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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Dr Gillian Small
Perinatal cause in 90% of these
Prenatal cause in 10%
In 75% cause is prenatal
15% perinatal eg birth asphyxia
10% postnatal eg meningitis, head injury, cardiac arrest
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)
Damage to pyramidal system causes
Made on follow up of at risk infants from neonatal unit in about half of cases
Acquired from later severe illness in first year
Obstetric, perinatal history
Feeding problems, poor weight gain
To identify motor delay/abnormality
Take gestational age into account for first 2yrs
Dysmorphism, head growth, length, wt gain
Normally Grasp gone by 4 months, Moro by 6 months
Persistent thumb adduction, fisting, head lag
If persistent, indicates hemiplegia
Speech and language therapy
Definition of epileptic seizure
Paroxysmal discharge of cerebral neurones sufficient to cause a clinically definable event apparent to the observer or subject
Eye witness account
General and neuro examination
Lack of eye witness
Presence of clonic jerks/incontinence
Positive family history
Over interpretation of EEG
- Perinatal insults
- Intracranial infections/trauma
- Prolonged febrile fits
- Cerebral haemorrhage or infarct
- Tumours or AVMs
81% 15yrs later (Goodridge and Shorvon)
82% achieve 2 year remission after 8 years
reflex anoxic seizures blue or pallid
Cryptogenic (20%) normal development & CT
Prenatal–Tuberous sclerosis, cong infection
Perinatal-hypoxia, birth injury
Exclude systemic cause eg infection, hypothyroidism, inborn error of metabolism, congenital lax ligaments
Chromosomal abnormalities eg Down’s, Prader Willi
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.
Guillain Barre neuritis
Myasthenia gravis. Transient in baby of myasthenic mother, or persistent
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.
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