Paediatric Neurology Flashcards
What is epilepsy?
a condition where there is a tendency to have seizures
seizures are transient episodes of abnormal electrical activity in the brain.
What are tonic clonic seizures? How can they be managed?
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
What are focal seizures? How can they be managed?
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
What are absence seizures? How can they be managed?
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
What are atonic seizures? How can they be managed?
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
What are myclonic seizures? How can they be managed?
sudden brief muscle contractions, like a sudden “jump”
patient usually remains awake during the episode
mx: sodium valproate first line
How can infantile spasms (West syndrome) be managed?
Prednisolone
Vigabatrin
How can suspected epilepsy be investigated?
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
What general advice can be given to parents of children with epilepsy?
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
MOA sodium valproate? ADRs?
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
ADRs of carbamazapine?
Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions
ADRs of phenytoin?
Folate and vitamin D deficiency
Megaloblastic anaemia (folate deficiency)
Osteomalacia (vitamin D deficiency)
ADRs of Ethosuximide?
Night terrors
Rashes
ADRs of lamotrigine?
Stevens-Johnson syndrome or DRESS syndrome
Leukopenia
General advice for parents when child is having a seizure?
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
Define status epilepticus
a seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.
How should status epilepticus be managed?
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
What is an extradural haemorrhage?
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.
How does an extradural haemorrhage present on CT?
bi-convex shape, does not cross cranial suture lines
What is the immediate mx of a suspected bleed on the brain?
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
What is the surgical mx of an intracranial haemorrhage?
Craniotomy (open surgery by removing a section of the skull)
Burr holes (small holes drilled in the skull to drain the blood)
What is a subdural haemorrhage?
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
How does a subdural haemorrhage present on CT?
they have a crescent shape and are not limited by the cranial sutures (they can cross over the sutures lines)
What is a subarachnoid haemorrhage?
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.
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?
Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine
What are the 5 stages of migraine?
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
How do migraines usually present?
Usually unilateral but can be bilateral
Pounding or throbbing pain
Photophobia
Phonophobia
Osmophobia (discomfort with strong smells)
Aura (visual changes)
Nausea and vomiting
What is aura?
acute sensory disturbance
Sparks in the vision
Blurred vision
Lines across the vision
Loss of visual fields (e.g., scotoma)
How do hemiplegic migraines present?
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
What can trigger migraines?
Stress
Bright lights
Strong smells
Certain foods (e.g., chocolate, cheese and caffeine)
Dehydration
Menstruation
Disrupted sleep
Mx options for an acute migraine attack?
NSAIDs (e.g., ibuprofen or naproxen)
Paracetamol
Triptans (e.g., sumatriptan)
Antiemetics if vomiting occurs (e.g., metoclopramide or prochlorperazine)
Triptans are used to abort migraines when they start to develop. What is their MOA?
5-HT receptor agonists (they bind to and stimulate serotonin receptors)
Cause cranial vasoconstriction
Inhibit the transmission of pain signals
What can be used for migraine prophylaxis?
Propranolol (a non-selective beta blocker)
Amitriptyline (a tricyclic antidepressant)
Topiramate (teratogenic and very effective contraception is needed)
What is abdominal migraine?
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
What infections are associated with headache in children?
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
How do tension headaches present? How can they be managed?
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)
What are breath holding spells?
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
What is muscular dystrophy?
an umbrella term for genetic conditions that cause gradual weakening and wasting of muscles.
most common = Duchennes muscular dystrophy
Children with proximal muscle weakness use a specific technique to stand up from a lying position called Gower’s sign: describe it.
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.
What is spinal muscular atrophy?
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
What are the categories of SMA?
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
How can SMA be managed?
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
Give some causes of raised ICP
idiopathic intracranial hypertension
traumatic head injuries
meningitis
tumours
hydrocephalus
Give some features of raised ICP
headache
vomiting
reduced levels of consciousness
papilloedema
Cushing’s triad:
widening pulse pressure
bradycardia
irregular breathing
How can raised ICP be investigated?
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
How can raised ICP be managed?
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