Paediatric Neurology

Question 1

What is epilepsy?

Answer 1

a condition where there is a tendency to have seizures

seizures are transient episodes of abnormal electrical activity in the brain.

Question 2

What are tonic clonic seizures? How can they be managed?

Answer 2

There is LOC and tonic (muscle tensing) and clonic (muscle jerking) movements. There may be associated tongue biting, incontinence, groaning and irregular breathing.

After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or low.

First line: sodium valproate
Second line: lamotrigine or carbamazepine

Question 3

What are focal seizures? How can they be managed?

Answer 3

seizures that start in the temporal lobes and affect hearing, speech, memory and emotions.

There are various ways that focal seizures can present:
Hallucinations
Memory flashbacks, Déjà vu
Doing strange things on autopilot

Mx is the reverse of tonic-clonic seizures:
First line: carbamazepine or lamotrigine
Second line: sodium valproate or levetiracetam

Question 4

What are absence seizures? How can they be managed?

Answer 4

usually in children, patient becomes blank, stares into space and then abruptly returns to normal
during the episode they are unaware of their surroundings and won’t respond
typically only lasts 10 to 20 seconds

more than 90% stop having absence seizures as they get older

mx: sodium valproate or ethosuximide

Question 5

What are atonic seizures? How can they be managed?

Answer 5

also known as drop attacks

they are characterised by brief lapses in muscle tone and don’t usually last more than 3 minutes

mx:
sodium valproate first line

Question 6

What are myclonic seizures? How can they be managed?

Answer 6

sudden brief muscle contractions, like a sudden “jump”
patient usually remains awake during the episode

mx: sodium valproate first line

Question 7

How can infantile spasms (West syndrome) be managed?

Answer 7

Prednisolone
Vigabatrin

Question 8

How can suspected epilepsy be investigated?

Answer 8

Children are allowed one simple seizure before being investigated for epilepsy
after second simple tonic-clonic seizure: perform EEG

MRI brain when:
The first seizure is under 2 years
Focal seizure
no response to first line anti-epileptic medications

Other investigations:
ECG
Blood electrolytes including sodium, potassium, calcium and magnesium
Blood glucose for hypoglycaemia and diabetes
Blood cultures, urine cultures and lumbar puncture where sepsis, encephalitis or meningitis is suspected

Question 9

What general advice can be given to parents of children with epilepsy?

Answer 9

Take showers rather than baths
Be very cautious with swimming unless seizures are well controlled and they are closely supervised
Be cautious with heights
Be cautious with traffic
Be cautious with any heavy, hot or electrical equipment

Question 10

MOA sodium valproate? ADRs?

Answer 10

first line option for most forms of epilepsy (except focal seizures), works by increasing the activity of GABA

ADRs: TTHH
Teratogenic
Tremor
Liver damage and hepatitis
Hair loss

Question 11

ADRs of carbamazapine?

Answer 11

Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions

Question 12

ADRs of phenytoin?

Answer 12

Folate and vitamin D deficiency
Megaloblastic anaemia (folate deficiency)
Osteomalacia (vitamin D deficiency)

Question 13

ADRs of Ethosuximide?

Answer 13

Night terrors
Rashes

Question 14

ADRs of lamotrigine?

Answer 14

Stevens-Johnson syndrome or DRESS syndrome
Leukopenia

Question 15

General advice for parents when child is having a seizure?

Answer 15

Put the patient in a safe position (e.g. on a carpeted floor)
Place in the recovery position if possible
Put something soft under their head to protect against head injury
Remove obstacles that could lead to injury
Make a note of the time at the start and end of the seizure
Call an ambulance if lasting more than 5 minutes or this is their first seizure

Question 16

Define status epilepticus

Answer 16

a seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.

Question 17

How should status epilepticus be managed?

Answer 17

Secure the airway
Give high-concentration oxygen
Assess cardiac and respiratory function
Check blood glucose levels!!!

if vascular access = IV lorazepam
If no vascular access = buccal midazolam or rectal diazepam

after 5 mins if still convulsing, repeat first dose of benzo
after another 5 mins if still convulsing, give levetiracetam

Question 18

What is an extradural haemorrhage?

Answer 18

bleeding between the skull and dura mater that is usually caused by a rupture of the middle meningeal artery in the temporoparietal region

A typical hx is a young patient with a traumatic head injury and an ongoing headache. They have a period of improved neurological symptoms and consciousness, followed by a rapid decline as the haematoma gets large enough to compress the intracranial contents.

Question 19

How does an extradural haemorrhage present on CT?

Answer 19

bi-convex shape, does not cross cranial suture lines

Question 20

What is the immediate mx of a suspected bleed on the brain?

Answer 20

CT head is required to establish the diagnosis
FBC (for platelets) and a coagulation screen

Initial management will involve:
Admission to a specialist stroke centre
Discuss with a specialist neurosurgical centre to consider surgical treatment
Consider intubation, ventilation and intensive care if they have reduced consciousness
Correct any clotting abnormality (e.g. platelet transfusions or vitamin K for warfarin)
Correct severe hypertension but avoid hypotension

Question 21

What is the surgical mx of an intracranial haemorrhage?

Answer 21

Craniotomy (open surgery by removing a section of the skull)
Burr holes (small holes drilled in the skull to drain the blood)

Question 22

What is a subdural haemorrhage?

Answer 22

bleeding between the dura mater and arachnoid mater and is caused by a rupture of the bridging veins in the outermost meningeal layer

may occur in elderly and alcoholic patients, who have more atrophy in their brains, making the vessels more prone to rupture

Question 23

How does a subdural haemorrhage present on CT?

Answer 23

they have a crescent shape and are not limited by the cranial sutures (they can cross over the sutures lines)

Question 24

What is a subarachnoid haemorrhage?

Answer 24

bleeding in the subarachnoid space between the pia mater and the arachnoid membrane (where CSF is located)

usually the result of a ruptured cerebral aneurysm

The typical history is a sudden-onset severe “thunderclap” headache during strenuous activity, such as heavy lifting or sex.

Question 25

Migraine is a complex neurological condition causing episodes or attacks of headache and associated symptoms. It is very common, tends to affect women more often than men and is most common in teenagers and young adults.

How can it be categorised?

Answer 25

Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine

Question 26

What are the 5 stages of migraine?

Answer 26

Premonitory or prodromal stage (can begin several days before the headache)

Aura (lasting up to 60 minutes)

Headache stage (lasts 4 to 72 hours)

Resolution stage (the headache may fade away or be relieved abruptly by vomiting or sleeping)

Postdromal or recovery phase

Question 27

How do migraines usually present?

Answer 27

Usually unilateral but can be bilateral
Pounding or throbbing pain
Photophobia
Phonophobia
Osmophobia (discomfort with strong smells)
Aura (visual changes)
Nausea and vomiting

Question 28

What is aura?

Answer 28

acute sensory disturbance

Sparks in the vision
Blurred vision
Lines across the vision
Loss of visual fields (e.g., scotoma)

Question 29

How do hemiplegic migraines present?

Answer 29

main sx is hemiplegia (unilateral limb weakness). Other symptoms may include ataxia (loss of coordination) and impaired consciousness

Familial hemiplegic migraine is an autosomal dominant genetic condition

Question 30

What can trigger migraines?

Answer 30

Stress
Bright lights
Strong smells
Certain foods (e.g., chocolate, cheese and caffeine)
Dehydration
Menstruation
Disrupted sleep

Question 31

Mx options for an acute migraine attack?

Answer 31

NSAIDs (e.g., ibuprofen or naproxen)
Paracetamol
Triptans (e.g., sumatriptan)
Antiemetics if vomiting occurs (e.g., metoclopramide or prochlorperazine)

Question 32

Triptans are used to abort migraines when they start to develop. What is their MOA?

Answer 32

5-HT receptor agonists (they bind to and stimulate serotonin receptors)

Cause cranial vasoconstriction
Inhibit the transmission of pain signals

Question 33

What can be used for migraine prophylaxis?

Answer 33

Propranolol (a non-selective beta blocker)
Amitriptyline (a tricyclic antidepressant)
Topiramate (teratogenic and very effective contraception is needed)

Question 34

What is abdominal migraine?

Answer 34

may occur in young children before they develop traditional migraines as they get older. They present with episodes of central abdominal pain lasting more than 1 hour.

Examination will be normal. There may be associated:
Nausea and vomiting
Anorexia
Headache
Pallor

Question 35

What infections are associated with headache in children?

Answer 35

viral upper respiratory tract infection, otitis media, sinusitis and tonsillitis

sinusitis = facial pain behind the nose, forehead and eyes, tenderness over the effected sinuses, usually resolves within 2 – 3 weeks

Question 36

How do tension headaches present? How can they be managed?

Answer 36

typically mild ache or pressure in a band-like pattern around the head. They develop and resolve gradually and do not produce visual changes

sxs may be very non-specific in younger children. They may become quiet, stop playing, turn pale or become tired. They tend to resolve more quickly in children compared with adults, often within 30 minutes.

associated with stress, infection, skipping meals and dehydration

Mx: Reassurance
Simple analgesia (e.g., ibuprofen or paracetamol)

Question 37

What are breath holding spells?

Answer 37

involuntary episodes during which a child holds their breath, usually triggered by something upsetting or scaring them. They typically occur between 6 and 18 months of age

most children grow out of it, mx is reassurance and treating any associated iron deficiency anaemia

Question 38

What is muscular dystrophy?

Answer 38

an umbrella term for genetic conditions that cause gradual weakening and wasting of muscles.

most common = Duchennes muscular dystrophy

Question 39

Children with proximal muscle weakness use a specific technique to stand up from a lying position called Gower’s sign: describe it.

Answer 39

To stand up, they get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose. They then shift their weight backwards and transfer their hands to their knees. Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect. This is because the muscles around the pelvis are not strong enough to get their upper body erect without the help of their arms.

Question 40

What is spinal muscular atrophy?

Answer 40

a rare autosomal recessive condition that causes a progressive loss of motor neurones, leading to progressive muscular weakness

affects the lower motor neurones in the spinal cord

= LMN signs, such as fasciculations, reduced muscle bulk, reduced tone, reduced power and reduced or absent reflexes

Question 41

What are the categories of SMA?

Answer 41

SMA type 1 has an onset in the first few months of life, usually progressing to death within 2 years.

SMA type 2 has an onset within the first 18 months. Most never walk, but survive into adulthood.

SMA type 3 has an onset after the first year of life. Most walk without support, but subsequently loose that ability. Respiratory muscles are less affected and life expectancy is close to normal.

SMA type 4 has an onset in the 20s. Most will retain the ability to walk short distances but require a wheelchair for mobility

Question 42

How can SMA be managed?

Answer 42

Physiotherapy can be helpful in maximising strength in the muscles and retaining respiratory function. Splints, braces and wheelchairs can be used

non-invasive ventilation may be required, particularly during sleep. Children with SMA type 1 may require a tracheostomy with mechanical ventilation

Percutaneous endoscopic gastrostomy (PEG) feeding may be required when a weak swallow makes swallowing unsafe

Question 43

Give some causes of raised ICP

Answer 43

idiopathic intracranial hypertension
traumatic head injuries
meningitis
tumours
hydrocephalus

Question 44

Give some features of raised ICP

Answer 44

headache
vomiting
reduced levels of consciousness
papilloedema

Cushing’s triad:
widening pulse pressure
bradycardia
irregular breathing

Question 45

How can raised ICP be investigated?

Answer 45

neuroimaging (CT/MRI)

invasive ICP monitoring:
catheter placed into the lateral ventricles of the brain to monitor the pressure
may also be used to take collect CSF samples and also to drain small amounts of CSF to reduce the pressure
a cut-off of > 20 mmHg is often used to determine if further treatment is needed to reduce the ICP

Question 46

How can raised ICP be managed?

Answer 46

investigate and treat the underlying cause
head elevation to 30º
IV mannitol may be used as an osmotic diuretic
controlled hyperventilation :
aim is to reduce pCO2 → vasoconstriction of the cerebral arteries → reduced ICP
drainage of CSF