
• Pseudotics
• Secondary – ass Strep infection “PANDAS”

• Obsessions and compulsions - OCD
• ADHD
50 – 60% of TS precedes tics by 2-3 years
• Sleep disorders
• Learning problems 5X more special ed
• Behavioural problems
• Mood disorders

Tics
TS
OCD
ADHD

• Genetics: lot of data pointing to inheritable disorder
– probably autosomal dominant with variable expression
• Treatment:
-Pharmacological treatment only indicated if tics become incapacitating – rarely needed
Haloperidol usually effective
-Management of co-morbid disorders probably more important!
-

• “Paediatric Autoimmune Neuropsychiatric
Disorder associated with Streptococcal infection”
• Tics , dystonia = broader spectrum movement disorders post GABHS infections
• Emotional and behavioural changes common – OCD, anxiety, “personality change”, social phobias…

• Auto-antibody @basal ganglia
• Criteria diagnosis:
1. Prepubertal
2. Tics or OCD
3. Sudden onset / fluctuating course
4. Ass with GABHS inf.
5. Neurological abn.
• ? Re role antibiotics / immune modulation / subgroup Tourette’s

SLOW
Abn movement
FAST
Dystonias
Athetosis
Rhythmic
Tremor
Stereotyped
(involuntary)
Non-stereotyped
Not
Rhythmic
Tics
Myoclonus
Chorea

(involuntary, arythmical, asymmetric, sudden, brief, >proximal)
150 causes described!
• Infectious:
– Rheumatic fever / Sydenham chorea
– Herpes encephalitis
– HIV
• Systemic diseases:
– SLE
• Metabolic:
– Wilson`s disease
– Galactosaemia

• Vascular:
– Cyanotic heart disease
• Intoxication:
– CO
– Methyl alcohol
• Primary genetic:
– Benign hereditary
– Huntington`s (presents in children with hypokinesia )

• Described in 1686
• Major feature of Rheumatic Fever
• In older than 10 year group: > in girls
• Later symptom
• Progressive – starts with behaviour problems, clumsiness, difficulty writing , restlessness then after weeks chorea becomes evident
• Present weeks to months; usually good outcome

• “Milk maid sign” : let child grasp index finger of examiner
• Ask child to extend arms above head with palms upward – will find it difficult to maintain the pose and will excacerbate chorea.

SLOW
Abn movement
FAST
Dystonias
Athetosis
Rhythmic
Tremor
Stereotyped
(involuntary)
Non-stereotyped
Not
Rhythmic
Tics
Myoclonus
Chorea

(co-contraction; abn. posture)
• Def: involuntary sustained or intermittent muscle contractions causing twisting or repetitive movements, abnormal postures or both. Can affect any part of the body incl. arms, legs, trunk, neck, eyelids, face, vocal cords. Can be painful
• Disappears with sleep
• Caused by an imbalance of neurotransmitters

• Primary:
– Idiopathic torsion dystonia
– Transient idiopathic dystonia of infants
– Juvenile parkinsonism
• Secondary:
– Metabolic diseases
– Drug induced
– Stroke, trauma, tumour, neurodegenerative
• In children two major types
– Primary torsion dystonia
– Dopa-responsive dystonia

• Genetic in origin – most autosomal dominant with variable penetration. Begins in feet and spreads to become generalised
• Severely disabling; cognition spared
• Watch out – these can look bizarre. Do not mistake for a psychogenic disorder; rather refer.

• 2 groups : Choreoathetotic and dystonic
• Often severe hypoxia in term baby; previously kernicterus
• History of insult + UMN signs
• Incidence of MR 30%

• Alternating dystonia
• Tone alternates between flexion and extension
• Distal muscles more affected than proximal
• Slow writhing movements

• Voluntary often rhythmical movements – e.g. head banging, head rolling, thumb sucking. Selfstimulating behaviour can also be included here.
• Can occur in otherwise normal children
- head banging: 5% - 15 % normal infants (9 mo to 3yrs; >boys)
• Some patterns more prevalent in children with mental retardation and behaviour disorders like autism – e.g. self- mutilation, bruxism, hand washing.

SLOW
Abn movement
FAST
Dystonias
Athetosis
Rhythmic
Tremor
Stereotyped
(involuntary)
Non-stereotyped
Not
Rhythmic
Tics
Myoclonus
Chorea

(rapid rhythmical)
• Worsened by activity and anti-gravity posture; classified as rest, postural & kinetic
• Cerebellar: typical intention tremor
• Non-cerebellar:
– Idiopathic:
• Chronic – physiological; essential (familial)
• Transient – jitteriness; shuddering attacks
– Secondary:
• Malnutrition – vit B12 defficiency ; Kwashiorkor
• Hydrocephalus

(simple, sudden, single)
• Physiological:
– Sleep myoclonus
– Startle responses (awake)
• Non-epileptic:
– Benign neonatal sleep myoclonus
– Benign myoclonus of early infancy
• Epileptic:
– Myoclonic epilepsy
– Component of epileptic syndromes with different seizure types

1. Ataxia
2. Hypotonia
3. Dysarthria / abn speech
4. Dysmetria: Struggle to judge distance
5. Dysdiadochokinesia: Struggle to start and stop rapid movements
6. Nystagmus

Ataxia - 3 broad categories
• Acute ataxia
• Chronic non-progressive
• Chronic progressive

• Sudden onset
• Can’t walk
• Extremely clumsy
• Can’t feed due to tremor
• Dysarthria
• Nystagmus unusual
• Look for signs of infections e.g. chickenpox
• History of possible intoxication
• If signs are symmetrical, no raised ICP, and no focal signs, usually benign

• Infections
– Cerebellar abscess
– Viral cerebellitis
– Bacterial
• Metabolic:
– Organic acidurias
– Leigh’s encephalopaties
– Hypoglycaemia
– Hyperammonaemia
• Toxins
– Alcohol
– Phenytoin
– Phenobarbitone,
– Lead
– Glue
– Vit A
• Posterior fossa tumour
• Vascular
– Haemorrhage
– Embolism
– AVM
• Pseudo-ataxia

• Ataxic/Hypotonic CP
• Often a congenital malformation of the cerebellum

– Perinatal insults
• Birth asphyxia
• Metabolic
• Intra ventricular haemorrhage
• Meningitis
– Congenital malformations
• Primary cerebellar hypoplasia
• Hydrocephalus
– Foetal alcohol syndrome
– Joubert syndrome
– Cerebellar/ kidney associations
– Postnatal acquired
• Hypoxia
• Hypoglycaemia
• Chronic phenytoin
• Thiamine deficiency
• Trauma

• Lesion in cerebellum with loss of:
– Purkinje cells
– Cerebellar nuclei
– Afferent or efferent pathways
• Olivary atrophy
• Spinocerebellar degeneration
• Post column demyelination
• Peripheral nerve lesion

• Progressive ataxia(1-4 years)
• Abnormal eye movements - oculomotor apraxia
• Telangiectasia(3years-adolescence)
• Cutaneous manifestations
• High risk for malignancies
• Abnormality in cellular and humoral immunity
• Elevated alpha feto protein

• Slowly progressive cerebellar ataxia
• Telangiectasis of skin and congunctivae
• Frequent sinobronchopulmonary infections
• Chorea-athetosis
• Malignancies – lymphoreticular
• Sensitivity to ionizing radiation
• Ocularmotor apraxia

• Most common cause for progressive ataxia in children under 10 next to posterior fossa tumours!

• Friedreich’s ataxia
– Onset before 20 years
– Autosomal recessive inheritance
– Progressive ataxia - gait difficulties, speech problems
– No nystagmus
– Weakness
– Positive Babinski but absent ankle and knee reflexes - involvement of the corticospinal tract
– Loss of position and vibration sense
– Positive Romberg test - involvement of the posterior columns
– Bladder dysfunction
– Involvement of cranial nerves
– Scoliosis
– Cardiomyopathy
– Diabetes mellitis