Abnormal%20movements%20in%20%20children

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Abnormal movements in children

• Dr E Lubbe
• Prof I Smuts
• Dept Paediatrics
• PAH

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Bibliography

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

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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

• Extra pyramidal system
• Basal ganglia
• Thalamus
• Subthalamic nuclei
• Substantia nigra
• Red nucleus
• Brainstem reticular formation

Fluent movement Start and stop of movement
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Functional equilibrium in EPS
Dopamine
Ach
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Overview

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

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Involuntary movements

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

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

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DSM IV

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

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Tourettes
Chronic
Transient
Multiple motor AND single/multiple vocal
Single/multiple motor OR vocal
Single/multiple motor AND/OR vocal

• gt 1 year
• Not tic free gt 3 months

gt 1 year Not tic free gt 3 months
/gt 4 weeks lt 1 year
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• Tourettes Syndrome
• - combination motor and vocal tics
• - present gt 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

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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

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• Diff dx
• Chorea
• Myoclonus
• Stereotypies
• Compulsions
• Pseudotics
• Secondary ass Strep infection PANDAS

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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

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Tics
OCD
TS
ADHD
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• 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!

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Associated with Strep infPANDAS

• 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 _at_basal ganglia
• Criteria diagnosis
• Prepubertal
• Tics or OCD
• Sudden onset / fluctuating course
• Ass with GABHS inf.
• Neurological abn.
• ? Re role antibiotics / immune modulation /
  subgroup Tourettes

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Differentiating between types
Abn movement
SLOW
FAST
Non-stereotyped
Stereotyped (involuntary)
Dystonias Athetosis
Not Rhythmic
Rhythmic
Chorea
Tics Myoclonus
Tremor
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Chorea (involuntary, arythmical, asymmetric,
sudden, brief, gtproximal)

• 150 causes described!
• Infectious
• Rheumatic fever / Sydenham chorea
• Herpes encephalitis
• HIV
• Systemic diseases
• SLE
• Metabolic
• Wilsons disease
• Galactosaemia

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• Vascular
• Cyanotic heart disease
• Intoxication
• CO
• Methyl alcohol
• Primary genetic
• Benign hereditary
• Huntingtons (presents in children with
  hypokinesia )

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Sydenham Chorea

• Described in 1686
• Major feature of Rheumatic Fever
• In older than 10 year group gt 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

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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.

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

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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

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• 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.

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Dyskinetic CP

• 2 groups Choreo-athetotic and dystonic
• Often severe hypoxia in term baby previously
  kernicterus
• History of insult UMN signs
• Incidence of MR 30

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Athetosis

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

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Stereotypies

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

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

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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

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Cerebellar Disorders
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Cerebellar signs

• Ataxia
• Hypotonia
• Dysarthria / abn speech
• Dysmetria Struggle to judge distance
• Dysdiadochokinesia Struggle to start and stop
  rapid movements
• Nystagmus

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Ataxia - 3 broad categories

• Acute ataxia
• Chronic non-progressive
• Chronic progressive

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Acute ataxia

• Sudden onset
• Cant walk
• Extremely clumsy
• Cant 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

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Acute ataxia Aetiology

• Infections
• Cerebellar abscess
• Viral cerebellitis
• Bacterial
• Metabolic
• Organic acidurias
• Leighs encephalopaties
• Hypoglycaemia
• Hyperammonaemia
• Toxins
• Alcohol
• Phenytoin
• Phenobarbitone,
• Lead
• Glue
• Vit A

• Posterior fossa tumour
• Vascular
• Haemorrhage
• Embolism
• AVM
• Pseudo-ataxia

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Chronic non progressive ataxia

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

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Aetiology Chronic Non progressive ataxia

• Joubert syndrome
• Cerebellar/ kidney associations
• Postnatal acquired
• Hypoxia
• Hypoglycaemia
• Chronic phenytoin
• Thiamine deficiency
• Trauma

• Perinatal insults
• Birth asphyxia
• Metabolic
• Intra ventricular haemorrhage
• Meningitis
• Congenital malformations
• Primary cerebellar hypoplasia
• Hydrocephalus
• Foetal alcohol syndrome

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Chronic progressive ataxia

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

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Ataxia telangiectasia

• 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

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Ataxia-Telangiectasia

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

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Ataxia-Telangiectasia

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

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Chronic progressive

• Friedreichs 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

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