Status Migrainosus

Authors:

Muhammad Umair MD,

Muhammad Roshan Asghar MD,

Junaid Kalia MD

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

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

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

• 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

• 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

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

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