Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 24-01: Headache + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Headache, usually pulsatile, lasting 4 to 72 hours Pain typically, but not always, unilateral May be accompanied by Nausea Vomiting Photophobia Phonophobia Pain is aggravated with routine physical activity Classically, an aura of transient neurologic symptoms (typically visual) may precede head pain Commonly, head pain occurs with no aura +++ General Considerations ++ Pathophysiology of migraine probably relates to neuronal dysfunction in the trigeminal system resulting in release of vasoactive neuropeptides such as calcitonin gene-related peptide leading to neurogenic inflammation, sensitization, and headache Migraine aura is hypothesized to result from cortical spreading depression, a wave of neuronal and glial depolarization that moves slowly across the cerebral cortex corresponding to the clinical symptoms (ie, occipital cortex and visual aura) +++ Demographics ++ Migraine often exhibits a complex, polygenic pattern of inheritance Sometimes, an autosomal dominant inheritance pattern is apparent, as in familial hemiplegic migraine (FHM), in which attacks of lateralized weakness represent the aura + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs +++ Typical migraine ++ May be lateralized or generalized May be dull or throbbing Sometimes associated with Anorexia, nausea, vomiting Photophobia, phonophobia, osmophobia Cognitive impairment Blurring of vision Usually builds up gradually and may last for several hours or longer Visual disturbances occur quite commonly and may consist of Field defects Luminous visual hallucinations, such as stars, sparks, unformed light flashes (photopsia), geometric patterns, or zigzags of light Some combination of field defects and luminous hallucinations (scintillating scotomas) Migraine with aura may be a risk factor for stroke Other focal disturbances Aphasia or numbness Paresthesias Clumsiness Dysarthria Dysequilibrium Weakness in a circumscribed distribution +++ Common migraine ++ Commonly bilateral and periorbital Visual disturbances and other focal neurologic deficits do not occur +++ Basilar artery migraine ++ Blindness or disturbances throughout both visual fields are accompanied or followed by Dysarthria Dysequilibrium Tinnitus Perioral and distal paresthesias Blindness or visual disturbances sometimes followed by Transient loss or impairment of consciousness or a confusional state This, in turn, is followed by a throbbing (usually occipital) headache, often with nausea and vomiting +++ Ophthalmoplegic migraine ++ Lateralized pain, often about the eye, is accompanied by Nausea Vomiting Diplopia due to transient external ophthalmoplegia Due to third nerve palsy, sometimes with accompanying sixth nerve involvement, and may outlast the orbital pain by several days or even weeks Ophthalmic division of the fifth nerve has also been affected in some patients +++ Migraine equivalent ++ In rare instances, the neurologic or somatic disturbance accompanying typical migrainous headaches becomes the sole manifestation of an attack without headaches occurring ("migraine equivalent") +++ Familial hemiplegic migraine (FHM) ++ Attacks of lateralized weakness represent aura +++ Sporadic hemiplegic migraine ++ No other family members are affected +++ Differential Diagnosis ++ Other causes of headache Cluster headache Brain tumor Temporal (giant cell) arteritis Sinusitis Subarachnoid hemorrhage Pseudotumor cerebri Transient ischemic attack + Diagnosis Download Section PDF Listen +++ +++ Imaging Studies ++ Changes in brainstem regions involved in sensory modulation may be seen, suggesting that migraine relates to failure of normal sensory processing +++ Diagnostic Procedures ++ Inquire about precipitating and exacerbating factors Inquire about family history + Treatment Download Section PDF Listen +++ +++ Medications ++ A simple analgesic (eg, aspirin) taken immediately often provides relief However, treatment with extracranial vasoconstrictors or other drugs is sometimes necessary Cafergot, a combination of ergotamine tartrate (1 mg) and caffeine (100 mg), is often particularly helpful One or two tablets are taken at the onset of headache or warning symptoms, followed by one tablet every 30 min If necessary, up to six tablets per attack can be taken but no more than 10 tablets per week Ergotamine-containing preparations should be avoided During pregnancy When cardiovascular disease or risk factors are present In patients taking potent CYP 3A4 inhibitors (clarithromycin, telithromycin, itraconazole, ketoconazole, nefazodone, atanazavir, darunavir, indinavir, lopinavir, nelfinavir, ritonavir, saquinavir, tipranavir) Sumatriptan is a rapidly effective agent for aborting attacks when given subcutaneously by an autoinjection device (4–6 mg once subcutaneously, may repeat once after 2 hours if needed; maximum dose 12 mg/24 h) Zolmitriptan is effective for acute treatment Optimal initial oral dose is 5 mg Relief usually occurs within 1 hour May repeat once after 2 hours if needed Maximum dose is 10 mg/24h Can also be taken by nasal spray 5 mg in one nostril once; may repeat once after 2 hours Maximum dose is 10 mg/24h Other triptans are available Rizatriptan (5–10 mg orally at onset, may repeat every 2 hours twice (maximum dose 30 mg/24 h) Naratriptan (1–2.5 mg orally at onset, may repeat once after 4 hours (maximum dose 5 mg/24 h) Almotriptan (6.25–12.5 mg orally at onset, may repeat dose once after 2 hours (maximum dose 25 mg/24 h) Eletriptan (20–40 mg orally at onset, may repeat after 2 hours once (maximum dose 80 mg/24 h) Frovatriptan (2.5 mg orally at onset, may repeat after 2 hours once) has longer half-life than eletriptan; helps patients with prolonged attacks (maximum dose 7.5 mg/24 h) or attacks provoked by menstrual periods Triptans Should be avoided in pregnancy and in patients with risk factors for stroke Are contraindicated in patients with coronary or peripheral vascular disease May cause nausea or vomiting Lasmiditan Dosage: 50–200 mg taken once at headache onset; no more than one dose in 24 hours Lacks the vasoconstrictive properties of triptans Can be given safely to patients with cardiovascular risk factors Side effects: dizziness and somnolence Patients should not drive within 8 hours of administration Opioid analgesics are sometimes required when other therapies fail Intravenous propofol in subanesthetic doses may help in intractable cases When oral medication fails to help due to impaired absorption or vomiting, the following regimens may be used Cafergot given rectally (one-half to one suppository containing 2 mg of ergotamine) Dihydroergotamine mesylate (0.5–1 mg intravenously or 1–2 mg subcutaneously or intramuscularly) Prochlorperazine administered rectally (25 mg suppository) or intravenously or intramuscularly (5–10 mg), or orally (5–10 mg every 6–8 h) Intravenous metoclopramide (10–20 mg) or chlorpromazine (25–50 mg) is useful in the emergency department setting Erenumab FDA approved for the preventive treatment of migraine in adults Dosage: 70 mg subcutaneously once a month Some patients may benefit from 140 mg subcutaneously once a month A dose of 140 mg should be administered as 2 consecutive subcutaneous injections of 70 mg each +++ Therapeutic Procedures ++ Management of migraine consists of avoidance of any precipitating factors, together with prophylactic or symptomatic pharmacologic treatment if necessary During acute attacks, many patients find it helpful to rest in a quiet, darkened room until symptoms subside Transcranial magnetic stimulation Effective in aborting migraine with aura in one sham-controlled trial A handheld device is approved in the United States and European Union but is costly Some other neurostimulation techniques look promising, including single-pulse transcranial magnetic stimulation, vagus nerve stimulators, and implantable occipital nerve stimulation, but critical appraisal is necessary In chronic migraine (at least 15 days per month with headaches lasting 4 hours per day or longer) Acupuncture is as effective as prophylactic pharmacologic treatment Botulinum toxin type A reduces headache frequency + Outcome Download Section PDF Listen +++ +++ Complications ++ Serotonin syndrome (potentially fatal agitation, confusion, fever, incoordination, vomiting, tachycardia, alterations in blood pressure) may develop when triptans are used with selective serotonin or serotonin/norepinephrine reuptake inhibitors Very rarely, the patient may be left with a permanent neurologic deficit following a migrainous attack +++ Prevention ++ Prophylactic treatment may be necessary if migraine headaches occur more frequently than two or three times a month (Table 24–1) Several drugs may have to be tried in turn before the headaches are brought under control Once a drug has been found to help, it should be continued for several months If the patient remains headache free, the dose can then be tapered and the drug eventually withdrawn Botulinum toxin type A, injected into specific head and neck muscles, may prevent migraines Acupuncture is as effective as prophylactic drug treatment with more rapid pain relief and fewer side effects Some neurostimulation techniques look promising, including occipital nerve stimulation + References Download Section PDF Listen +++ + +Bertels Z et al. Emerging treatment targets for migraine and other headaches. Headache. 2019 Jul;59 (Suppl 2):50–65. [PubMed: 31291018] + +Halker Singh RB et al. Neuromodulation for the acute and preventive therapy of migraine and cluster headache. Headache. 2019 Jul;59 (Suppl 2):33–49. [PubMed: 31291017] + +Puledda F et al. An update on migraine: current understanding and future directions. J Neurol. 2017 Sep;264(9):2031–9. [PubMed: 28321564] + +Santos-Lasaosa S et al. Calcitonin gene-related peptide in migraine: from pathophysiology to treatment. Neurologia. 2019 Jul 17. [Epub ahead of print] [PubMed: 31326215] + +Vukovic-Cvetkovic V et al. Neurostimulation for the treatment of chronic migraine and cluster headache. Acta Neurol Scand. 2019 Jan;139(1):4–17. [PubMed: 30291633]