Cluster Headache: John Bibs
Headache for Educators > Cluster Headache Case

John Bibs

Presentation

John Bibs is a 42 y/o male who presents with unilateral periorbital headaches.
What are causes of unilateral periorbital pain?

 

HPI

Causes of unilateral periorbital pain to consider include:
  • Headache syndromes - migraine, cluster, chronic paroxysmal hemicrania (rare)
  • Temporal arteritis
  • Trigeminal neuralgia
  • Herpes zoster; postherpetic neuralgia
  • Sinusitis
  • Intracranial mass

View the patient interview: Windows Media or Quicktime

What is your differential diagnosis?
What characteristics distinguish cluster from migraine headaches?

 

PMHx

The periodic nature of these headaches make the most likely diagnosis either cluster headache or paroxysmal hemicrania. The latter headache syndrome produces more frequent episodes of pain (more than 5 a day) of shorter duration (2 to 30 minutes). Trigeminal neuralgia produces episodic, brief severe pain that does not occur with any periodicity. Sinusits, herpes zoster and postherpetic neuralgia would not produce episodic pain. Temporal arteritis is unlikely in a patient less than 50, and tends to produce constant pain. The length of Mr. Bibs' pain makes a brain tumor very unlikely.

Characteristics that distinguish cluster from migraine include the lancinating, boring, or burning nature of the pain (in contrast to the migraneur's complaints of throbbing pain), the restlessness experienced by the cluster sufferer, and associated autonomic symptoms suggesting trigeminal nerve activation (unilateral lacrimation, rhinorrhea or nasal congestion, and a partial Horner's syndrome).
The most important distinguishing characteristic of cluster headaches is the periodicity that leads to their name. Patient with cluster headaches typically will awaken from pain several hours after sleep with a headache that will reach maximal intensity within 15 minutes, last about 2 hours, and then subside. They will frequently have second, third or rarely more headaches occurring at regular intervals in a 24 hour cycle. Patients will have these headaches in clusters of 6 to 8 weeks duration, followed by remission, with most sufferers having 1 or 2 periods of headaches a year. Headache patterns are usually consistent for a patient.
As noted in the interview, Mr. Bib has been relatively healthy.
What triggers cluster headaches?

 

Social Hx

Mr. Bib does drink beer. Unlike migraines, though, cluster headaches are not usually triggered by ingested substances. Due to the periodicity of the headaches, and their predilection for causing night-time headaches, researchers have suspected that hypothalamic dysfunction plays an important role in cluster headache pathogenesis, a hypothesis recently strengthened by PET study findings. Epidemiologic studies have demonstrated that most attacks occur within several weeks of the winter and summer solstice.
Do cluster headaches run in families?

 

Family Hx

Mr. Bibs' father had a history of headaches.

Even though cluster headaches are relatively uncommon the disorder occurs in 7% of first degree relatives of cluster headache patients, and several studies have identified large families with many generations affected. There is an important genetic component to cluster headaches, but the exact nature of inheritance patterns remains undetermined.
Can patients with cluster headaches have an aura?

 

ROS

Mr. Bibs reports that his headaches are associated with mild photophobia, nausea, lacrimation, rhinorrhea and bloodshot eyes.

In one study, 12% of patients reported migraine like auras. Nausea, photophobia and phonophobia occurred in about 50% of patients.
What physical exam findings would you expect to see in a cluster headache patient with an acute attack?

 

Physical

During acute attacks, most patients will demonstrate unilateral lacrimation, rhinorrhea or nasal congestion. Many will have eyelid swelling, as well as a partial Horner's syndrome (eyelid ptosis and/or pupillary miosis). This link provides a good illustration: Pain of Cluster Headache

 

Head Normal
Eyes Normal
Ears Normal
Oropharynx Normal
Neck Normal
Chest Normal
Heart Normal
Abdomen Normal
Genitalia Deferred
Anus Deferred
Hips Deferred
Extremities Normal
Neurological Normal
Skin Normal
Are any further tests required to make the diagnosis of cluster headache?

 

 

Radiology

No further testing is required to make the diagnosis in this patient. Like migraine patients, the diagnosis of cluster headaches is based on criteria developed by the International Headache Society:

A. At least five attacks fulfilling B-D

B. Severe unilateral orbital, supraorbital, or temporal pain (or both) lasting 15 to 180 minutes if left untreated

C.Headache associated with at least one of the following signs, which have to be present on the same side as the pain:

  1. Conjunctival injection
  2. Lacrimation
  3. Nasal congestion
  4. Rhinorrhoea
  5. Forehead and facial sweating
  6. Miosis
  7. Ptosis
  8. Eyelid oedema

D. Frequency of attacks: from one every other day to 8/day

E. At least one of the following:

  1. History, physical and neurological examinations do not suggest headaches secondary to organic or systemic metabolic disease
  2. History, physical or neurological examinations do suggest such a disorder, but it is ruled out by appropriate investigations
  3. Such a disorder is present, but cluster attacks do not occur for the first time in close temporal relation to the disorder

 

1. During your examination of Mr. Bibs, he develops a severe headache consistent with his cluster headaches. Which of the following is the best therapy to offer him for acute treatment of his current headache?

A. Morphine Sulfate, 10 mg, mg, IM, along with promethazine 50 mg, IM. (Incorrect)
B. IV methylprednisolone, 120 mg. (Incorrect)
C. Oxygen 7L/minute (Correct)
D. Sumatriptan, 50 mg PO (Incorrect)

 

Management

There are three steps for managing patients with cluster headaches:

  1. Abortive therapy for the acute headahce
  2. Transition to maintenance therapy; and
  3. Maintenance therapy.

For acute attacks, the preferred therapy is oxygen at 7L/minute for 15 minutes. This has been shown in a small randomized controlled trial(RCT) to be effective. For therapy of acute attacks at home, patients can use subcutaneous sumatriptan 6mg, which has been shown to be effective in several (RCT's). 10 mg of oral zolmatriptan has also been found to be effective in one RCT.

Maintenance preventive therapies take several weeks to take effect. For this reason, transition therapy is needed to suppress cluster headaches until maintenance therapy takes effect. Dihydroergotamine has traditionally been used, but this prevents the use of triptans as abortive agents. One case series showed that prednisone, 60 mg a day and tapered over 18 days, quickly suppresses cluster attacks. It can be used with triptans and other agents, and is now the preferred agent.

The goal of maintenance therapy is to suppress cluster headaches. One RCT showed that verapamil, 120 mg three times a day, lessened headache frequency and need for rescue medications. Case series suggest that gabapentin, lithium, methysergide topiramax and valproic acid may be effective in preventing attacks. For patients who prefer complementary or alternative therapies, two small RCT's suggest that oral melatonin 10 mg a day and intranasal capsaicin, administered on the same side of the headache, also reduce attack frequency.
Refractory cases may require two medications, and rarely may require surgery.

What would you prescribe for Mr. Bibs at his visit today?

 

 

Follow-up

Mr. Bibs should receive a prescription for an abortive agent, such as subcutaneous sumatriptan, a transitional agent, prednisone 60 mg a day, and a maintenance medication, verapamil, at 120 mg a day. He should begin both the prednisone and the verapamil at the same time. The prednisone will be tapered over 18 days, and the verapamil continued for the duration of his typical cluster episode. For a patient with cluster headaches for the first time, this would be about 9 weeks, based on the average length of cluster episodes. Prednisone and verapamil should be re-started at the first occurrence of his next episode of cluster headaches. For patients with chronic cluster headaches, verapamil could be continued indefinetely.

 

Teaching Points

The diagnosis of cluster headache is made on clinical grounds. Key features include boring, lancinating or burning peri-orbital pain associated with restlessness, lacrimation, rhinorrhea and a partial Horner's syndrome. The headaches occur in a typical daily pattern, usually awakening a patient from sleep. Headaches tend to occur in episodes at the same time each year.

Treatment for cluster headache includes oxygen or sumatriptan for acute attacks, prednisone as transitional therapy, and verapamil as a maintenance medication. Maintenance therapy is continued for the duration of a patient's cluster episode.