Central nervous system

Question 1

When is asymmetry of motor skills (i.e. hand dominance) abnormal?

Answer 1

<1 year

Question 2

What system classifies motor functional ability? e.g. in CP, and what are the levels?

Answer 2

Gross Motor Function Classification System (GMFCS)

1: walks without limitations
2: walks with limitations
3: walks using a handheld mobility device
4: self-mobility with limitations; may use powered mobility
5: transported in a manual wheelchair

Question 3

Types of CP

Answer 3

1. Spastic
   - unilateral (hemiplegia)
   - bilateral (quadriplegia)
   - bilateral (diplegia)
2. Dyskinetic
3. Ataxic (hypotonic)

Question 4

Features of spasticity in CP

Answer 4

• velocity-dependent: the faster it’s stretched the greater the resistance
• hallmark of spasticity: ‘dynamic catch’
• clasp knife (increase tone may suddenly yield under pressure)

Question 5

What is unilateral (hemiplegia) spastic CP and the cause?

Answer 5

• damage to UMN

- arm and leg on opposite side to lesion affected

Question 6

What is bilateral (quadriplegia) spastic CP and the cause?

Answer 6

• damage to UMN

- all 4 limbs affected, often severely

Question 7

What is bilateral (diplegia) spastic CP and the cause?

Answer 7

• damage to UMN

- all 4 limbs but legs much more affected than arms

Question 8

What is dyskinetic CP and the cause?

Answer 8

• damage/dysfunction in basal ganglia or associated pathways (extrapyramidal)
• dyskinesia = involuntary, uncontrolled movements
• chorea - irregular, sudden, brief, dance-like movements (non-repetitive)
• athetosis - slow writhing movements distally
• dystonia - simultaneous contraction of agonist and antagonist muscles of trunk and proximal muscles, causing twisting appearance

Question 9

What is ataxic (hypotonic) CP and the cause?

Answer 9

• mostly genetic
• if acquired: cerebellum or its connections
• signs on same side as lesion
• early trunk and limb hypotonia, poor balance, delayed motor movements
• later incoordinate movements, intention tremor, ataxic gait

Question 10

What medications are used to relax muscles in CP?

Answer 10

• diazepam
• baclofen (can be given intrathecally)
• botulinum injections

Question 11

Features of febrile convulsions (consider another diagnosis if these are not present)

Answer 11

• generalised tonic clonic
• <15mins
• without recurrence within next 24h or within same febrile illness

Question 12

Age range for febrile seizures

Answer 12

6 months - 6 years

Question 13

What features suggest a focal frontal seizure?

Answer 13

motor phenomena

Question 14

What features suggest a focal temporal seizure?

Answer 14

auditory or sensory (smell or taste) phenomena

Question 15

What features suggest a focal occipital seizure?

Answer 15

positive or negative visual phenomena

Question 16

What features suggest a focal parietal seizure?

Answer 16

contralateral altered sensation (dysaesthesia)

Question 17

What generalised seizures are there?

Answer 17

• myoclonic
• tonic
• tonic-clonic
• atonic
• absence

Question 18

Investigations for ?epilepsy

Answer 18

• ECG to rule out convulsive syncope due to arrythmia (if any diagnostic uncertainty)
• EEG
• MRI and CT (unless characteristic Hx of certain type)
• metabolic Ix if related to feeds/fasting or developmental arrest/regression
• genetic tests if indicated

Question 19

How to provoke absence seizure

Answer 19

hyperventilation e.g. blowing a toy windmill

Question 20

Long term management of absence seizures

Answer 20

sodium valproate, ethosuxamide or lamotrigine

Question 21

Which children with epilepsy are given rescue medication and what are they given?

Answer 21

• children with prolonged epileptic seizures (convulsive with LOC >5min)
• buccal midazolam (or rectal diazepam)

Question 22

Long term management of tonic clonic seizures

Answer 22

sodium valproate, carbamazepine, lamotrigine

Question 23

Long term management of myoclonic seizures

Answer 23

sodium valproate, levetiracetam, topiramate

Question 24

Long term management of focal seizures

Answer 24

sodium valproate, carbamazepine, levetiracetam, lamotrigine

Question 25

Non-pharmacological Mx options for epilepsy

Answer 25

- ketogenic diet - vagal nerve stimulation - surgery

Question 26

Advice/things to discuss RE epilepsy

Answer 26

- avoid situations where seizure would be dangerous - no driving until 1y seizure free - SUDEP very rare in childhood - self-help groups - liase with school RE managing seizures

Question 27

What is status epilepticus

Answer 27

epileptic seizure lasting 5 mins or repeated epileptic seizures for 5 mins without full recovery of consciousness

Question 28

Management of status epilepticus

Answer 28

0 mins: airway, high flow O2, glucose? 5 mins: IV/IO lorazepam, buccal midazolam or rectal diazepam 15 mins: IV/IO lorazepam, call for senior help, reconfirm it is an epileptic seizure 25 mins: senior help and anaesthetist needed, anaesthetist/ICU advice, consider rectal paraldehyde, phenytoin IV/IO over 20 min (or phenobarbitone over 5 min if already on phenytoin) 45 mins: RSI of anaesthesia with thiopental

Question 29

Who is at risk of developing rebound chronic daily headaches?

Answer 29

people who use analgesics or triptans on >2 days/week

Question 30

When to do Ix/imaging in children's headaches

Answer 30

only if red flags present

Question 31

Prevention of headaches in children

Answer 31

for frequent, intrusive headaches - propranolol - topiramate (or valproate) - amitriptyline

Question 32

Management of headaches in children

Answer 32

- lifestyle e.g. help with stressors; ensuring adequate rest, food, etc. - paracetamol + NSAIDs taken asap in an individual episode - antiemetics e.g. cyclizine - triptans e.g. sumitriptan - nasal preparation in EARLY migraine attack (with paracetamol and NSAID) - physical e.g. cold compress, warm pads, etc.