Migraine Subtypes
The ICHD-3 classification recognizes several migraine subtypes, each with distinct clinical features and management implications. Not all migraines are treated the same — subtype identification directly impacts drug selection, contraindications, and workup. This topic focuses on the subtypes that change clinical decision-making.
Bottom Line
- Migraine with aura: Avoid combined estrogen contraceptives (stroke risk); triptans are safe to use during the headache phase
- Hemiplegic migraine: Triptans and ergots traditionally avoided (limited evidence for harm, but consensus-based); preventive therapy is the priority
- Vestibular migraine: Most common cause of episodic vertigo; diagnosis is clinical; treat with standard migraine preventives
- Migraine with brainstem aura: Previously "basilar migraine"; triptans are no longer contraindicated per recent AHS guidance
- Retinal migraine: True monocular visual loss; rare diagnosis of exclusion — must rule out vascular and ophthalmologic causes first
- Status migrainosus: Debilitating attack >72 hours; often requires parenteral therapy (IV DHE, antiemetics, corticosteroids)
Migraine Without Aura
The most common subtype (~70% of migraine patients). Diagnosis requires at least 5 attacks meeting these criteria:
- Attacks lasting 4-72 hours (untreated or unsuccessfully treated)
- At least 2 of: unilateral, pulsating, moderate-severe intensity, aggravated by routine physical activity
- At least 1 of: nausea/vomiting, photophobia and phonophobia
- Not better accounted for by another diagnosis
No special treatment restrictions. All acute and preventive therapies are appropriate based on severity and frequency.
Migraine With Aura
Occurs in ~25-30% of migraine patients. Aura is a fully reversible focal neurological symptom that develops gradually over 5-60 minutes.
ICHD-3 Aura Criteria
- At least 2 attacks with aura
- One or more fully reversible aura symptoms: visual, sensory, speech/language, motor, brainstem, retinal
- At least 3 of:
- At least one aura symptom spreads gradually over ≥5 minutes
- Two or more aura symptoms occur in succession
- Each individual aura symptom lasts 5-60 minutes
- At least one aura symptom is unilateral
- At least one aura symptom is positive (e.g., scintillations, pins and needles)
- Aura accompanied or followed within 60 minutes by headache
Visual aura is by far the most common (>90%): scintillating scotoma, fortification spectra, flickering zigzag lines. Sensory aura (paresthesias migrating up the arm to face) is second most common.
Treatment Implications: Migraine With Aura
- Estrogen-containing contraceptives are contraindicated — migraine with aura + combined OCP increases ischemic stroke risk 6-8 fold (WHO Class 4)
- Progestin-only methods, IUDs, and non-hormonal options are safe
- Triptans are safe during the headache phase; avoid taking during the aura itself (vasoconstriction concern is theoretical; efficacy is poor during aura regardless)
- Aura-specific prevention: limited evidence for lamotrigine reducing aura frequency (small trials)
- PFO closure is not recommended solely for migraine with aura — PREMIUM and PRIMA trials showed no benefit
Hemiplegic Migraine
Defined by aura that includes fully reversible motor weakness, plus at least one other aura symptom (visual, sensory, or speech). Motor symptoms can last hours to days, sometimes mimicking stroke.
Familial Hemiplegic Migraine (FHM)
Autosomal dominant with three known gene mutations:
| Type | Gene | Channel/Protein | Notable Features |
|---|---|---|---|
| FHM1 | CACNA1A | P/Q calcium channel | Most common; may have cerebellar ataxia; allelic with EA2 and SCA6 |
| FHM2 | ATP1A2 | Na+/K+ ATPase | Associated with epilepsy in some families |
| FHM3 | SCN1A | Sodium channel | Rare; same gene as Dravet syndrome |
Sporadic hemiplegic migraine meets the same criteria without a first-degree relative with motor aura. Genetic testing is not required for diagnosis but can be informative.
Treatment Implications: Hemiplegic Migraine
- Triptans and ergots: Traditionally listed as contraindicated due to theoretical vasospasm risk. However, no case reports of harm exist, and some headache specialists do use triptans cautiously. AHS does not make a firm recommendation.
- Preventive therapy is the mainstay: Verapamil, flunarizine (outside US), lamotrigine, and acetazolamide (especially FHM1 with cerebellar features) are commonly used
- CGRP therapies: Limited data; not contraindicated but efficacy in HM specifically is unknown. Trials such as STRIVE and ARISE did not specifically enroll hemiplegic migraine patients.
- Acute attacks with prolonged weakness: May require ED evaluation to rule out stroke; MRI with DWI can differentiate
Vestibular Migraine
The most common cause of episodic vertigo and the second most common vestibular disorder (after BPPV). Prevalence is approximately 1-3% of the general population. It is significantly underdiagnosed.
Diagnostic Criteria (ICHD-3 / Barany Society)
- At least 5 episodes of vestibular symptoms of moderate or severe intensity lasting 5 minutes to 72 hours
- Current or past history of migraine (with or without aura)
- At least 50% of episodes associated with at least one migraine feature: headache, photophobia, phonophobia, or visual aura
- Not better accounted for by another vestibular or ICHD diagnosis
Vestibular symptoms include spontaneous vertigo, positional vertigo, visually induced vertigo, head motion-induced vertigo, and head motion-induced dizziness with nausea.
🔴 Diagnostic Pitfalls
- Headache is not required during every episode — only migraine features in ≥50% of attacks
- Can occur independently of headache phase; some patients have vertigo as their only migraine manifestation
- Must exclude posterior fossa pathology, Ménière disease (audiometric testing), and BPPV (Dix-Hallpike)
- Vestibular testing (VNG, VEMP) is often normal between attacks; abnormalities do not confirm or exclude the diagnosis
Treatment: Vestibular Migraine
- Acute: Triptans (limited data but commonly used), vestibular suppressants (meclizine, ondansetron) for symptom relief
- Prevention: Same agents as migraine prevention — no head-to-head trials specific to VM. Commonly used: venlafaxine, topiramate, propranolol, nortriptyline
- CGRP therapies: Emerging data suggests benefit; post-hoc analyses of STRIVE (erenumab) and REGAIN (galcanezumab) trials showed improvement in vestibular symptoms. No RCT specifically powered for VM yet.
- Vestibular rehabilitation can be a useful adjunct
Migraine With Brainstem Aura
Previously called "basilar-type migraine" or "basilar migraine." The name was changed because the pathophysiology is cortical spreading depression, not basilar artery vasospasm.
Diagnostic Criteria
- Aura with at least two brainstem symptoms (no motor weakness — that would be hemiplegic migraine):
- Dysarthria
- Vertigo
- Tinnitus
- Hypacusis (hearing loss)
- Diplopia
- Ataxia
- Decreased level of consciousness (GCS ≤13)
- At least one other aura symptom (usually visual) that is bilateral
Treatment Implications: Brainstem Aura
- Triptans: Previously contraindicated. The 2019 AHS position statement states triptans may be used in migraine with brainstem aura, as the original contraindication was based on the incorrect "basilar artery spasm" theory
- Standard migraine preventives are appropriate
- Must rule out posterior circulation stroke or TIA, especially in first presentation or older patients
Retinal Migraine
Fully reversible monocular visual disturbance (scintillations, scotoma, or blindness) associated with migraine headache. This is a rare and often overdiagnosed entity.
Diagnostic Criteria
- At least 2 attacks of fully reversible monocular positive and/or negative visual phenomena confirmed by either: patient description or examination during an attack (visual field defect)
- Accompanied or followed within 60 minutes by migraine headache
- Normal ophthalmologic examination between attacks
- Other causes of transient monocular visual loss excluded
🔴 Retinal Migraine: A Diagnosis of Exclusion
- Rule out: carotid stenosis/dissection, GCA (if >50 years), retinal artery/vein occlusion, retinal detachment, optic neuritis
- Requires ophthalmologic evaluation including dilated fundoscopy
- Carotid imaging (CTA or duplex) should be performed
- Most "retinal migraine" referrals turn out to be migraine with typical visual aura perceived as monocular
- True monocular symptoms should always prompt vascular workup before attributing to migraine
Status Migrainosus
A debilitating migraine attack lasting >72 hours continuously, with pain and/or associated symptoms of severe intensity. Brief remissions due to sleep or medication are permitted if the attack otherwise meets criteria.
ICHD-3 Criteria
- Headache attack fulfilling criteria for migraine without aura (or migraine with aura)
- Duration >72 hours
- Pain and/or associated symptoms are debilitating
- Not better accounted for by another diagnosis
Treatment: Status Migrainosus
- IV fluids: Many patients are dehydrated from nausea/vomiting; NS bolus is standard
- Antiemetics: Prochlorperazine 10 mg IV or metoclopramide 10-20 mg IV — also have independent analgesic effect
- IV DHE protocol: Dihydroergotamine 0.5-1 mg IV every 8 hours (with antiemetic pretreatment) for up to 3 days. The DHE IV for Intractable Migraine trial showed 89% became headache-free within 48 hours.
- Corticosteroids: Dexamethasone 10 mg IV (single dose or short course) may reduce recurrence; evidence is mixed
- Ketorolac: 30 mg IV — useful if no contraindications; limit to single dose to avoid MOH contribution
- Avoid: Repeated opioids and barbiturates (contribute to chronification and medication overuse)
🔴 When to Investigate Further
- First episode of prolonged headache or new headache phenotype — consider secondary causes
- Fever, nuchal rigidity, altered mental status — rule out meningitis/encephalitis
- New neurologic deficits — imaging to exclude stroke, CVT, or mass lesion
- Thunderclap onset within the prolonged course — consider SAH, RCVS, or dissection
Subtype Quick Reference
| Subtype | Key Feature | Triptans | Estrogen OCP | Special Considerations |
|---|---|---|---|---|
| Without aura | No focal neuro symptoms | Yes | Yes (WHO Class 2) | No restrictions |
| With aura | Visual/sensory/language aura | Yes | No (WHO Class 4) | Consider lamotrigine for aura frequency |
| Hemiplegic | Motor weakness in aura | Controversial | No | Genetic testing optional; preventives are priority |
| Vestibular | Episodic vertigo | Yes | Per aura status | Most common cause of episodic vertigo; underdiagnosed |
| Brainstem aura | ≥2 brainstem symptoms | Yes (AHS 2019) | No | No longer called "basilar migraine" |
| Retinal | Monocular visual loss | Yes | Caution | Diagnosis of exclusion; needs vascular workup |
| Status migrainosus | Attack >72 hours | Limited utility | Per subtype | Requires parenteral therapy (DHE, antiemetics) |
References
- Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1-211.
- Lempert T, et al. Vestibular migraine: diagnostic criteria (Consensus document of the Barany Society and the IHS). J Vestib Res. 2012;22(4):167-172.
- Burch RC, et al. Migraine: Epidemiology, burden, and comorbidity. Neurol Clin. 2019;37(4):631-649.
- Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol. 2011;10(5):457-470.
- Dodick DW, et al. Consensus statement: cardiovascular safety profile of triptans (5-HT1B/1D agonists) in the acute treatment of migraine. Headache. 2004;44(5):414-425.
- Sheikh HU, et al. Risk of stroke associated with use of estrogen-containing contraceptives in women with migraine: A systematic review. Headache. 2018;58(1):5-21.
- Raskin NH. Repetitive intravenous dihydroergotamine as therapy for intractable migraine. Neurology. 1986;36(7):995-997.
- Goadsby PJ, et al. A Controlled Trial of Erenumab for Episodic Migraine (STRIVE). N Engl J Med. 2017;377(22):2123-2132.
- Detke HC, et al. Galcanezumab in chronic migraine (REGAIN). Neurology. 2018;91(24):e2211-e2221.