Most people are probably familiar with the two main types of Migraine – Migraine without Aura and Migraine with Aura, but that barely scratches the surface of the types of Migraine and the subtypes. This can be valuable information, so we’re going to delve into the types of Migraine as well as the subtypes.
At first glance, the various types of Migraine and the subtypes may seem confusing, but when we consider that the breakdown into the various types and subtypes is possible only because of increased research, it’s easier to recognize the progress made in the field. For Migraine and other Headache disorders, the gold standard for diagnosis and classification is the International Headache Society’s International Classification of Headache Disorders (ICHD), now in its third edition (ICHD-3).
Using a standardized system for classifying and diagnosing the types of Migraine is important for a number of reasons:
- It simplifies things enormously when we need to see a specialist or other doctor other than our regular doctor. It serves to keep everyone on the same page.
- It helps us find the correct information when we’re educating ourselves about our Migraines.
- It allows us to have more productive conversations with others.
Standardized diagnostic and classification criteria avoids the all-out confusion that would ensue if everyone used different terms and various people meant different things when they discussed a particular form of Migraine. We often see that kind of confusion now when people who don’t conform to the standardized criteria talk about ocular, optical, ophthalmic, or eye Migraines.
The ICHD-3 recognizes the following types and subtypes of Migraine:
1.1 Migraine without aura
Recurrent headache disorder manifesting in attacks lasting 4-72 hours. Typical characteristics of the headache are unilateral location, pulsating quality, moderate or severe intensity, aggravation by routine physical activity and association with nausea and/or photophobia and phonophobia.
1.2 Migraine with aura
Recurrent attacks, lasting minutes, of unilateral fully reversible visual, sensory or other central nervous system symptoms that usually develop gradually and are usually followed by headache and associated Migraine symptoms.
1.2.1 Migraine with typical aura
Migraine with aura in which aura consists of visual and/or sensory and/or speech/language symptoms, but no motor weakness, and is characterized by gradual development, duration of each symptom no longer than 1 hour, a mix of positive and negative features and complete reversibility.
1.2.1.1 Typical aura with headache
Migraine with typical aura in which aura is accompanied or followed within 60 minutes by headache with or without Migraine characteristics.
1.2.1.2 Typical aura without headache
Migraine with typical aura in which aura is neither accompanied nor followed by headache of any sort.
1.2.2 Migraine with brainstem aura
Migraine with aura symptoms clearly originating from the brainstem, but no motor weakness.
1.2.3 Hemiplegic Migraine
Migraine with aura including motor weakness.
1.2.3.1 Familial hemiplegic Migraine (FHM)
Migraine with aura including motor weakness, and at least one first- or second-degree relative has Migraine aura including motor weakness.
1.2.3.1.1 Familial hemiplegic Migraine type 1
Form of FHM determined by a mutation on the CACNA1A gene
1.2.3.1.2 Familial hemiplegic Migraine type 2
Form of FHM determined by a mutation on the ATP1A2 gene
1.2.3.1.3 Familial hemiplegic Migraine type 3
Form of FHM determined by a mutation on the SCN1A gene
1.2.3.1.4 Familial hemiplegic Migraine, other loci
Form of FHM in which genetic testing has demonstrated no mutation on the CACNA1A, ATP1A2 or SCN1A genes
1.2.3.2 Sporadic hemiplegic Migraine
Migraine with aura including motor weakness, and no first- or second-degree relative has Migraine aura including motor weakness.
1.2.4 Retinal Migraine
Repeated attacks of monocular visual disturbance, including scintillations, scotomata or blindness, associated with Migraine headache.
1.3 Chronic Migraine
Headache occurring on 15 or more days per month for more than 3 months, which has the features of Migraine headache on at least 8 days per month.
1.4 Complications of Migraine
Code separately for both the Migraine subtype and for the complication.
1.4.1 Status migrainosus
A debilitating Migraine attack lasting for more than 72 hours.
1.4.2 Persistent aura without infarction
Aura symptoms persisting for 1 week or more without evidence of infarction on neuroimaging.
1.4.3 Migrainous infarction
One or more Migraine aura symptoms associated with an ischaemic brain lesion in the appropriate territory demonstrated by neuroimaging.
1.4.4 Migraine aura-triggered seizure
A seizure triggered by an attack of Migraine with aura.
1.5 Probable Migraine
1.5.1 Probable Migraine without aura
1.5.2 Probable Migraine with aura
1.6 Episodic syndromes that may be associated with Migraine
1.6.1 Recurrent gastrointestinal disturbance
Recurrent episodic attacks of abdominal pain and/or discomfort, nausea and/or vomiting, occurring infrequently, chronically or at predictable intervals, that may be associated with Migraine.
1.6.1.1 Cyclical vomiting syndrome
Recurrent episodic attacks of intense nausea and vomiting, usually stereotypical in the individual and with predictable timing of episodes. Attacks may be associated with pallor and lethargy. There is complete resolution of symptoms between attacks.
1.6.1.2 Abdominal Migraine
An idiopathic disorder seen mainly in children as recurrent attacks of moderate to severe midline abdominal pain, associated with vasomotor symptoms, nausea and vomiting, lasting 2–72 hours and with normality between episodes. Headache does not occur during these episodes.
1.6.2 Benign paroxysmal vertigo
A disorder characterized by recurrent brief attacks of vertigo, occurring without warning and resolving spontaneously, in otherwise healthy children.
1.6.3 Benign paroxysmal torticollis
Recurrent episodes of head tilt to one side, perhaps with slight rotation, which remit spontaneously. The condition occurs in infants and small children, with onset in the first year.
A1.6.6 Vestibular Migraine*
Previously used terms: Migraine-associated vertigo/dizziness; migraine-related vestibulopathy; migrainous vertigo.
* Vestibular Migraine is found in the appendix of ICHD-3. Entities in the appendix are explained in this way, “The primary purpose of the Appendix is to present research criteria for a number of novel entities that have not been sufficiently validated by research conducted so far. The experience of the experts in the Classification Committee, and publications of variable quality, suggest that there are still a number of diagnostic entities that are believed to be real but for which better scientific evidence must be presented before they can be formally accepted. Therefore, as has happened between ICHD-II, ICHD-3 beta and ICHD-3, it is anticipated that some disorders now in the Appendix will move into the main body of the classification at the next revision.”
A diagnosis of “Migraine” is an incomplete diagnosis. A complete diagnosis includes the type or types of Migraine we have, and it’s not unusual to have more than one type. People who have Migraine with aura, seldom have an aura with every Migraine attack, so most people who are diagnosed with Migraine with aura are also diagnosed with Migraine without aura. Chronic Migraine is also an incomplete diagnosis. Here too, a complete diagnosis includes the type or types of Migraine.
If your doctor can’t or won’t give you a complete diagnosis, it’s possible that they simply aren’t experienced or knowledgeable enough about Migraine disease. Doctors are taught very little about Migraine in medical school. If you’re having problems getting an accurate and complete diagnosis, it may be time to seek care with a Migraine specialist. For more information on this, please see Finding a Migraine Specialist: Why, How, and Where.
Sources
- 1. Headache Classification Committee of the International Headache Society. “The International Classification of Headache Disorders, 3rd Edition (ICHD-3).” Cephalalgia, Volume: 38 issue: 1, page(s): 1-211.