Transcript Abnormal movements in children

Abnormal movements in
children
Dr E Lubbe
Prof I Smuts
Dept Paediatrics
PAH
Bibliography
• Paediatrics and Child Health
Goovadia and
Wittenberg
• Rudolph`s Fundamentals of
Paediatrics
Rudolph et all
• Movement Disorders in
Children
Fernandez-Alverez and
Aicardi
Control of Movement
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
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Extra pyramidal system
•Basal ganglia
•Thalamus
•Subthalamic nuclei
•Substantia nigra
•Red nucleus
•Brainstem reticular
formation
Fluent movement
Start and stop of movement
Functional equilibrium in EPS
Dopamine
Ach
Overview
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Tics
Tremors
Chorea
Dystonias
Stereotypies
Myoclonus
Ataxia
Involuntary movements
• 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
Differentiating between types
SLOW
Abn movement
FAST
Stereotyped
(involuntary)
Dystonias
Athetosis
Non-stereotyped
Rhythmic
Not
Rhythmic
Tremor
Tics
Myoclonus
Chorea
Tics (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
DSM IV
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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 Single/multiple
motor AND/OR motor OR vocal
vocal
Multiple motor
AND
single/multiple
vocal
> 1 year
Not tic free >
3 months
> 1 year
Not tic free >
3 months
=/> 4 weeks
< 1 year
• 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
Diagnosis
• 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:
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Chorea
Myoclonus
Stereotypies
Compulsions
Pseudotics
Secondary – ass Strep infection “PANDAS”
Associated disorders
• 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
ADHD
OCD
• 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!
Associated with Strep inf:
PANDAS
• “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…
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Auto-antibody @basal ganglia
Criteria diagnosis:
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Prepubertal
Tics or OCD
Sudden onset / fluctuating course
Ass with GABHS inf.
Neurological abn.
? Re role antibiotics / immune modulation /
subgroup Tourette’s
Differentiating between types
SLOW
Abn movement
FAST
Stereotyped
(involuntary)
Dystonias
Athetosis
Non-stereotyped
Rhythmic
Not
Rhythmic
Tremor
Tics
Myoclonus
Chorea
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 )
Sydenham Chorea
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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
Clinical manoevres:
• “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.
Differentiating between types
SLOW
Abn movement
FAST
Stereotyped
(involuntary)
Dystonias
Athetosis
Non-stereotyped
Rhythmic
Not
Rhythmic
Tremor
Tics
Myoclonus
Chorea
Dystonia (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
Classification
• 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.
Dyskinetic CP
• 2 groups : Choreoathetotic and dystonic
• Often severe hypoxia in
term baby; previously
kernicterus
• History of insult + UMN
signs
• Incidence of MR 30%
Athetosis
• Alternating dystonia
• Tone alternates between flexion and
extension
• Distal muscles more affected than proximal
• Slow writhing movements
Stereotypies
• 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.
Differentiating between types
SLOW
Abn movement
FAST
Stereotyped
(involuntary)
Dystonias
Athetosis
Non-stereotyped
Rhythmic
Not
Rhythmic
Tremor
Tics
Myoclonus
Chorea
Tremors (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
Myoclonus (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
Cerebellar Disorders
Cerebellar signs
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Ataxia
Hypotonia
Dysarthria / abn speech
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
Acute ataxia
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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
Acute ataxia: Aetiology
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Infections
– Cerebellar abscess
– Viral cerebellitis
– Bacterial
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Organic acidurias
Leigh’s encephalopaties
Hypoglycaemia
Hyperammonaemia
Toxins
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Alcohol
Phenytoin
Phenobarbitone,
Lead
Glue
Vit A
Posterior fossa tumour
Vascular
– Haemorrhage
– Embolism
– AVM
Metabolic:
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Pseudo-ataxia
Chronic non progressive ataxia
• Ataxic/Hypotonic CP
• Often a congenital malformation of the
cerebellum
Aetiology: Chronic Non progressive
ataxia
– 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
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Hypoxia
Hypoglycaemia
Chronic phenytoin
Thiamine deficiency
Trauma
Chronic progressive ataxia
• Lesion in cerebellum with loss of:
– Purkinje cells
– Cerebellar nuclei
– Afferent or efferent pathways
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Olivary atrophy
Spinocerebellar degeneration
Post column demyelination
Peripheral nerve lesion
Ataxia telangiectasia
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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
Ataxia-Telangiectasia
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Slowly progressive cerebellar ataxia
Telangiectasis of skin and congunctivae
Frequent sinobronchopulmonary infections
Chorea-athetosis
Malignancies – lymphoreticular
Sensitivity to ionizing radiation
Ocularmotor apraxia
Ataxia-Telangiectasia
• Most common cause for progressive ataxia in
children under 10 next to posterior fossa
tumours!
Chronic progressive
• Friedreich’s ataxia
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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