Movement disorders
in Children
Types, Clinical Presentation, Diagnosis, Management
Prof. Imran Iqbal
Fellowship in Pediatric Neurology (Australia)
Prof of Paediatrics (2003-2018)
Prof of Pediatrics Emeritus, CHICH
Prof of Pediatrics, CIMS
Multan, Pakistan
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Clinical Presentation
of
CNS diseases
CNS disease – Clinical Presentation
• Seizures / Convulsions / Fits
• Impaired Conscious level
• Developmental delay
• Intellectual handicap
• Neurological Paralysis / Paresis
• Neuromuscular disorders
• Movement disorders
• Headache
• Regression of acquired milestones
• Behaviour problems
Movement disorders
 Ataxia
 Dystonia
 Involuntary movements
ATAXIA
(incoordination)
in children
Clinical Presentation, Types, Causes
Ataxia
Definition
• Inability to make smooth, accurate, coordinated movements
• Usually due to dysfunction of Cerebellum or its connections
Ataxia
Clinical Presentation
• Broad based gait
• Falls in walking
• Clumsiness of hand movements
• Difficult fine movements
• Dysmetria (lack of precision in movements)
• Intention tremors
• Dysarthria
• Nystagmus
Ataxia
Clinical Types
• Motor Ataxia – (with open eyes)
Cerebellum and its connections
• Differentiate from:
• Sensory Ataxia – (with closed eyes only)
Posterior columns (proprioceptive sensations)
Motor Ataxia
Clinical Presentation (Video)
Sensory Ataxia
Romberg sign (Video)
Acute Ataxia - Etiology
(Sudden Onset)
• Drugs / Poisoning / Intoxication
• Muscle Weakness - (GBS)
• Acute Cerebellar Inflammation – Acute Cerebellar Ataxia
Acute Cerebellar Ataxia
• Age – One to three years
• Sudden onset of severe ataxia
• Post - viral illness (Varicella)
• Auto-immune response
• Improves spontaneously in weeks to months
• Corticosteroids may help in recovery
Chronic Ataxia - Etiology
(Slow Onset)
• Brain tumors
• Neuroblastoma
• Cerebellar malformations
• Genetic causes
• Friedrich’s Ataxia
• Ataxia Telangiectasia
Friedrich’s Ataxia
Dysfunctions
• Autosomal Recessive Genetic disease
• Ataxia – starts before 10 years of age
• Slowly progressive
• Spinal Cord - Corticospinal tract, Spinocerebellar Tract and
Dorsal columns affected
• Cardiomyopathy
• Scoliosis
• Pes cavus
Ataxia Telangiectasia
• Autosomal Recessive Genetic disease
• Ataxia – starts around 2 years of age
• Immunodeficiency – frequent sino-pulmonary infections
• Telangiectasia (on bulbar conjunctiva) – develop later
Ataxia Telangiectasia
Dystonia
(tonic muscle contraction)
in children
Clinical Presentation, Causes, Management
Dystonia
Clinical Presentation
• Involuntary, sustained skeletal muscle contraction
• Twisting movements
• Abnormal postures
• Contraction of agonist and antagonist muscles
• Failure to relax
• Painful muscle contractions
Dystonia
Pictures
Dystonia
Etiology – Basal Ganglia
• Acute Dystonia –
• Drugs – Metoclopramide, Haloperidol
• Encephalitis
• Chronic Dystonia –
• Cerebral Palsy (dyskinetic type) - due to Kernicterus
• Wilson disease
• Genetic disorders
Dystonia
Management
• Muscle relaxants – Baclofen
• Sedatives – Diazepam, Clonazepam
• Anti-cholinergics - Procyclidine
• Dopamine agonists – Levodopa
• Paralytics – Botulinum toxin injection
Involuntary movements
in children
Types, Clinical Presentation, Causes
Involuntary movements
• Chorea – small amplitude peripheral movement
• Athetosis – writhing movement
• Tremors – oscillating movement
• Tics – habitual repetitive movements
• Myoclonus – sudden small jerky movements
• Ballismus – large amplitude jerky movement
Chorea
in children
Clinical Presentation, Causes, Management
Chorea
• Involuntary movements
• Semi-purposive
• Random
• Variable movements
• Movements at rest
• Movements usually involve peripheral parts of body
• Inability to maintain static posture
Chorea (Video)
Chorea
Tongue sign
Chorea
Etiology
• Rheumatic Fever - Sydenham’s Chorea
• SLE
• Genetic disorders
Chorea
Management
• Sedatives – Diazepam, Clonazepam
• Anti-epileptics – Phenobarbitone, SodiumValproate
• Anti-Psychotics – Haloperidol, Chlorpromazine
Tic disorder
in children
Clinical Presentation, Causes
Motor Tics (Video)
Tics (Stereotype Movements)
• Motor movements or Vocalisations
• Involuntary, abrupt, repetitive, non-rhythmic
• Simple or complex
• Can be suppressed by voluntary effort
• often associated with other Behavior disorders
Myoclonus
in children
Clinical Presentation, Types, Causes
Myoclonus
Video
Myoclonus
• Muscle jerks – Involuntary, Sudden, Quick
• ETIOLOGY
• Physiological myoclonus – in sleep
• Essential myoclonus – aggravated by stress
• Epilepsy (myoclonic epilepsy)
• Acute Encephalopathy
• Degenerative Brain disorders
• MANGEMENT
• No treatment needed for Physiological myoclonus
Prof Imran Iqbal
Fellowship Pediatric Neurology-1991
Royal Children Hospital, Melbourne, Australia

Movement disorders in Children 2022.pdf