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Neurological Problems

Neurological Problems. Dr Gillian Small Consultant Paediatrician. Neurological Problems. Congenital anomalies Cerebral Palsy Seizures Meningitis/encephalitis Encephalopathy Neurodegenerative Disorders Neuromuscular Disorders. Cerebral palsy.

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Neurological Problems

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  1. Neurological Problems Dr Gillian Small Consultant Paediatrician

  2. Neurological Problems • Congenital anomalies • Cerebral Palsy • Seizures • Meningitis/encephalitis • Encephalopathy • Neurodegenerative Disorders • Neuromuscular Disorders

  3. Cerebral palsy • Disorder of posture and movement due to non progressive damage of developing brain. • First described 150 years ago • Prevalence 2-3 per 1000 live births • Slight increase in recent years due to increase in survival of sick preterms

  4. Aetiology Preterm babies Perinatal cause in 90% of these • Periventricular leukomalacia • Intraventricular haemorrhage Prenatal cause in 10% • Brain malformation

  5. Aetiology Term babies In 75% cause is prenatal • Genetic • Cerebral malformation eg migration defect • Intrauterine infection eg CMV, rubella, toxo • Maternal – placental illness 15% perinatal eg birth asphyxia 10% postnatal eg meningitis, head injury, cardiac arrest

  6. Neurophysiology Approx 40% of children with CP are preterm Damage to extrapyramidal system causes dyskinetic CP (basal ganglia) or ataxic CP (cerebellum) Spastic CP results primarily from damage to pyramidal system (motor cortex/ internal capsule)

  7. Neurophysiology Damage to pyramidal system causes • Loss of selective voluntary control • Dependence on primitive patterns of mobility • Abnormal muscle tone & weakness • Agonist/antagonist imbalance – causes fixed joint positions & contractures • Preserved primitive reflexes

  8. Classification of CP • Diplegia (44%) • Quadriplegia (7%) • Hemiplegia (34%) • Dyskinetic (9%) • Ataxic (6%)

  9. Associated Problems • Vision • Hearing • Epilepsy • Feeding • Constipation

  10. Diagnosis Made on follow up of at risk infants from neonatal unit in about half of cases Parental concern Routine surveillance Acquired from later severe illness in first year

  11. Diagnosis • History Obstetric, perinatal history Lethargic, irritable Feeding problems, poor weight gain

  12. Diagnosis • Examination To identify motor delay/abnormality Take gestational age into account for first 2yrs • General Dysmorphism, head growth, length, wt gain

  13. DIAGNOSIS • Tone Hypotonia, hypertonia • Movements • Primitive reflexes Normally Grasp gone by 4 months, Moro by 6 months • Abnormal posture/positioning Persistent thumb adduction, fisting, head lag

  14. Diagnosis • Asymmetry If persistent, indicates hemiplegia • Trunk control • General developmental delay

  15. Investigations • MRI • Chromosomes • Rubella/toxo/CMV titres • TFTs, creatine kinase • Urine metabolic screen

  16. Management • Multidisciplinary team approach Physios Speech and language therapy Occupational therapy Preschool support Dieticians Doctors

  17. Febrile Fits • Fits precipitated by fever not due to intracranial infection or other CNS cause and are not preceded by afebrile fits • Common: 2 – 5% of children under 5 yrs

  18. Aetiology • Genetic factors important • Strong family history – 17-31% in first degree relatives • Polygenic mode of transmission • Fever usually high > 38.5 C • Fits usually occur early in course of fever • Perinatal factors do not play a role

  19. Clinical Features • Usually between 6 months and 6 years of age • Onset before 6 months suggestive of meningitis • Fits usually brief, bilateral, tonic clonic • Complicated febrile fits – if last more than 15 mins, if focal, those followed by Todds paresis. Higher risk of later epilepsy

  20. Clinical Features • Fits lasting > 30mins – Status epilepticus • May leave sequelae if untreated eg association with hippocampal sclerosis and consequent mesial temporal epilepsy • EEGs poorly correlate with later occurrence of epilepsy

  21. Clinical Features • Differential diagnosis – meningitis encephalitis • Consider LP in infants, especially < 6 mnths • Herpes encephalitis may present in infants with febrile partial seizures

  22. Prognosis • Favourable • 60 – 70% have only 1 fit • Only 9% have more than 3 episodes • Risk of recurrence greater if family history, aged under 1 year, had 2 or more episodes, had complex febrile fits. • Partial epilepsy more common after long lasting focal fits • Neurodevelopmental outcome usually excellent

  23. Treatment • Treat underlying illness • Cooling measures advised but no evidence that antipyretics prevent fits • Treat prolonged fits • Regular anticonvulsants are not advised

  24. Prognosis • Risk of developing afebrile fits – 2-5% (background risk 1%) • Risk low in simple seizures, increases with younger age, neurological/developmental abnormality, family history of epilepsy, complex febrile fits

  25. EPILEPSY Definition of epileptic seizure Paroxysmal discharge of cerebral neurones sufficient to cause a clinically definable event apparent to the observer or subject

  26. Diagnosis • Require Eye witness account Background history General and neuro examination • Causes of misdiagnosis Lack of eye witness Presence of clonic jerks/incontinence Positive family history Over interpretation of EEG

  27. Predisposing Factors • Genetic • Developmental brain abnormalities • Acquired structural abnormalities - Perinatal insults - Intracranial infections/trauma - Prolonged febrile fits - Cerebral haemorrhage or infarct - Tumours or AVMs

  28. Recurrence • Recurrence after first seizure quoted between 71 – 82% • Chances of remission 81% 15yrs later (Goodridge and Shorvon) 82% achieve 2 year remission after 8 years (Elwes 1984)

  29. Differential Diagnosis • Syncope and related disorders vasovagal episodes reflex anoxic seizures blue or pallid long QT • Behavioural/ psychiatric disorders • Neurological disorders • Sleep related phenomenon

  30. Types of seizure • Focal (simple or complex) • Generalised Absence Myoclonic Atonic Tonic Tonic clonic Spasms

  31. Absence seizures • Sudden cessation of activity with blank facial expression & eyelid flickering • Uncommon < 5yrs • More common in girls • Rarely longer than 30 seconds • No postictal drowsiness • Precipitated by hyperventilation • EEG – 3/second spike and wave • Treat with sodium valproate or ethosuximide

  32. Infantile Spasms • 3 – 12 months • Aetiology Cryptogenic (20%) normal development & CT Prenatal–Tuberous sclerosis, cong infection Perinatal-hypoxia, birth injury Postnatal-meningitis, encephalitis,trauma

  33. Infantile spasms • Flexor spasms – of neck, arms & legs on to trunk • Extensor spasms – extension of trunk and extremities & are least common • Mixed • Very brief, occur in clusters, may be preceded by a cry, occur when drowsy or awakening

  34. Infantile spasms • EEG most commonly associated shows hypsarrythmia • Treat with vigabatrin or steroids • Associated with developmental delay

  35. Floppy infant • Marked hypotonia, lies in frog position, head lag • CAUSES Exclude systemic cause eg infection, hypothyroidism, inborn error of metabolism, congenital lax ligaments

  36. Floppy Infant - Causes • Central Encephalopathy Chromosomal abnormalities eg Down’s, Prader Willi Ataxic CP

  37. Floppy Infant - Causes • Peripheral Spinal cord; transection, compression . Ant. horn cell; Werdnig Hoffman (acute spinal muscular atrophy), A.R., lack of fetal movements, floppy at birth, muscle fasciculation esp of tongue. Diagnosed on EMG, muscle biopsy. Death before 18 months from resp failure. Milder forms exist.

  38. Floppy infant - Causes • Peripheral Nerve Guillain Barre neuritis • Neuromuscular Junction Myasthenia gravis. Transient in baby of myasthenic mother, or persistent

  39. Floppy infant - Causes • Muscle Congenital myopathy Myotonic dystrophy; A.D., involves face and neck muscles producing myopathic face, ptosis, open/fish like mouth. Myotonia – difficulty in relaxing grasp. Also cardiac involvement and cataracts. Many die in newborn period, those surviving show some recovery.

  40. Floppy infant - Causes Metabolic myopathy Glycogen storage disease type II (Pompe Disease) Muscular Dystrophy; Duchenne MD X linked recessive. Rarely symptoms in early infancy. Late walkers, hypertrophied calves, onset of weakness aged 2 yrs, Gower’s sign. Elevated creatine kinase Genetic counselling, prenatal diagnosis Death in late adolescence, early adult life

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