Headaches, Cluster

SOURCE

Source: Minor DS, Harrell T. Headache disorders. In: DiPiro JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey L, eds. Pharmacotherapy: A Pathophysiologic Approach. 10th ed. New York, NY: McGraw-Hill; 2017. https://accesspharmacy.mhmedical.com/content.aspx?bookid=1861&sectionid=146063736. Accessed September 11, 2018.

DEFINITION

• Most severe of the primary headache disorders, known as trigeminal autonomic cephalalgia

• Classified by attacks of excruciating, unilateral head pain that occurs in series lasting for weeks or months (ie, cluster periods) separated by remission periods usually lasting months or years

EPIDEMIOLOGY

• Exact prevalence is uncertain.

• Estimated lifetime prevalence of 124 per 100,000 people or 0.12%

• Male to female ratio is approximately 4:1 with the age onset typically in the third to fifth decade.

PATHOPHYSIOLOGY

• Pathophysiologic mechanisms are not completely understood.

• Neuroimaging studies performed during acute attacks have shown activation of the ipsilateral hypothalamic gray area, implicating the hypothalamus as a modulator of cluster headaches.

  • Activation of the trigeminal-autonomic reflexes leads to ipsilateral pain and cranial autonomic features.

CLINICAL PRESENTATION

• One hallmark of cluster headaches is the circadian rhythm of painful attacks.

• Episodic cluster headaches are the most common cluster headache subtype.

  • Attacks occur daily for a week to several months, followed by pain-free intervals.

  • Average remission periods are 2 years in length.

• Attacks occur commonly at night and more commonly in the spring and fall.

• Attacks occur suddenly, pain peaks quickly after onset and lasts 15–180 min.

• Pain presentation

  • Excruciating

  • Penetrating

  • Boring intensity in the orbital, supraorbital, and temporal unilateral locations

  • Auras are not present with cluster headaches.

• Cranial autonomic symptoms

  • Conjunctival injection

  • Lacrimation

  • Nasal stuffiness

  • Rhinorrhea

  • Eyelid edema

  • Facial sweating

  • Miosis/ptosis

  • Typically resolve with resolution of the headache

DIAGNOSIS

• Diagnostic criteria per International Headache Society (IHS) classification system:

  1. At least five attacks fulfilling criteria B–D

  2. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15–180 min (when untreated)

  3. Either or both of the following:

     • At least one of the following symptoms or signs, ipsilateral to the headache:

       1. Conjunctival injection and/or lacrimation

       2. Nasal congestion and/or rhinorrhea

       3. Eyelid edema

       4. forehead and facial sweating

       5. miosis and/or ptosis

     • Sense of restlessness or agitation

  4. Occurring with a frequency between one every other day and eight per day

  5. Not better accounted for by another ICHD-3 diagnosis

TREATMENT: ABORTIVE THERAPY

• Directed at managing the acute attack

• Oxygen

  • Standard acute treatment is 100% oxygen at a rate of at least 12 L/min for 15 min

• Triptans

  • Safe and effective due to quick onset of subcutaneous and intranasal routes

    • Subcutaneous sumatriptan (6 mg) is the most effective agent.

    • Nasal sprays are less effective but may be better tolerated in some patients.

    • Orally administered triptans have limited use in cluster attacks because of their relatively slow onset of action.

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