Transcript Related Guidelines

Status Migrainosus

Randolph W. Evans, MD · Baylor College of Medicine

Disclosures

September 21, 2020

This transcript has been edited for clarity.

The term “status migrainosus” was coined by Tavener in 1978. Status migrainosus or migraine status is defined by ICHD-3 as occurring in a patient with a history of migraine without or with aura and typical of previous attacks, except for duration and severity.[1,2] The attack should be unremitting; more than 72 hours and pain and/or associated symptoms are debilitating.

The prevalence of status migrainosus has been little studied. Estimates range from 3% to about 20% of people with migraine. Risk factors may include emotional stress, depression, medication overuse, menses, and a high attack frequency. A French study found an attack duration ranging from 3 to 10 weeks with an average of 4.8 weeks.

Status migrainosus lasting multiple weeks may be difficult to distinguish from episodic migraine transforming into chronic migraine, new daily persistent headache, and hemicrania continua. At times, status migrainosus may present with a severe sudden or thunderclap onset which has also been called “crash migraine.” A rare disorder is episodic status migrainosus where patients only have attacks of status which may occur in the absence of other attacks of shorter duration or in those with episodic migraine or lower frequency also.[4] Another rare disorder is migraine aura status defined as 3 episodes of aura occurring over 3 consecutive days. One study found a duration ranging from 3 to 5 weeks.[3]

Diagnostic testing should be considered to exclude secondary causes if red flags are present.  There are many secondary mimics of status migrainosus including a hemorrhagic pituitary adenoma, sphenoid sinusitis, RCVS [reversible cerebral vasoconstriction syndrome], SAH [subarachnoid hemorrhage], subdural hematoma, pseudotumor cerebri, brain tumor, intermittent hydrocephalus, meningitis, and acute glaucoma.

The optimal treatment of status has been little studied with an absence of high-quality randomized trials. Outpatient rescue treatments may include sumatriptan SC, ketorolac IM, DHE [dihydroergotamine] nasal spray, butalbital combinations, intranasal lidocaine, opioids, and pericranial nerve blocks. A brief course of corticosteroids may decrease the recurrence but it is not certain that the duration of the attack is shortened and there are uncommon side effects of steroids including aseptic necrosis and sepsis. Emergency department treatments may include sumatriptan SC, IV prochlorperazine, metoclopramide, valproic acid, or ketorolac. In some cases, hospitalization may be indicated for an IV regimen which may include a DHE.[5]

It is not known if starting a migraine preventive [treatment] at the time of the status will shorten the duration. If headaches were frequent prior to the onset of status, preventive medication may be indicated. For those with status with menstrual migraine, a variety of interval treatments may be helpful for prevention including use of frovatriptan, estrogen supplement starting at day –3 for 6 days, and continuous combined oral contraception.

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