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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

Neurological Problems

Dr Gillian Small

Consultant Paediatrician


Neurological problems1
Neurological Problems

  • Congenital anomalies

  • Cerebral Palsy

  • Seizures

  • Meningitis/encephalitis

  • Encephalopathy

  • Neurodegenerative Disorders

  • Neuromuscular Disorders


Cerebral palsy
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


Aetiology
Aetiology

Preterm babies

Perinatal cause in 90% of these

  • Periventricular leukomalacia

  • Intraventricular haemorrhage

    Prenatal cause in 10%

  • Brain malformation


Aetiology1
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


Neurophysiology
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)


Neurophysiology1
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


Classification of cp
Classification of CP

  • Diplegia (44%)

  • Quadriplegia (7%)

  • Hemiplegia (34%)

  • Dyskinetic (9%)

  • Ataxic (6%)


Associated problems
Associated Problems

  • Vision

  • Hearing

  • Epilepsy

  • Feeding

  • Constipation


Diagnosis
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


Diagnosis1
Diagnosis

  • History

    Obstetric, perinatal history

    Lethargic, irritable

    Feeding problems, poor weight gain


Diagnosis2
Diagnosis

  • Examination

    To identify motor delay/abnormality

    Take gestational age into account for first 2yrs

  • General

    Dysmorphism, head growth, length, wt gain


Diagnosis3
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


Diagnosis4
Diagnosis

  • Asymmetry

    If persistent, indicates hemiplegia

  • Trunk control

  • General developmental delay


Investigations
Investigations

  • MRI

  • Chromosomes

  • Rubella/toxo/CMV titres

  • TFTs, creatine kinase

  • Urine metabolic screen


Management
Management

  • Multidisciplinary team approach

    Physios

    Speech and language therapy

    Occupational therapy

    Preschool support

    Dieticians

    Doctors


Febrile fits
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


Aetiology2
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


Clinical features
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


Clinical features1
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


Clinical features2
Clinical Features

  • Differential diagnosis – meningitis

    encephalitis

  • Consider LP in infants, especially < 6 mnths

  • Herpes encephalitis may present in infants with febrile partial seizures


Prognosis
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


Treatment
Treatment

  • Treat underlying illness

  • Cooling measures advised but no evidence that antipyretics prevent fits

  • Treat prolonged fits

  • Regular anticonvulsants are not advised


Prognosis1
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


Epilepsy
EPILEPSY

Definition of epileptic seizure

Paroxysmal discharge of cerebral neurones sufficient to cause a clinically definable event apparent to the observer or subject


Diagnosis5
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


Predisposing factors
Predisposing Factors

  • Genetic

  • Developmental brain abnormalities

  • Acquired structural abnormalities

    - Perinatal insults

    - Intracranial infections/trauma

    - Prolonged febrile fits

    - Cerebral haemorrhage or infarct

    - Tumours or AVMs


Recurrence
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)


Differential diagnosis
Differential Diagnosis

  • Syncope and related disorders

    vasovagal episodes

    reflex anoxic seizures blue or pallid

    long QT

  • Behavioural/ psychiatric disorders

  • Neurological disorders

  • Sleep related phenomenon


Types of seizure
Types of seizure

  • Focal (simple or complex)

  • Generalised

    Absence

    Myoclonic

    Atonic

    Tonic

    Tonic clonic

    Spasms


Absence seizures
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


Infantile spasms
Infantile Spasms

  • 3 – 12 months

  • Aetiology

    Cryptogenic (20%) normal development & CT

    Prenatal–Tuberous sclerosis, cong infection

    Perinatal-hypoxia, birth injury

    Postnatal-meningitis, encephalitis,trauma


Infantile spasms1
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


Infantile spasms2
Infantile spasms

  • EEG most commonly associated shows hypsarrythmia

  • Treat with vigabatrin or steroids

  • Associated with developmental delay


Floppy infant
Floppy infant

  • Marked hypotonia, lies in frog position, head lag

  • CAUSES

    Exclude systemic cause eg infection, hypothyroidism, inborn error of metabolism, congenital lax ligaments


Floppy infant causes
Floppy Infant - Causes

  • Central

    Encephalopathy

    Chromosomal abnormalities eg Down’s, Prader Willi

    Ataxic CP


Floppy infant causes1
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.


Floppy infant causes2
Floppy infant - Causes

  • Peripheral Nerve

    Guillain Barre neuritis

  • Neuromuscular Junction

    Myasthenia gravis. Transient in baby of myasthenic mother, or persistent


Floppy infant causes3
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.


Floppy infant causes4
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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