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Headache: A Merck Manual of Patient Symptoms podcast

Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical. Acute treatment is with parenteral triptans, dihydroergotamine, or O₂. Prevention is with verapamil, lithium, topiramate, divalproex, or a combination.

Cluster headache affects primarily men, typically beginning at age 20 to 40; prevalence in the US is 0.4%. Usually, cluster headache is episodic; for 1 to 3 mo, patients experience ≥ 1 attack/day, followed by remission for months to years. Some patients have cluster headaches without remission.

Pathophysiology is unknown, but the periodicity suggests hypothalamic dysfunction. Alcohol intake triggers cluster headache during the attack period but not during remission.

Symptoms and Signs

Symptoms are distinctive. Attacks usually occur at the same time each day, often awakening patients from sleep. Pain is always unilateral in an orbitotemporal distribution. It is excruciating, peaking within minutes; it usually subsides spontaneously within 30 min to 1 h. Patients are agitated, restlessly pacing the floor, unlike migraine patients who prefer to lie quietly in a darkened room.

Autonomic features, including nasal congestion, rhinorrhea, lacrimation, facial flushing, and Horner's syndrome, are prominent and usually occur on the same side as the headache.

Diagnosis

• Clinical evaluation

Diagnosis is based on the distinctive symptom pattern and exclusion of intracranial abnormalities.

Other unilateral primary headache syndromes with autonomic symptoms should be excluded:

• Chronic paroxysmal hemicrania: Attacks are more frequent (> 5/day) and much briefer (usually just minutes) than in cluster headache
• Hemicrania continua: Moderately severe continuous unilateral head pain occurs with superimposed brief episodes of more intense pain.

These 2 painful disorders, unlike cluster headache (and migraine), respond dramatically to indomethacin Some Trade Names
INDOCIN
Click for Drug Monograph
, but not to other NSAIDs.

Treatment

• For aborting attacks, parenteral triptans, dihydroergotamine Some Trade Names
  D.H.E. 45
  MIGRANAL
  Click for Drug Monograph
  , or 100% O₂
• For long-term prophylaxis, verapamil Some Trade Names
  CALAN
  ISOPTIN
  Click for Drug Monograph
  , lithium Some Trade Names
  ESKALITH
  LITHOBID
  LITHONATE
  Click for Drug Monograph
  , topiramate Some Trade Names
  TOPAMAX
  Click for Drug Monograph
  , divalproex Some Trade Names
  DEPAKOTE
  Click for Drug Monograph
  , or a combination

Acute attacks of cluster headache can be aborted with either parenteral triptans or dihydroergotamine Some Trade Names
D.H.E. 45
MIGRANAL
Click for Drug Monograph
alone (see Table 4: Headache: Drugs for Migraine and Cluster Headaches*) and/or 100% O₂ inhalation given by nonrebreathing face mask.

All patients require preventive drugs because cluster headache is frequent, severe, and incapacitating. Prednisone Some Trade Names
DELTASONE
Click for Drug Monograph
(eg, 60 mg po once/day) or a greater occipital nerve block (with a local anesthetic and a corticosteroid) can provide prompt temporary prevention while preventive drugs with slower onset of action (eg, verapamil Some Trade Names
CALAN
ISOPTIN
Click for Drug Monograph
, lithium Some Trade Names
ESKALITH
LITHOBID
LITHONATE
Click for Drug Monograph
, topiramate Some Trade Names
TOPAMAX
Click for Drug Monograph
, divalproex Some Trade Names
DEPAKOTE
Click for Drug Monograph
) are initiated.

Table 4
Drugs for Migraine and Cluster Headaches* Drug Dosage Comments Prevention Amitriptyline Some Trade Names ELAVIL ENDEP Click for Drug Monograph 10–100 mg po at bedtime Used only for migraines Has anticholinergic effects; causes weight gain Helpful for patients with insomnia Small doses often effective β-Blockers Atenolol Some Trade Names TENORMIN Click for Drug Monograph 25–100 mg po once/day Metoprolol Some Trade Names LOPRESSOR TOPROL Click for Drug Monograph 50–200 mg po once/day Nadolol Some Trade Names CORGARD Click for Drug Monograph 20–160 mg po once/day Propranolol Some Trade Names INDERAL Click for Drug Monograph 20–160 mg po bid Timolol Some Trade Names BLOCADREN TIMOPTIC Click for Drug Monograph 5–20 mg po once/day Used only for migraines Only β-blockers without intrinsic sympathomimetic activity used Avoided in patients with bradycardia, hypotension, or asthma Divalproex Some Trade Names DEPAKOTE Click for Drug Monograph Regular-release: 250–500 mg po bid Extended-release: 500–1000 mg po once/day Can cause alopecia, GI upset, hepatic dysfunction, thrombocytopenia, tremor, and weight gain Lithium Some Trade Names ESKALITH LITHOBID LITHONATE Click for Drug Monograph 300 mg po bid to qid Used only for cluster headaches May cause weakness, thirst, tremor, and polyuria Periodic checking of drug levels required Topiramate Some Trade Names TOPAMAX Click for Drug Monograph 50–200 mg po usually once/day Can cause weight loss, and CNS adverse effects (eg, confusion, depression) Verapamil Some Trade Names CALAN ISOPTIN Click for Drug Monograph † 240 mg once/day to tid Most useful for patients with cluster headache Can cause hypotension and constipation Treatment Dihydroergotamine Some Trade Names D.H.E. 45 MIGRANAL Click for Drug Monograph 0.5–1 mg sc or IV 4 mg/mL nasal spray Can cause nausea Contraindicated in patients with hypertension or coronary artery disease Cannot be used concurrently with triptans Triptans‡ Almotriptan Some Trade Names AXERT Click for Drug Monograph 12.5 mg po Eletriptan Some Trade Names RELPAX Click for Drug Monograph 20–40 mg po Frovatriptan Some Trade Names FROVA Click for Drug Monograph 2.5 mg po Naratriptan Some Trade Names AMERGE Click for Drug Monograph 2.5 mg po Rizatriptan Some Trade Names MAXALT Click for Drug Monograph 10 mg po Sumatriptan Some Trade Names IMITREX Click for Drug Monograph 50–100 mg po, 5–20 mg nasal spray, or 6 mg sc Zolmitriptan Some Trade Names ZOMIG Click for Drug Monograph 2.5–5 mg po or 5 mg nasal spray Can cause flushing, paresthesias, and sense of pressure in chest or throat Can repeat doses up to 3 times/day if headache recurs Contraindicated in patients with coronary artery disease or uncontrolled hypertension Injections or nasal spray used for cluster headache Valproate Some Trade Names DEPAKENE Click for Drug Monograph 500–1000 mg IV Usually for patients who cannot tolerate triptans or vasoconstrictors Can cause alopecia, GI upset, hepatic dysfunction, thrombocytopenia, tremor, and weight gain *Drugs can be used for either type of headache unless specified otherwise. †The regular-release formulation is usually used. ‡Given once, and then repeated as needed.

Last full review/revision April 2008 by Stephen D. Silberstein, MD

Content last modified April 2008