NeurologyTier 1Disease (DEADMAN)

Migraine and common headache disorders

Description

The primary headaches

	Duration	Character	Key features
Migraine without aura	4-72 h	Unilateral, pulsating, moderate-severe, worse on activity	Nausea/vomiting OR photophobia + phonophobia; prodrome, postdrome
Migraine with aura	Aura 5-60 min, fully reversible	Visual > sensory > speech	Positive then negative phenomena, spreading over minutes
Tension-type	30 min - 7 days	Bilateral, pressing/tightening, mild-moderate, NOT worse on activity	No nausea/vomiting; at most one of photo/phonophobia
Cluster headache	15-180 min, 1-8/day	Strictly unilateral, orbital/supraorbital, excruciating, boring	Ipsilateral autonomic features + agitation/restlessness; circadian and circannual clustering
Medication overuse headache	>=15 days/month	Variable, often daily	Regular overuse >3 months of an acute agent

Chronic migraine = headache >=15 days/month for >3 months, of which >=8 have migraine features
Trigeminal autonomic cephalalgias (TACs) - separated by attack duration and frequency
- Cluster 15-180 min | Paroxysmal hemicrania 2-30 min, absolute response to indomethacin | SUNCT/SUNA 1-600 s, very frequent | Hemicrania continua continuous, indomethacin-responsive
Trigeminal neuralgia - paroxysms of seconds, electric, V2/V3, cutaneous triggers

Migraine aura - what it actually looks like

Visual (~90%): scintillating scotoma, fortification spectra, zigzags expanding across a hemifield over 20-30 min
Sensory: unilateral paraesthesia marching hand -> face over minutes
Speech/language: dysphasia
Brainstem aura: vertigo, diplopia, dysarthria, tinnitus, dec consciousness (previously "basilar migraine")
Hemiplegic migraine: motor weakness, familial (CACNA1A, ATP1A2, SCN1A)
*Aura evolves over minutes and marches; a stroke is maximal at onset. That, plus positive symptoms, is the discrimination*

Epidemiology

Migraine ~15% of adults; F>M ~3:1 after puberty (M=F before)
- Peak 25-55 yrs - the most productive decades; the leading cause of years lived with disability in people under 50
Migraine with aura ~1/3 of migraineurs
Chronic migraine ~2%; medication overuse headache ~1-2% of the population
Tension-type headache - commonest headache overall, lifetime prevalence up to 70%
Cluster headache ~0.1%; M>F ~3:1; onset 20-40; strongly associated with smoking

Aetiopathogenesis

Migraine mechanism

A primary brain disorder of sensory processing, not a vascular disease
Cortical spreading depression - a wave of neuronal depolarisation followed by suppression spreading at ~3 mm/min -> the aura
Trigeminovascular activation -> release of CGRP and PACAP from trigeminal afferents -> neurogenic inflammation, meningeal vasodilatation and nociception
- *CGRP is the therapeutic target - and the reason gepants and CGRP monoclonals work*
Brainstem/hypothalamic generator (dorsal pons, hypothalamus) active in the premonitory phase - explains yawning, food craving, mood change hours before the pain
Central sensitisation -> cutaneous allodynia (scalp tenderness, cannot bear glasses or a ponytail)
- Once allodynia is established, triptans work far less well - treat early
Polygenic; CACNA1A, ATP1A2, SCN1A in familial hemiplegic migraine

Triggers - the ones worth asking about

Sleep change (too little or too much), missed meals, dehydration, stress and post-stress "let-down"
Menstruation (oestrogen withdrawal), alcohol (red wine), caffeine excess or withdrawal
Bright/flickering light, strong smells, weather change
Dietary triggers are less consistent than patients believe; the premonitory phase causes food craving, so the "chocolate trigger" is often the migraine itself

Drugs reported to worsen migraine

Combined oral contraceptives and hormone replacement therapy
Decongestants (also a cause of rebound headache)
SSRIs
Proton pump inhibitors
Vasodilators - GTN, dipyridamole, sildenafil, CCBs
And any acute headache drug taken too often -> medication overuse headache

Diagnosis

Clinical - based on pattern, and on a normal examination
Migraine is under-diagnosed as "sinus" or "tension" headache; most self-diagnosed sinus headache is migraine

SNOOP red flags - when to image


S	Systemic - fever, weight loss, cancer, HIV/immunosuppression, pregnancy
N	Neurological signs, seizure, dec conscious state, papilloedema
O	Onset - thunderclap, maximal in <1 min (SAH, RCVS, dissection, pituitary apoplexy, CVST)
O	Older - new headache >50 yrs (GCA, tumour)
P	Pattern change, positional (low CSF pressure, colloid cyst), precipitated by Valsalva/exertion, papilloedema, pregnancy/puerperium

Add: new headache in a patient with cancer or immunosuppression; anticoagulation; jaw claudication and scalp tenderness (GCA - ESR/CRP)
A normal neurological examination plus a stable, typical pattern needs no imaging

Investigations

None in typical migraine
MRI brain if red flags; MRV if papilloedema or puerperal headache (CVST); CTA for thunderclap
ESR/CRP in anyone over 50 with new headache
LP after imaging if SAH, meningitis or raised/low pressure suspected; record the opening pressure
Cluster: MRI brain with pituitary views at least once - secondary TACs exist

Headache diary

The single most useful investigation - headache days, acute medication days, menstrual relationship, triggers
Defines chronic migraine, detects medication overuse, and measures response to preventives

Management

A. Migraine - acute

*Treat early, at full dose, at the first sign of pain - once allodynia is established, response is poor*
Simple analgesia: aspirin 900 mg or ibuprofen 400-600 mg or naproxen; paracetamol is weaker
Antiemetic even without vomiting - metoclopramide 10 mg or prochlorperazine; treats nausea and gastric stasis and adds analgesic effect
Triptans (5-HT1B/1D agonists) if simple analgesia fails
- Sumatriptan 50-100 mg PO; nasal or subcutaneous if vomiting or rapid onset needed
- Failure of one triptan does not predict failure of another - try at least 2-3 different triptans before abandoning the class
- Contraindicated in ischaemic heart disease, uncontrolled hypertension, cerebrovascular disease, hemiplegic and brainstem aura**
When triptans fail or are contraindicated
- Gepants (CGRP receptor antagonists) - rimegepant is the agent available in Australia (acute and preventive); ubrogepant/zavegepant elsewhere
- Lasmiditan (5-HT1F agonist) - no vasoconstriction, so usable in vascular disease; driving restriction after dosing
*Limit acute treatment to <10 days/month for triptans/combination analgesics and <15 days/month for simple analgesics*
Avoid opioids and butalbital - poor efficacy, high medication overuse and chronification risk

B. Migraine - prevention

Offer if >=4 migraine days/month, disabling attacks, or acute treatment failing/overused
Set expectations: success = 50% reduction in migraine days; allow 8-12 weeks at target dose

Agent	Best when	Watch
Propranolol	Hypertension, anxiety	Asthma, bradycardia, fatigue; avoid in aura with vascular risk
Amitriptyline	Comorbid insomnia, tension-type, depression	Anticholinergic, weight gain, sedation
Candesartan	Hypertension	Renal, potassium
Topiramate	Obesity	Teratogenic - cleft palate; cognitive slowing, paraesthesia, renal stones, glaucoma; interacts with the COCP
Sodium valproate	Effective, but	*Avoid in anyone of childbearing potential - neural tube defects and neurodevelopmental impairment; only under a pregnancy prevention programme*
Pizotifen	Children	Weight gain, sedation
Verapamil	Cluster	ECG monitoring

CGRP monoclonal antibodies - erenumab (receptor), galcanezumab, fremanezumab, eptinezumab (ligand)
- Monthly or quarterly injection; well tolerated, constipation with erenumab; work within 1 month
- PBS-subsidised in Australia for chronic migraine after failure of at least 3 preventive agents - the criteria, not the evidence, are what limit access
Atogepant and rimegepant - oral preventives; rimegepant is TGA-approved and available in Australia; atogepant approved but not yet marketed at the time of writing
Botulinum toxin A (PREEMPT protocol) for chronic migraine only - not episodic
Menstrual migraine: perimenstrual naproxen or long-acting triptan (frovatriptan) for 5-6 days, or continuous COCP to avoid the hormone-free interval (only if no aura)
Non-drug: aerobic exercise, regular sleep and meals, CBT and relaxation, riboflavin 400 mg, magnesium, coenzyme Q10

C. Medication overuse headache

Diagnose it before escalating preventives - otherwise nothing will work
Withdraw the offending agent (abrupt for simple analgesics and triptans; taper opioids and barbiturates)
Warn about 1-2 weeks of rebound worsening; bridge with naproxen, an antiemetic, or a short prednisolone course
Start a preventive at the same time - CGRP monoclonals are effective even without withdrawal
Follow up at 2-4 weeks; relapse ~30%

D. Cluster headache

Acute
- Subcutaneous sumatriptan 6 mg - the most effective single treatment
- High-flow 100% oxygen 12-15 L/min via a non-rebreather mask, started within 15 min of onset, for 15-20 min
- Intranasal zolmitriptan/sumatriptan; intranasal or dripped lidocaine into the ipsilateral nostril
- Oral triptans and simple analgesics are too slow to be useful
Transitional - prednisolone 50-60 mg daily tapered over 2-3 weeks, or a greater occipital nerve block (fast, effective, and often overlooked)
Preventive
- Verapamil 360-560 mg/day - first-line, with ECG before and with each dose increase (heart block)
- Lithium, topiramate
- Galcanezumab is now approved for prevention of episodic cluster headache
Smoking cessation and alcohol avoidance during a bout
Refractory: occipital nerve stimulation, sphenopalatine ganglion procedures

E. Other primary headaches

Tension-type: simple analgesia, limit to <15 days/month; amitriptyline for chronic form; posture, stress, exercise
Paroxysmal hemicrania and hemicrania continua: indomethacin - the response is absolute and diagnostic (with a PPI)
Trigeminal neuralgia: carbamazepine first-line; oxcarbazepine, lamotrigine, baclofen; MRI to exclude a structural cause and to look for neurovascular conflict; microvascular decompression or gamma knife if refractory

Associations

Migraine with aura specifically

Patent foramen ovale
Ischaemic stroke - roughly doubled risk, and multiplied further by smoking and combined oral contraceptives
- *Combined hormonal contraception is contraindicated in migraine with aura* - progestogen-only or non-hormonal instead
Restless legs syndrome
Parkinson disease
Bipolar disorder and panic disorder

Migraine generally

Depression and anxiety (bidirectional), fibromyalgia, IBS, chronic fatigue
Epilepsy, patent foramen ovale, hypertension
Asthma and allergic disease
CADASIL (migraine with aura + early stroke + white matter change + family history), MELAS, antiphospholipid syndrome
Endometriosis; obesity (a risk factor for chronification)

Cluster headache

Heavy smoking (>80%), alcohol as an in-bout trigger
Obstructive sleep apnoea, depression and elevated suicide risk ("suicide headache")

Natural history & complications

Migraine typically improves after menopause and with age; attacks may persist as aura without headache in later life
Chronification affects ~2.5%/year of episodic migraineurs
- Risk factors: high attack frequency, acute medication overuse, obesity, depression, snoring/OSA, stressful life events, allodynia, female sex, low socioeconomic status
- Reversion to episodic is achievable - treat medication overuse, weight, mood and sleep
Cluster: episodic in ~80-90% (bouts of weeks-months, remission of months-years); ~10-20% become chronic (<3 months remission per year)

Complications

Status migrainosus - a debilitating attack >72 h; treat with IV fluids, metoclopramide/chlorpromazine, ketorolac, dexamethasone (reduces early recurrence); avoid opioids
Migrainous infarction - aura symptoms >60 min with infarction in the corresponding territory; rare
Persistent aura without infarction; migraine aura-triggered seizure
Medication overuse headache
Disability, absenteeism, "presenteeism", loss of income
Depression, anxiety, and suicide risk in cluster headache

Monitor

Headache days and acute medication days per month - the two numbers that matter
Preventive: adequate dose, adequate duration (8-12 weeks), then review
Contraception and pregnancy planning on topiramate and valproate
Blood pressure, weight, mood

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