Introduction
Headaches can be divided into primary disorders (commonly migraines) and secondary disorders (commonly caused by a viral illness, but include serious conditions such as raised intracranial pressure, intracranial haemorrhage or infection). Headache may also be a manifestation of underlying mental health, substance abuse or psychosocial issues. Headaches in children reach a peak at approximately 11-13 years of age with migraine and tension type headaches as the most predominant forms1.
The pain of headache is not associated with the brain, meninges or skull as these structures do not have nociception. Perception of pain arises from blood vessels (intra- & extracranial), cranial and spinal nerves, face, skull and neck muscles, and other skull structures (teeth, sinuses and ears).2
Headaches account for approximately 1% of paediatric ED presentations.2 Primary disorders represent about 40% and secondary disorders 60% of all paediatric headache presentations to ED. Serious underlying disease are found in 7-15% of paediatric headache presentations.3
Primary headache disorders
Type | Description | Duration | Frequency |
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Tension headache | - typically, bilateral, dull, deep or band like
- mild to moderate in severity
- not aggravated by exertion
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30 minutes to 7 days Months/years if chronic
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Most common type of headache affecting patients of all ages.
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Migraine without aura | - at least two of the following:
- bilateral or unilateral location
- pulsating
- moderate to severe pain
- made worse with activity
- at least one associated symptom including nausea, vomiting, photophobia and/or phonophobia
|
1- 48 hours
| Most common around 15 years of age (onset age 7 years in boys, 11 years in girls). Prevalence of 3% at age 3-7 years, 4-11% at 7-11 years and 8-23% over 11 years.4 |
Migraine with aura |
An aura (perceptual disturbance that precedes onset of the headache) may consist of:
- visual disturbance (e.g. scintillations, gleam of light, blurred vision, blind spots)
- an odour
- paraesthesia in the hand or face.
Generally consistent for an individual. |
Auras may occur from a few seconds to an hour before headache onset.
|
Cluster headache | - typically occur as 5 or more episodes ranging from every other day to 8 in a single day.
- severe, sharp stabbing pain on one side of the head
- associated with autonomic symptoms (nasal stuffiness, rhinorrhoea, lacrimation, conjunctival injection, Horner’s syndrome) on the side of pain.
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15 minutes to 3 hours
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Rare in children under 12 years of age
|
Migraines
Migraines have a complicated pathophysiology including cranial vasodilation and sensitisation of trigeminovascular pathways. They are classified as simple or complicated based on their clinical presentation.
Complicated migraines include:
- Migraine with brainstem aura- the aura is characterised by vertigo, ataxia, nystagmus, dysarthria, tinnitus/hyperacusis, bilateral parasthesias, diplopia or visual disturbance. The aura can be unilateral or bilateral but does not involve motor weakness and the accompanying headache often is occipital.
- confusional migraine is characterised by altered mental status, often accompanied by aphasia or impaired speech and followed by a headache.
- hemiplegic migraine is rare and is defined as a migraine with aura including motor weakness- characterised by prolonged hemiplegia, visual symptoms numbness, aphasia and confusion.
Episodes of complicated migraine, especially the initial episode, can be dramatic and will usually prompt presentation to an ED. In this initial presentation, complete evaluation including imaging is warranted (preferably MRI and MR angiogram) to exclude other causes including stroke, mass lesions and intoxication.
The criteria for a paediatric migraine diagnosis as defined by The International Headache Society6 includes at least five attacks of migraine without aura as described in table above, or at least two attacks of a migraine with one or more fully reversible aura symptoms which can include visual, sensory, speech and or language, motor, brainstem or retinal features with at least three of the following features:
- at least one aura symptom that gradually spreads over ≥5 minutes
- Two or more aura symptoms occur in succession
- at least one aura symptom is unilateral – aphasia and dysarthria are regarded as this
- aura present for less than one hour, motor symptoms for more than one hour
- At least one aura symptom is positive- scintillations, parasthesia
- headache accompanying or within one hour of aura
Secondary headache disorders
Serious causes of secondary headachesRaised intracranial pressure (mass effect due to tumour/cyst/vascular lesion, cerebral oedema or increase in fluid (CSF/hydrocephalus or blood) or idiopathic intracranial hypertension (see table below)
| - Headache is:
- progressive
- causing night wakening
- worse with exercise and valsalva (sneezing, coughing, toileting)
- associated with persistent vomiting
- may be associated with neurological deficits (including lethargy and personality/behavioural change)
- Specific physical signs can include gait/coordination disturbance, papilloedema, abnormal external ocular movements and pupillary responses (3rd, 4th and 6th nerve palsies).
- Cushings triad (hypertension, bradycardia and respiratory depression) is a late phenomenon.
|
Intracranial infection (including meningitis, encephalitis and brain abscess)
| - Headache is associated with fever, altered mental status, neck stiffness, photophobia, nausea/vomiting, pain with eye movements and neurological deficits.
- More common in a child who is immunosuppressed or has a VP shunt.
|
Intracranial haemorrhage | - The “thunderclap” headache is a classical feature but child typically presents with additional neurological signs.
- Risk factors include congenital heart disease, AV malformations, sepsis, coagulation defects, brain tumours, meningitis, leukaemia, autoimmune disease and sickle cell disease.
|
Trauma | - Post traumatic headaches develop within a week of injury.
- Often associated with post-concussive symptoms (sleep, balance, cognitive and mood changes). Refer to Head Injury Guideline.
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Ischaemic stroke | - Headache associated with sudden onset of unilateral neurological symptoms that persist and do not progress to other side.
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Venous thrombosis | - Rare cause of headache with insidious onset that is usually associated with head/neck infection, severe dehydration or prothrombotic states, and may be associated with abnormal vision or focal weakness.
- Risk factors include arteriopathies (eg. Moya-Moya, sickle cell), cardiac disease (PFO, arrythmia, congenital or acquired heart diseases), hypercoagulable states, chronic head and neck disorders including tumours, sepsis, genetic and metabolic conditions (eg. Trisomy 21, mitochondrial diseases)8
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Other | - Facial and eye conditions (including refractive error, glaucoma, optic neuritis, temporomandibular joint dysfunction, dental caries/abscess and sinusitis) and hypertension.
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Idiopathic intracranial hypertensionDiagnostic Criteria9 Signs and symptoms of raised intracranial pressure - headache
- transient visual disturbance
- pulsatile tinnitus
- gait instability
- papilloedema
Absence of localising neurological signs (excluding cranial nerve palsies) Normal CSF analysis Elevated opening pressure ≥25cmH2O in non sedated or obese children Normal to small ventricles on neuroimaging and absence of any tumour | Associated Conditions Primary IIH Secondary IIH Venous Anomalies & Obstruction - cerebral venous sinus thrombosis
- SVC syndrome
- brachiocephalic vein thrombosis
- increased right heart pressure
- Chiari malfomation
- Intracranial mass or bleed
- Hydrocephalus
- Traumatic brain injury
Endocrine Disorders - Addison’s disease
- hypoparathyroidism
- Hypothyroidism
Drugs - corticosteroids (particularly withdrawal)
- Growth Hormone, Progestogen, Levothyroxine
- cytarabine, cyclosporine
- lithium
- antibiotics – sulfa, tetracycline
- vitamin A and cis-retinoic acid
- Phenytoin
Infectious diseases - CNS infection
- HIV infection
- Lyme disease
- post varicella
Other medical conditions - anaemia
- antiphospholipid antibody syndrome
- Behcet’s disease
- occult craniosynostosis
- sarcoidosis
- sleep apnoea
- systemic lupus erythematosus
- Trisomy 21
- Vitamin A or D deficiency
- MOG antibody disease
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