Table of Contents
- Introduction
- Epidemiology
- Defining Status Migrainosus
- Pathophysiology and Genetics
- Etiology and Common Precipitating Factors
- Diagnostic Criteria
- Differential Diagnosis
- Evaluation
- Treatment and Management
- Acute/Emergency Management
- Emergency Treatment
- Choice of Treatment
- Non-pharmacological
- Pharmacological Treatment
- First Line
- Second Line
- Figure 1A: No contraindication to DHE and Triptans
- Figure 1B: Contraindication to DHE and Triptans
- Complications and Prognosis
- Further Reading
- Bibliography
Primary Category
Headache & Pain
P-Category
Secondary Category
S-Category
Introduction
- Debilitating and unremitting migraine headache which lasts at least 72 hours and has been refractory to typical abortive therapy.
- Status migrainosus is classified as a subclass of migraine-related complications
- Headache-free intervals of less than 4 hours may occur.
- Also known as an Intractable Migraine with or without aura.
- Should be addressed in an urgent care or emergency department.
Epidemiology
- Migraine is a common disorder that affects up to 12 percent of the general population.
- Prevalence of migraine is inversely correlated with the level of education and household income in the United States.
- It is more frequent in women than in men, with attacks occurring in up to 17 percent of women and 6 percent of men each year.
- Ranked among the ten most disabling conditions.
Defining Status Migrainosus
International Classification of Headache Disorders (ICHD), 3rd Ed
Migraine Headache:
- Unremitting for > 72 hours
- Pain and/or associated symptoms are debilitating
Pathophysiology and Genetics
- Exact mechanism is unknown.
- Possible altered circadian secretion of melatonin, suggesting a role of the pineal gland.
- Angiographic examination revealed presence of multiple segmental stenoses and dilatations of the cerebral arteries along with evidence of inflammation.
- Another hypothesis of it’s pathogenesis emphasizes the activation and sensitization of the trigeminovascular system via the involvement of different neuropeptides.
- A genetic component to migraine is indicated by the fact that approximately 70% of patients have a first-degree relative with a history of migraine.
Etiology and Common Precipitating Factors
- Emotional stress
- Depression
- Medication overuse
- Anxiety
- Diet
- Hormonal factors
- Multiple non-specific factors
- Organic sleep disorders
- Generalized anxiety disorder
- Post traumatic stress disorder
Diagnostic Criteria
International Classification of Headache Disorders (ICHD-3)
Diagnostic Criteria for Status Migrainosus
Criteria A
- A headache attack fulfilling criteria B and C.
Criteria B
- Occurring in a patient with:
- Migraine without aura, i.e. headache that has at least two of the following four characteristics; unilateral location, pulsating quality, moderate or severe pain intensity and aggravation by or causing avoidance of routine physical activity, AND/OR
- Migraine with aura; aura with both of the following: fully reversible visual, sensory and/or speech/language symptoms and no motor, brainstem or retinal symptoms,
- and typical of previous attacks except for its duration and severity.
Criteria C
- Both of the following characteristics:
- Unremitting for > 72 hours
- Pain and/or associated symptoms are debilitating
Criteria D
- Not better accounted by another ICHD-3 diagnosis.
Note
- Remissions of up to 12 hours due to medication or sleep are accepted.
- Milder cases, not meeting criterion C2, are Probable migraine without aura.
Source: 1.4.1 status migrainosus. (n.d.). ICHD-3. https://ichd-3.org/1-migraine/1-4-complications-of-migraine/1-4-1-status-migrainosus/
Differential Diagnosis
- Tension headache
- Cervical spine disease (greater occipital neuralgia)
- Acute cervical strain (whiplash)
- Intracranial mass (subacute headache and worsening)
- Meningitis
- Subarachnoid hemorrhage (worst headache ever)
- Optic neuritis
- Acute glaucoma
- Pseudotumor cerebri
- Carotid artery dissection
- Temporomandibular joint (TMJ) syndrome
- Sinusitis
- Rhinogenic headache ("sinus headache")
Evaluation
- American Academy of Neurology Practice Parameter
- Neuroimaging (CT or MRI) recommended in patients with:
- unexplained abnormal neurologic signs (AAN Grade B),
- atypical headache features or headaches that do not fill specific diagnostic criteria (AAN Grade C).
- Neuroimaging is not usually recommended for patients with migraine and normal neurologic exam (AAN Grade B).
- No recommendations made for presence or absence of neurologic symptoms.
- Insufficient evidence to support guidelines for EEG.
Treatment and Management
Acute/Emergency Management
- Emergency treatment for patients with status migrainosus should be aggressive.
- It includes rest; rehydration and electrolyte replacement; detoxification; round-the-clock parenteral analgesic therapy; symptomatic treatment of nausea, anxiety, insomnia, and withdrawal symptoms; concurrent initiation of prophylactic therapy; and behavioral treatment.
Emergency Treatment
Initial
- IV fluids, normal saline 2-3 L bolus or 80-100 cc/h for as long as patient is in emergency department
- IV diphenhydramine 12.5-25 mg
- IV dopamine receptor antagonist medication (typically use metoclopramide 10 mg or prochlorperazine 10 mg)
- IV magnesium sulfate 500 mg / 1 g
- IV ketorolac 30 mg
Advanced
Medications are given in succession separated by 15 to 20 minutes
- If patient does not improve, other options include IV sodium valproate (500 mg), IV levetiracetam (500 mg), or IV methylprednisolone (200 mg)
- IV dihydroergotamine 0.5-1.0 mg may be used if patient has not used triptan within 24 hours and no contraindications exist
Choice of Treatment
Non-pharmacological
- Behavioral methods
- Biofeedback
- Monitor body temperature on electromyography.
- Relaxation Techniques
- Progressive muscle relaxation
- Imaginary-based relaxation
- Reinforcement of maintaining a regular schedule, sleep, diet and exercise.
- Resting or sleeping in a dark, quiet room should be encouraged.
- Triggers (skipping meals, dehydration, stress, change in sleep patterns, infections, etc.) should be identified and corrected.
Pharmacological Treatment
First Line
- Triptans
- Sumatriptan 6 mg
- Administered subcutaneously
- May be repeated if ineffective after 1 hour
- Ergot alkaloids
- Dihydroergotamine
- Administered intramuscularly
- Requires premedication with Prochlorperazine or Metoclopramide 10 mg intramuscularly
- May be repeated after 1 to 2 hours if ineffective
Second Line
- Intravenous Hydration
- Aspirin and NSAIDs
- Intravenous Ketorolac
- Prochlorperazine
- 25 mg rectal suppository, OR
- 10 mg intramuscularly, OR
- 10 mg intravenously
- Opioids
- Hydromorphone (2 mg orally or 3 mg per-rectal)
- Morphine (15-30 mg orally or 30 mg per-rectal)
- Steroids
- Prednisone 80 mg, tapered over 4 days (20 mg each day)
- Dexamethasone 8 mg, tapered over 4 days (2 mg each day)
- Nerve blocks
- Occipital and trigeminal nerve blocks (safe in pregnancy)
Figure 1A: No contraindication to DHE and Triptans
Figure 1B: Contraindication to DHE and Triptans
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Derived From: Dawn A Marcus (2001) Treatment of status migrainosus, Expert Opinion on
Pharmacotherapy, 2:4, 549-555, DOI: 10.1517/14656566.2.4.549
Complications and Prognosis
- Being headache free upon discharge and having continued pain freedom for at least 48 hours post-discharge is a suggested goal for ED treatment of patients with episodic migraine (known as sustained headache freedom)
- Most ED trials suggest this only occurs in about 20% to 25% of patients.
- 30% of patients with migraine report a moderate to severe headache within 1 day after ED discharge, and 50% are functionally disabled by that headache.
- Predictors of poor post-ED outcomes include severe pain at baseline, presence of nausea, and longer duration of headache.
Further Reading
- Treating status migrainosus in the emergency setting: what is the best strategy?, Expert Opinion on Pharmacotherapy, DOI: 10.1080/14656566.2018.1516205
Bibliography
- Unrecognised challenges of treating status migrainosus: An observational study. (n.d.). PubMed Central (PMC). https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7367756/
- Treating status migrainosus in the emergency setting: what is the best strategy?, Expert Opinion on Pharmacotherapy, DOI: 10.1080/14656566.2018.1516205
- Migraine prevalence. A review of population-based studies. Neurology, 44(6 Suppl 4), S17–S23
- Prevalence and burden of migraine in the United States: data from the American Migraine Study II. Headache, 41(7), 646–657. https://doi.org/10.1046/j.1526-4610.2001.041007646.x
- Treatment of status migrainosus, Expert Opinion on Pharmacotherapy, 2:4, 549-555, DOI: 10.1517/14656566.2.4.549
- Evaluation of Triggers of Status Migrainosus and its impact on morbidity (5479) Neurology Apr 2020, 94 (15 Supplement) 5479
- Status Migrainosus: Causative and Therapeutic Aspects and disability.
- Reversible MRI abnormalities in a patient with recurrent status migrainosus. doi:10.1111/j.1468-2982.2008.01803.x
- A REVIEW ON CLASSIFICATION, PATHOPHYSIOLOGY, DIAGNOSIS, AND PHARMACOTHERAPY OF HEADACHE. Innovare Journal of Medical Sciences. 8. 1-12. 10.22159/ijms.2020.v8i6.37667.
- 1.4.1 status migrainosus. (n.d.). ICHD-3. https://ichd-3.org/1-migraine/1-4-complications-of-migraine/1-4-1-status-migrainosus/
- Birth and Growth Medical Journal 2019;28(3): 132-140. doi:10.25753/BirthGrowthMJ.v28.i3.15431
- Migraine ☆. Reference Module in Neuroscience and Biobehavioral Psychology. doi:10.1016/b978-0-12-809324-5.03097-2
- www.neurology.org. Neurology, 55(6), 768-768. https://doi.org/10.1212/wnl.55.6.768
- Parenteral dexamethasone for acute severe migraine headache: meta-analysis of randomised controlled trials for preventing recurrence. BMJ 2008;336(7657):1359Y1361.doi:10.1136/bmj.39566.806725.BE.
- Subcutaneous sumatriptan for treatment of acute migraine in patients admitted to the emergency department: a multicenter study. Ann Emerg Med 1995;25(4):464Y469. doi:10.1016/ S0196-0644(95)70259-8.
- Randomized trial of IV dexamethasone for acute migraine in the emergency department. Neurology 2007;69(22)
- Recurrence of primary headache disorders after emergency department discharge: frequency and predictors of poor pain and functional outcomes. Ann Emerg Med 2008;52(6):696Y704. doi:10.1016/ j.annemergmed.2008.01.334.
- Treatment of status migrainosus, Expert Opinion on Pharmacotherapy, 2:4, 549-555, DOI: 10.1517/14656566.2.4.549
- CONTINUUM (MINNEAP MINN) 2018;24(4, HEADACHE):1092–1107
- Emergency department and inpatient management of status migrainosus and intractable headache. Continuum: Lifelong Learning in Neurology, 21(4), 1004-1017
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