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Neurology - Headache - Fast Facts | NEJM Resident 360

Headaches are categorized as either primary or secondary, based on etiology, and are considered chronic if they occur for more than 15 days per month, for 3 months or more.

Primary Headache

Primary headache disorders include migraine, tension-type, cluster, and trigeminal neuralgia.

• Migraine headache is characterized by disabling, unilateral, pulsating pain, moderate-to-severe in intensity and lasting 2 to 72 hours. Migraines are often associated with nausea or photophobia and phonophobia and may occur with or without aura (the experience of transient sensory disturbance). The aura may precede or accompany the headache, is often visual, and typically lasts 20−30 minutes. Migraine headache is the second-most common type of headache after tension-type.

• Tension-type headache is the most common type of headache and is characterized by bilateral, steady pain, pressing or tightening in quality with “bandlike” distribution. The intensity is usually mild to moderate, lasting 30 minutes to several days, and is not associated with activity level. Tension-type headaches are not accompanied by nausea, vomiting, photophobia, or phonophobia. Tension headaches are further classified as infrequent (<1 episode per month, with little disability), frequent (at least 10 episodes per month), and chronic (occurring on 15 or more days per month).

• Trigeminal autonomic cephalalgias is a group of headaches characterized by unilateral prominent autonomic features that typically lateralize to the side of the headache.

  • Cluster headache is an often-disabling unilateral headache lasting between 15 and 180 minutes and is associated with agitation and autonomic symptoms (e.g., lacrimation, nasal congestion, and conjunctival injection). Cluster headaches can be chronic or episodic and may occur several times per day.

• Other primary headache disorders include the following:

  • primary exertional or exercise-related headache

  • primary cough headache

  • primary stabbing headache

  • hypnic headache (“alarm-clock” headache wakes the patient from sleep)

  • primary headache associated with sexual activity

  • primary thunderclap headache (striking suddenly, like a clap of thunder)

  • new daily persistent headache

Secondary Headache

Secondary headaches are caused by an underlying condition, including vascular disorders (subarachnoid hemorrhage or giant-cell arteritis), infection (meningitis), trauma, and substance use or withdrawal. Secondary headaches occur in close temporal relation to an event or disorder known to cause headache. Typically, as the underlying disorder either worsens or improves, so does the headache.

• post-traumatic headache

• headache related to infection (e.g., meningitis or encephalitis)

• headache related to cervical or cranial vascular disorder (e.g., subarachnoid hemorrhage or giant-cell arteritis)

• headache related to intracranial neoplasm

• headache related to intracranial disorder (e.g., epileptic headache)

• headache related to substance use or withdrawal (e.g., cocaine-induced or caffeine withdrawal)

• headache related to disorders of hemostasis (e.g., altitude headache, dialysis headache)

• facial pain–related headache (e.g., due to sinusitis)

• headache related to a psychiatric disorder (e.g., somatization disorder)

• medication-overuse headache

• lumbar puncture headache

Presentation

Headaches are one of the most common reasons that patients present for medical attention. Assessment begins with ruling out secondary causes.

History and symptom assessment are paramount and should include the following:

• assessment of frequency, location, duration, and characteristics of headache pain

• assessment of associated symptoms, variations with activity, triggering or relieving factors, and effects on quality of life

• screening for signs and symptoms concerning for secondary causes of headache (the SNNOOP10 list is a useful tool)

The following table provides a list of red-flag features for secondary headache that can be used to discriminate between primary and secondary causes of severe headache:

Features Suggestive of Secondary Headache

• New onset of headache (particularly in persons older than 50 years of age)

• Headache lasting >72 hr

• Vision, sensory, and language symptoms lasting >1 hr

• Very sudden onset of headache or neurologic symptoms

• Abnormal neurologic examination

• Associated fever, systemic illness

Physical examination should include assessment of features such as neck stiffness or fever, papilledema, and motor deficits.

Neurologic examination and further investigation with imaging (CT or MRI) may be warranted in patients with abnormalities or deficits that are detected on examination.

Psychological assessment should be considered to rule out an underlying or concomitant psychiatric disorder. 

Workup

Most of the workup revolves around investigating red-flag symptoms and the identification of secondary causes where suspected.

• complete blood count and erythrocyte sedimentation rate: to investigate possible infection, anemia (may indicate neoplasm or systemic disease), elevated erythrocyte sedimentation rate (ESR; may be associated with occult malignancy, infection, collagen diseases, etc.)

• imaging: CT or MRI

• lumbar puncture: if imaging is negative, to assess for infectious cause or subarachnoid hemorrhage

Management

Primary Headache

Nonpharmacologic treatment:

• Headache diaries: encouraged to assist both patient and physician in characterizing the headache type and developing a treatment approach

• Behavioral approaches: may be useful for headache types associated with triggers (e.g., caffeine) or stress (e.g., tension-type)

Pharmacotherapy:

Pharmacotherapy for primary headaches involves treatment for acute attacks and prophylaxis to reduce their frequency.

• Most acute headache attacks can be treated initially with simple analgesic modalities (e.g., aspirin, acetaminophen, nonsteroidal anti-inflammatory drugs [NSAIDs]).

• Indomethacin therapy has been used with success for some primary headache subtypes (e.g., primary cough, primary stabbing, and exertional headaches)

• Tension-type headache

  • acute therapy: analgesics as above

  • prophylaxis: amitriptyline may be helpful

• Cluster headache

  • acute therapy: high-flow oxygen (6−12 liters per minute for 15−20 minutes) or triptans (e.g., sumatriptan)

  • prophylaxis: suboccipital steroid injections have been shown to be effective for short-term prevention; monoclonal antibodies (e.g., galcanezumab)

    • off-label treatment: verapamil and lithium

• Migraine headache

  • acute therapy: acetaminophen; NSAIDs (e.g., naproxen, ibuprofen); triptans (e.g., sumatriptan); ergots and antiemetics (e.g., metoclopramide), either alone or in combination (see table below)

    • Triptans have been shown to be the most effective treatment for migraine.

    • Calcitonin gene–related peptide (CGRP) receptor antagonists (e.g., rimegepant and ubrogepant) have shown promise in clinical trials for treatment of migraine.

  • prophylaxis: tricyclic antidepressants (e.g., amitriptyline), beta-blockers (e.g., propranolol), anticonvulsants, and monoclonal antibodies targeting CGRP or its receptor

The following tables provide an overview of therapies for treatment and prophylaxis of migraine headache.

Treatments for Acute Migraine

(Source: Migraine. N Engl J Med 2017.)

Prophylaxis for Migraine

(Source: Migraine. N Engl J Med 2017.)

Secondary Headaches

Secondary headaches may improve with analgesia and treatment of the underlying cause.

Headache due to lumbar puncture is a procedure-related secondary headache. In many cases it is self-limiting or may improve with changes in posture (e.g., lying flat following the procedure). Hydration or consumption of caffeine can also provide relief. An epidural blood patch may be needed for refractory cases.

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