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Movement Disorders
Dr. Tahira Nazir
Senior Registrar
Children hospital and UCHS Lahore
MYOCLONUS
• Myoclonus refers to very brief, abrupt, involuntary, non suppressible jerky
contractions involving a single muscle or muscle group.
TYPES
• Physiological myoclonus
• Benign Myoclonus
• Opsoclonus Myoclonus syndrome
• 40 % OMAS have a neuroblastoma .
• INVESTIGATIONS
• 24 hour urine catecholamines and serum neuron specific enolase (low yeild)
• MRI with gadolinium or CT with contrast chest and abdomen (high yeild)
TREATMENT OF MYOCLONUS
. Benzodiazipines( clonazepam)
• Valporic acid
• Levitriacetam
carbamazepine can worsen myoclonus
Infantile Spasms
• Myoclonic sizures
• WEST Syndrom
• With other neurocutaneous disorders
History and Examinatio
• Development
• When started
• Facial angiofibroma
• Ungual fibroma
• Hypomelanotic macules
• Shagreen patch
Investigations
• EEG
• MRI BRAIN
• Fundoscopy
• Echocardiography
• HRCT
• Renal imaging
Treatment
• ACTH
• Vigabatrin
• Ketogenic diet
• Benzodiazipines
• Zonisamide
• Pyridoxin
• IVIG
Chorea
• Rapid, chaotic movement that seems to flow from 1 body part to other.
• Increases with stresss and disappears in sleep.
• Signs of chorea
1. Darting tongue
2. Milkmaid grip
3. Pronator sign
4. Choreic hand( spooning of hand)
• Hallmarks of Sydenham chorea
1. Chorea
2. Hypotonia
3. Emotional lability
Management
• Phenobarbitone
• Haloparidol
• Chlorpromazine
• Corticosteroids may be given
Athetosis
• Characterized by slow continuous, writhing movement that involves the same
part of body, usually distal extermities , face , neck and trunk
• Athetosis tend to occur with other movement disorders such as chorea.
• Choreoathetosis is associated with cerebral palsy, kernicterus and other form
of basal ganglia injury.
Dystonia
• Dytonia is a disorder of movement characterized by sustained muscle
contractions, frequently causing twisting and repetitive movements or
abnormal postures.
• Inherited primary dystonia
• Drug induced dystonias
• Metabolic disorders
Ataxia
• Ataxia is inability to make smooth, accurate and coordinated movements.
• It occurs because of dysfunction of the cerebellum its input or output, its
sensory pathways in the posterior column of the spinal cord or combination
of these.
Causes of Ataxia
• Congrnital causes
• Degenerative or Genetic causes
• Endocrinologic causes
• Infectious , postinfectious and inflammatory causes
• Metabolic causes
• Neoplastic causes
• Primary psychogenic
• Toxic causes
• Traumatic causes
• Vascular causes

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movement disorders in pediatrics 204.pptx

  • 1. Movement Disorders Dr. Tahira Nazir Senior Registrar Children hospital and UCHS Lahore
  • 2. MYOCLONUS • Myoclonus refers to very brief, abrupt, involuntary, non suppressible jerky contractions involving a single muscle or muscle group. TYPES • Physiological myoclonus • Benign Myoclonus • Opsoclonus Myoclonus syndrome
  • 3. • 40 % OMAS have a neuroblastoma . • INVESTIGATIONS • 24 hour urine catecholamines and serum neuron specific enolase (low yeild) • MRI with gadolinium or CT with contrast chest and abdomen (high yeild) TREATMENT OF MYOCLONUS . Benzodiazipines( clonazepam) • Valporic acid • Levitriacetam carbamazepine can worsen myoclonus
  • 4. Infantile Spasms • Myoclonic sizures • WEST Syndrom • With other neurocutaneous disorders
  • 5. History and Examinatio • Development • When started • Facial angiofibroma • Ungual fibroma • Hypomelanotic macules • Shagreen patch
  • 6. Investigations • EEG • MRI BRAIN • Fundoscopy • Echocardiography • HRCT • Renal imaging
  • 7. Treatment • ACTH • Vigabatrin • Ketogenic diet • Benzodiazipines • Zonisamide • Pyridoxin • IVIG
  • 8. Chorea • Rapid, chaotic movement that seems to flow from 1 body part to other. • Increases with stresss and disappears in sleep. • Signs of chorea 1. Darting tongue 2. Milkmaid grip 3. Pronator sign 4. Choreic hand( spooning of hand)
  • 9. • Hallmarks of Sydenham chorea 1. Chorea 2. Hypotonia 3. Emotional lability
  • 10. Management • Phenobarbitone • Haloparidol • Chlorpromazine • Corticosteroids may be given
  • 11. Athetosis • Characterized by slow continuous, writhing movement that involves the same part of body, usually distal extermities , face , neck and trunk • Athetosis tend to occur with other movement disorders such as chorea. • Choreoathetosis is associated with cerebral palsy, kernicterus and other form of basal ganglia injury.
  • 12. Dystonia • Dytonia is a disorder of movement characterized by sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures.
  • 13. • Inherited primary dystonia • Drug induced dystonias • Metabolic disorders
  • 14.
  • 15. Ataxia • Ataxia is inability to make smooth, accurate and coordinated movements. • It occurs because of dysfunction of the cerebellum its input or output, its sensory pathways in the posterior column of the spinal cord or combination of these.
  • 16. Causes of Ataxia • Congrnital causes • Degenerative or Genetic causes • Endocrinologic causes • Infectious , postinfectious and inflammatory causes • Metabolic causes • Neoplastic causes • Primary psychogenic • Toxic causes • Traumatic causes • Vascular causes