Resident Editor: Amanda Wright, MD, Janet Chu, MD

Faculty Editor: Morris Levin, MD

Evaluation

• History:
  • Determine if new or old headache
  • Determine if primary (e.g., tension, migraine, cluster) or secondary (e.g., those caused by infection or vascular disease)
  • Focus on onset, duration, quality, frequency, associated symptoms (n/v, neurological symptoms, aura, photo/phonophobia, weight loss, fevers), alleviating and aggravating factors (activity, food, alcohol), treatments tried, medications (NSAID, opioids, OCP) illicit drug use, family history of headaches and migraines
  • Chronic headaches tend to be primary with low risk for emergency
• Physical Exam
  • Vital signs: fever, hypertension
  • HEENT: temporal pain to palpation, papilledema, pupillary constriction, visual fields, signs of meningismus, sinus tenderness, dental assessment
  • Neuro: evaluate for any focal neurological deficit, mental status changes
  • Skin: Rashes to suggest meningococcal meningitis
• Red Flags (use the mnemonic SNOOP)
  • Systemic symptoms: fevers, chills, weight loss, cancer, immunocompromised state (including HIV)
  • Neurologic signs/symptoms: papilledema, confusion, change in mental status, focal neuro exam findings, meningismus, or seizures)
  • Onset: New (especially if age >50), acute, sudden, split second ‘thunderclap’ (reaching max intensity in < 1 min)
  • Other associated conditions or features (e.g., head trauma, illicit drug use, or toxic exposure; headache awakens from sleep, worse with Valsalva maneuvers, or precipitated by cough, exertion, or sexual activity)
  • Previous headache history: first HA or different (change in frequency, severity or features) or progression
• Labs/Tests: often not indicated; consider ESR/CRP if temporal arteritis considered, LP as needed based on history
• Radiologic Studies: often not indicated. If red flags, consider CT vs MRI. MRI will have incidental findings in 1 of 10 patients.

Differential Diagnosis: 2 categories: Primary and Secondary

• Primary headaches
  • Tension: considered most common (though many will be migraine without the usual accompaniments). At least 2 of the following:  Bilateral location, pressing or tightening (non-pulsating) quality, mild to moderate intensity, lack of aggravation by routine physical activity.  Should have normal neuro exam.  Should have no N/V or photophobia&phonophobia.
  • Migraine: At least 2 of the following: unilateral, pulsating, moderate or severe pain intensity, aggravated by routine physical activity; +N/V or photophobia/phonophobia; +/- aura (5-60min, usually visual or sensory sx’s) familial pattern often exists. For diagnosis, consider POUND mnemonic (Pulsatile, One-day (or up to 72h), Unilateral, Nausea or vomiting), Disabling intensity) – 4/5 sx’s = 92% probability, 3/5 = 64% probability.
  • Cluster and other Trigeminal Autonomic Cephalgias (TACs) –
    • Cluster HA: episodic (6-12wks/yr), short-lasting (usually 30-60 min for cluster), excruciating unilateral/orbital/supraorbital pain, usually accompanied by at least one of the following ipsilateral autonomic symptoms:  lacrimation, nasal congestion or rhinorrhea, eyelid edema, forehead/facial sweating, miosis or ptosis, restlessness or agitation, conjunctival injection. Occurs in men more often than women.  In women, cluster headache features can be less prominent.
    • Paroxysmal hemicrania: 3 main features – many attacks daily (>5/day), and very short-lasting (2-30min), severe and strictly unilateral orbital, supraorbital or temporal pain; symptoms of parasympathetic activation on same side of pain. Responds to indomethacin.
• Secondary Headaches
• Associated with Infection
  • Dental caries/abscess – dental, jaw or maxillary region pain
  • Sinusitis – frontal or retro-orbital pain with symptoms of sinusitis, worse with bending forward
  • Meningitis – fever and stiff neck unless chronic from TB, fungus, cancer
  • Encephalitis – fever, mental status changes, seizures
  • Intracranial abscess – fever, focal neurological signs
• Associated with intracranial mass lesion/bleed:
  • Tumors – subacute, progressive, unilateral pain, may be worse in early morning and or supine; occasionally associated with nausea, vomiting, and focal neurological exam
  • Epidural hematoma – usually secondary to severe head trauma; lucid period followed by loss of consciousness progressing to coma
  • Subdural hematoma – may result from minor head trauma, most common in elderly and patients with substance abuse histories; patients may present with fluctuating level of consciousness.
  • Subarachnoid hemorrhage – classically reported as sudden onset of severe HA, associated with stiff neck, nausea, vomiting, syncope, sometimes focal neurological exam
  • Cerebral venous thrombosis – sometimes related to estrogen (OCP, pregnant), HA can range from acute to chronic. Diagnose with MRI with venography
• Associated with other diseases:
  • TMJ disease – pain evoked by talking, chewing, or lateral jaw movement; exam reveals tenderness of the joint
  • Temporal arteritis – unilateral, with visual abnormalities, temporal artery tenderness, malaise, fever, jaw claudication, high ESR (though any/all of these may be absent). Consider in all patients with new headache at age >50.  15% of cases associated with PMR.
  • Acute angle closure glaucoma –painful red eye, pupil mid-dilated and fixed, associated with periorbital headache, visual disturbance such as halos, nausea and vomiting.
  • Cerebrovascular ischemia – occasionally prior to or during stroke or TIA, more likely with posterior circulation involvement.
  • Carotid (or vertebral artery) dissection – severe HA, possibly without neck pain, with Horner’s (carotid) and associated TIA/stroke symptoms.
  • Idiopathic intracranial hypertension (pseudotumor cerebri): almost always with papilledema; occurs in young obese women.
  • Hydrocephalus – papilledema, ataxia
  • Intracranial hypotension – HA much worse with sitting or standing, relived in supine, MRI findings of cerebellar tonsillar descent and pachymeningeal enhancement may help to dx.
  • Pheochromocytoma – headache with generalized sweating, tachycardia, and/or sustained or paroxysmal hypertension
  • Malignant hypertension
  • Cervicogenic headache – generally in elderly with structural cervical spine disease, worse with provocation (ROM maneuvers)
• Associated with drugs:
  • Medication-overuse headache: associated with frequent use of  analgesics, opioids, ergotamine (>2x/wk); Clues are lack of response to acute and proph meds. Risk from highest to lowest: opioids, butalbital-containing combination analgesics, ASA/acetaminophen/caffeine combinations, triptans, NSAIDs
  • Drug-induced headache – nitrates, hormonal supplements, digoxin, caffeine, ethanol, illicit drugs (cocaine, methamphetamines)

Treatment of Primary Headaches

• Tension: 
  • Mild analgesics (acetaminophen, NSAIDs, ASA), avoidance of precipitating factors, relaxation techniques. Consider combination of caffeine with acetaminophen, NSAIDs
  • For chronic tension headaches (often actually migraine), consider prophylactic agents – amitriptyline 10mg-75mg at bedtime; SSRIs generally minimally helpful.
• Cluster/TAC
  • Abortive therapy: 100% oxygen, sumatriptan via injection, intranasal lidocaine
  • Prophylactic therapy:  verapamil, lithium, valproate; and prednisone (which can be used at the start of a cluster episode to shorten the headache cycle).
• Migraine
• Abortive therapy:
  • Mild migraine: acetaminophen, ASA, NSAIDs, combination analgesics (combination of any of the following: ASA, acetaminophen, caffeine).
  • Moderate to severe migraine: use migraine-specific drugs.  Triptans easier to use than ergotamines, with fewer side effects.
    • Triptans: Sumatriptan, rizatriptan, eletriptan, zolmitriptan all effective. Naratriptan mildest; Frovatriptan has longest ½ life; Contraindications: CAD, stroke, uncontrolled HTN, migraine with brainstem or hemiplegic aura, MAOIs, SSRIs
    • Ergotamine: less effective orally; C/I in CAD, PVD, pregnancy, triptans.
    • Isometheptene compound: i.e. Midrin – C/I in CAD, PVD – may not be available.
    • If severe nausea/vomiting: Nasal (sumatriptan or zolmitraptan), Subcutaneous (sumatriptan).  Also consider adding antiemetic (metoclopramide, prochlorperazine, or promethazine)
    • If poor response to initial triptan, consider 1) ↑ triptan dose, 2) trial of different triptan, 3) combination triptan/naproxen 4) DHE nasal spray (migranal)
    • Prophylactic therapy: recommend if 1) contraindications/intolerance to abortive tx, 2) HA > 2x/wk, 3) HA that severely limit quality of life despite abortive tx, 4) presence of uncommon migraine conditions. Start low, go slow: increase dose every 2-4 weeks. Maximal benefit may not be seen until 2-3 months after medication at target dose. Can discontinue after 6-12 months of control.
      • Beta-blockers – propranolol, atenolol, nadolol >> other BBs; consistent efficacy; s/e – fatigue, avoid in asthma
      • TCAs – amitriptyline 10 mg nightly initial dose; s/e – drowsiness, weight gain, anticholinergic effects, max dose 150 mg hs; nortriptyline 10-75 mg hs is a good alternative.
      • Anticonvulsants – divalproex500-1000 mg daily in 3 div doses but freq AEs, topiramate (start 25 mg/day, increase by 25 per week to 100 mg per day); AEs -  weight loss, fatigue, decreased appetite, nausea, diarrhea, cognitive dysfunction, paresthesias, kidney stones
      • Calcium-channel blockers (verapamil, amlodipine) – lower efficacy

When to Refer:

When diagnosis is unclear, patient has persistent symptoms despite first line prophylactic medications, or high disability.

References

Hainer BL, Matheson MM. Approach to Acute Headache in Adults. American Family Physician. 2013;87(10):682-687.

Simon, RP, Aminoff MJ, Greenberg DA (2017). Headache and Facial Pain. In Clinical Neurology, 10e. New york, NY: McGraw Hill.