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“Presiding over the entire attack there will be, in du Bois Reymond’s words, ‘a general feeling of disorder,’ which may be experienced in either physical or emotional terms, and tax or elude the patient’s powers of description.”

—Dr. Oliver Sacks, Migraine

Since antiquity, attempts have been made to describe and classify headache disorders. Hippocrates, the father of modern medicine, described headache disorders in his journals. In the second century AD, physicians like Galen of Pergamon and Aretaeus of Cappadocia described different subtypes of headaches including migraine.1 Despite these attempts, there was no formal classification system until late 20th century. In 1988, the first objective classification system of headache disorders was published by International Headache Society, and it was called the International Classification of Headache Disorders (ICHD). Since its first publication, there have been few revisions to the classification system. The most recent revision of the classification system is ICHD-3, and it was published in 2018, 30 years after the first edition.2 According to the ICHD-3, primary headache disorders are divided into many types and subtypes. We will use ICHD-3 as a framework for this chapter.2 It is important to note that a patient may have more than one subtype of primary headache disorder and all individual diagnoses should be given.


Types of primary headache disorders include the following:

  • Migraine

    • Migraine without aura

    • Migraine with aura

    • Migraine with brainstem aura

    • Migraine with motor aura: hemiplegic migraine

    • Migraine with retinal aura: retinal migraine

    • Complications of migraine:

      • Status migrainosus

      • Migraine with persistent aura

      • Migrainous infarction

      • Migraine aura–triggered seizure: migralepsy

  • Tension-type headache

  • Trigeminal autonomic cephalgia

  • Others


In children, migraine is the most prevalent headache disorder requiring medical attention. It is estimated that 90% of children seeking medical care for headaches have migraine.3 ICHD-3 uses the same criteria for migraine in children as in adults with some flexibility. We will describe the criteria and discuss the different subtypes of migraine. It is important to note that a patient may have more than one subtype of migraine and should be given all diagnoses. If a patient with a diagnosis of migraine has 15 or more headache days per month (any kind of headache) or 8 or more migraine-defining days per month for at least 3 months, the patient is considered to have chronic migraine. It is important to obtain a good history because medication overuse headaches (described in a later section) are a common cause of chronic migraine.

Migraine Without Aura

Migraine is described as a paroxysmal primary headache disorder involving the trigeminovascular system and cascades of events involving various neurotransmitters, resulting in neurogenic inflammation. There are 38 genes found to be associated with migraine.4 A total of 5 headaches with migraine features are required to ...

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