Neurological disorders

Question 1

What are the clinical features of migraines?

Answer 1

Without aura:
• 90% of migraines
• Often bilateral, but can be unilateral
• Pulsatile, over frontal or temporal area
• Accompanied by GI symptoms (Nausea, vomiting, abdominal pain)
• Photophobia & phonophobia
• Aggravated by physical activity, better with sleep

With aura:
• Presents with visual, sensory or motor aura
• May last for hours
• Commonly visual auras:
• Positive visual = Fortification of spectra (zigzag)
• Negative visual = Hemianopia
• Relieved by lying in quiet dark room

Question 2

What is the management of migraines?

Answer 2

If not severe, simple analgesia
Consider keeping a migraine diary for 8 weeks to identify triggers

If severe, and above 12, prescribe NASAL sumatriptan and analgesia
• NOTE: People <18 cannot have oral sumatriptan

Question 3

What are the clinical features of febrile seizures?

Answer 3

An epileptic seizure accompanied by a fever, in the absence of intracranial infection
Commonly affects 6 month - 6 year olds
Tends to occur early in a viral infection when temperatures are still rising
HHV6 is most common virus assosiated with febrile seizures

Question 4

What is the management of seizures?

Answer 4

If it has not ceased after 5 minutes = Buccal midazolam/Rectal diazepam
Repeat if it has not stopped after 5 minutes of taking medication

Call ambulance if it hasn’t stopped after 10 minutes of taking first dose or if there is ongoing twitching

Measure blood glucose if unrousable

Question 5

Which patients suffering from a seizure require paediatrician assessment?

Answer 5

§ First febrile seizure
§ Second seizure if not been assessed before
§ Diagnostic uncertainty about the cause
§ Seizure lasted > 15 mins
§ Focal features during the seizure
§ Seizure recurred in the same febrile illness (or within 24 hours)
§ Incomplete recovery after 1 hour
§ < 18 months old
§ No serious clinical findings but is currently taking antibiotics
§ Parents are anxious and cannot cope
§ Suspected cause of the fever (e.g. pneumonia)

Question 6

Which epileptic drug is given for each type of seizure?
Generalised: Tonic-clonic, absence, myoclonic
Focal

Answer 6

Tonic-clonic = Valproate
Absence = Ethosuximide or valproate
Myoclonic = Valproate

Focal = Carbamezapine or Lamotrigine

Question 7

What investigations are conducted for epilepsy?

Answer 7

Based on history
Clinical examination should involve looking for skin markers of neurocutaneous syndromes

Investigations are carried out to look for other causes of seizures:
○ ECG
§ Should be done in ALL children with seizures
§ You do NOT want to miss convulsive syncope due to an arrhythmia (e.g. long-QT syndrome)
○ EEG
§ Inter-ictal EEG can help categorise the epilepsy type and severity
○ Brain Imaging
§ Structural
□ MRI and CT are required routinely in childhood epilepsies
§ Functional
□ Abnormal metabolism may be suggestive of epileptogenic zones
□ PET and SPECT can identify metabolically active cells

Question 8

What are the clinical features of Duchenne’s muscular dystrophy?

Answer 8

X-linked recessive
Lack of dystrophin, leading to myofibre necrosis
Typically presents at age 5
	• Waddling gait
	• Language delay
	• Must go prone to stand (Gower's sign)
	• Mount stairs one and a time

Investigation:
Creatine Kinase = HIGH

Question 9

What is the management of Duchenne’s muscular dystrophy?

Answer 9

§ Physiotherapy helps prevent contractures
§ Exercise and psychological support
§ Tendoachilles lengthening and scoliosis surgery
§ Weakness of intercostal muscles may lead to nocturnal hypoxia
□ Presents with daytime headache, irritability and loss of appetite
□ Overnight CPAP may help
§ Glucocorticoids (e.g. prednisolone) may help delay wheelchair dependence

Prognosis: Most die by 20-30 due to respiratory failure

Question 10

What’s the difference between Duchenne’s and Becker Muscular dystrophy?

Answer 10

Both same disorder, but different mutation
Becker is more mild
□ Later onset
□ Live to middle-old age

Question 11

What are the clinical features of extradural haematoma?

Answer 11

Usually follows head trauma
• Lucid interval
• Conscious level deteriorates
• Seizures secondary to increasing size of haematoma
• Focal neurological signs:
□ Dilation of ipsilateral pupil
□ Paresis of contralateral limbs
□ False localising unilateral or bilateral VIth nerve palsies
• NOTE: in young children, the presentation may be anaemia and shock

Investigation:
• CT scan

Question 12

What is the management of extradural haematoma?

Answer 12

• Correct hypovolaemia
  
  • Urgent evacuation of haematoma
  • Arrest bleeding

Question 13

What are the clinical features of hydrocephalus?

Answer 13

Causes of non-communicating hydrocephalus (blockage in CSF flow):
• Aqueduct stenosis
• Atresia of the ventricle

Causes of communicating hydrocephalus (Failure to absorb at arachnoid villi):
• Subarachnoid haemorrhage
• Meningitis

Features:
• In infants who’s skull has not fused, increased head circumference
• Bulging anterior fontanelle
• Fixed downward gaze (Sunset sign)
• Older children will have signs of raised ICP

Investigations:
• Antenatal screening
• Cranial ultrasound
• CT/MRI

Question 14

What is the management of hydrocephalus?

Answer 14

Insertion of ventriculoperitoneal shunt for drainage

Question 15

What are the clinical features of infantile spasms?

Answer 15

Characteristic seizure of West syndrome
Onset = 1 month - 1 year
Characterised by:
• Infantile spasm (Referred to as Salaam attacks, violent flexor spasms of head, trunk and limbs, followed by extension of arms)
□ Only lasts for seconds, confused for colic
• Developmental plateau
• Hypsarrhythmia (Very random electrical pattern on EEG)

Management = Vigabatrin or corticosteroids