Paediatric Neurology

Question 1

What are the classifications of hydrocephalus?

Answer 1

Whether or not the ventricles communicate with the subarachnoid space.

If non-communication got obstruction is intra-ventricular

If communicating then the obstruction is extra-ventricular

Question 2

What features will be found on inspection of an infant with hydrocephalus?

Answer 2

Increased head circumference out of proportion with growth

Pressure on the anterior fontanelle is increased

If untreated then sun setting sign of the eyes can be present

Question 3

In older children what may the presenting features be?

Answer 3

Headche
Vomiting
Lethargy
Irritability 
Papilloedema

Question 4

How is hydrocephalus diagnosed?

Answer 4

In young children where the anterior fontanelle is still open USS can be used to assess the ventricles.

In older children CT will be used.

MRI is used to find the location of the obstruction

Question 5

What is the treatment of hydrocephalus?

Answer 5

Ventriculoperitoneal shunt

Question 6

What are the intraventricular causes of hydrocephalus?

Answer 6

Aqueduct stenosis
Dandy-Walker syndrome
Tumour
Ventriculitis

Question 7

What is dandy-walker syndrome?

Answer 7

A congenital condition where the cerebellar vermis is not formed which can lead to hydrocephalus

Question 8

What is craniosynostosis?

Answer 8

Premature fusion of the cranial sutures leading to abnormal shaped skull, most commonly the sagittal suture which leads to long and narrow skull

Question 9

What are the 3 main causes of encephalitis in children in the UK?

Answer 9

HSV I+II
Enterovirus
Varicella

Question 10

What is the definition of cerebral palsy?

Answer 10

A disorder of motor function die to a static lesion of the brain

Question 11

What are the clinical features of cerebral palsy?

Answer 11

Delayed motor milstones
abnormal tone and posturing in infancy
Feeding difficulties due to oromotor coordination
Delayed speech and language

Question 12

How is the diagnosis of cerebral palsy made?

Answer 12

It is a clinical diagnosis which is made by assessing tone, reflexes, abnormal movement (chorea), low power, abnormal posture or gait

Question 13

What are the 4 classification of cerebral palsy?

Answer 13

Spastic
Dyskinetic
Ataxic
Mixed

Question 14

Where is the brain lesion in spastic cerebral palsy and how may the disease manifest?

Answer 14

Pyramidal pathway which leads to increased done, brisk reflexes and reduced tone. Can affect any or all of the limbs

Question 15

Where is the brain lesion in ataxic cerebral palsy?

Answer 15

The cerebellum, abnormal gait and past pointing etc

Question 16

What treatment can be given of children who are experiencing hypertonia as a result of their cerebral palsy?

Answer 16

Botulinum toxin injections

Question 17

What is West Syndrome? what does it cause and how can it be treated?

Answer 17

epilepsy whereby a 4-6month old child has extremely violent seizures. Treatment is with ACTH

Question 18

What is Neurofibromatosis type 1, what is the inheritance pattern and what are the important clinical features?

Answer 18

An autosomal dominent condition where by there are neurofibrus lesions in the sin. It is autosomal dominant.

Clinical features:

• more that 6 cafe-au-lait spots
• Lisch nodule (pigmented hamartoma of the eye
• Neurofibromatomas which are palpable on periphery nerves at >8yo

Question 19

What is Neurofibromatosis type 2?

Answer 19

Similar pathophysiology to type 1 but is known as central neurofibromatosis as it affects the CNS.

Presentation is with seizures, learning difficulty or with a range of central nervous symptoms