Neurological disability Flashcards by Caroline Hazel

What is the definition of cerebral palsy?

= a permanent disorder of movement and of motor function due to a non-progressive abnormality of the developing brain

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What is the most common motor impairment of children?

Cerebral palsy

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How common is cerebral palsy?

2 per 1000

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What proportion of cerebral palsy cases are antenatal in origin?

80%

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What proportion of cerebral palsy cases are perinatal in origin?

10%

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What proportion of cerebral palsy cases are postnatal in origin?

10%

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What are the antenatal causes of CP?

1) Cerebrovascular haemorrhage or ischaemia
2) Cortical migration disorders
3) Structural maldevelopment of the brain
4) Congenital infection (e.g. CMV)

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What are the perinatal causes of CP?

HIE before/during delivery

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What are the postnatal causes of CP?

1) Meningitis/encephalitis/encephalopathy
2) Head injury
3) Symptomatic hypoglycaemia
4) Hydrocephalus
5) Hyperbilirubinaemia

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What are the antenatal RISK FACTORS for CP?

1) Pre-term birth
2) Chorioamnionitis
3) Maternal respiratory tract/GU infection requiring hospital treatment

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What are the perinatal RISK FACTORS for CP?

1) LBW
2) Chorioamnionitis
3) Neonatal encephalopathy
4) Neonatal sepsis
5) Maternal respiratory tract/GU infection requiring hospital treatment

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How might CP appear on MRI?

White matter damage
Basal ganglia or deep grey matter damage
Congenital malformation
Focal infarcts

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What are the most commonly delated motor milestones seen in CP?

Not sitting by 8 months
Not walking by 18 months
Early asymmetry of hand function (hand preference) before 1 year

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How may CP be categorised?

Spastic (90%)
Dyskinetic
Ataxic
Other

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What is the pathophysiology behind spastic CP?

Damage to the UMN pathway - pyramidal or corticospinal tract

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What are the different types of spastic CP?

Unilateral (hemiplegia)
Bilateral (quadriplegia)
Bilateral (diplegia)

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How do children with hemiplegic spastic CP tend to present?

Between 4-12 months

Fisting of the affected hands, flexed arm and pronated forearm

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Describe typical features of a child with quadriplegic spastic CP:

All limbs involved
Poor head control
Trunk involved, tendency for extensor posturing
Associated with seizures, microcephaly, intellectual, speech, vision, hearing and feeding impairments

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Describe the typical features of a child with diplegic spastic CP:

All limbs affected, but legs to a much greater extent
Young children will walk on their toes with scissoring of their legs
Older children may adopt a crouching gait pattern as they gain weight and are no longer able to walk sustained periods on tip-toes

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What type of CP is most commonly associated with preterm birth and PVL?

Diplegic spastic CP

What defines dyskinetic CP?

Involuntary uncontrolled movements - may be 1) chorea (irregular, brief, repetitive); 2) athetosis (slow writhing movements occurring more distally, e.g. finger fanning) or 3) dystonia (twisting appearance of movement)

What is the pathophysiology of dyskinetic CP?

Damage to the basal ganglia and their associated (extra-pyramidal) pathways

How will children with dyskinetic CP typically look?

Open mouth posture with internal rotation and extension of the arms

How is CP managed?

By the MDT

What are some novel approaches to treating hypertonia?

1) Botox 2) Selective dorsal rhizotomy - proportion of nerve roots in the spinal cord = selectively cut to reduce spasticity 3) Intrathecal baclofen 4) Deep brain stimulation of the basal ganglia

What are the types of disorder that can result in a floppy (hypotonic) infant?

1. Disorders of the anterior horn cell 2. Disorders of the peripheral nerve 3. Disorders of the neuromuscular transmission 4. Muscle disorders

What are the disorders of the anterior horn cell?

1. SMA | 2. Poliomyelitis

What are the disorders of the peripheral nerve?

1. Bells palsy 2. Guillan-Barre 3. Hereditary motor sensory neuropathies

What are the disorders of the neuromuscular transmission?

1. Myasthenia gravis

What are the muscle disorders?

1. DMD 2. Becker muscular dystrophy 3. Congenital muscular dystrophy 4. Metabolic myopathies 5. Congenital myopathies 6. Polymyositis/dermatomyositis 7. Benign acute myositis

What is the most common neuromuscular disorder in children?

DMD

What is the second most common neuromuscular disorder in children?

SMA

How is SMA inherited?

Autosomal recessive

How is DMD inherited?

X-linked recessive, but approx. 1/3rd = de novo mutations

How can DMD be Dx?

Clinically + markedly elevated plasma CK

What is the pathophysiology of DMD?

Mutation in DMD gene results in absence of dystrophin Absence = influx of Ca2+, breakdown of Ca2+ calmodulin complex + excess of free radicals Results in myofibre necrosis

What are the 3 classical features of DMD?

1. Waddling gait 2. Gower's sign 3. Pseudo-hypertrophy

What causes calf pseudo-hypertrophy?

Muscle fibres are replaced by fat and fibrous tissue

Becker muscular dystrophy is caused by a mutation in which gene?

DMD also - it is allelic to Duchenne's = different mutation, same gene

Why is Becker's clinically milder + progresses more slowly than Duchenne's?

Some functional dystrophin is produced

What is the location of 60% of childhood brain tumours?

Infratentoril (i.e. below the tentori cerebelli)

What are the 2 types of hydrocephalus?

1. Non-communicating - obstruction in the ventricular system | 2. Communicating - failure to resorb CSF

What can cause non-communicating hydrocephalus?

1. Congenital malformation - e.g. aqueduct stenosis, chiari malformation, atresia of outflow foramina of the 4th ventricle 2. Posterior fossa neoplasm or vascular malformation 3. IVH in pre-term infant

What can cause communicating hydrocephalus?

1. Subarachnoid haemorrhage | 2. Meningitis

Is shunt infection were to occur, what is the most common causative organism?

Coagulase -ve staphylococci

What is the definition of microcephaly?

Head circumference >2 SDs BELOW the mean for age and sex

What are the congenital causes of microcephaly?

1. Familial/inherited 2. Syndromes - e.g. Down's, William's, DiGeorge 3. Congenital infection - e.g. CMV, zika, rubella, toxoplasmosis 4. Drugs - e.g. alcohol 5. Other - e.g. maternal radiation, PKU, hypothyroidism

What are the post-natal onset causes of microcephaly?

1. Inborn errors of metabolism 2. Syndromes - e.g. Rett syndrome 3. TBI/HIE/stroke 4. Infection - e.g. meningitis 5. Toxins - e.g. chronic renal failure, lead poisoning 6. Deprivation (of nutrient/hormone etc.) - e.g. anaemia, hypothyroidism, CHD