Woman With Frequent Severe Headaches

THE CASE:
The patient is a 47-year-old white woman with frequent, intensely painful unilateral or bilateral headaches that occur behind her left eye or temporal area. These headaches cause throbbing pain that lasts about 40 to 50 minutes: the patient rates the pain severity as a 7 or 8 on a 10-point visual analog scale. She also experiences concomitant tearing and nasal congestion on the involved side only. She denies nausea, vomiting, and sensitivity to light and noise during these attacks. There is no clear frequency pattern to these headaches, which for the past 5 years have occurred 3 or 4 times a month.

The patient also has headaches that involve the entire left or right hemisphere—or even the entire head. During such attacks, the pain is more severe: the patient rates it as an 8 or 9 on a 10-point scale. Throbbing pain lasts up to 72 hours (average duration, 24 hours). These headaches occur once or twice a week and are usually accompanied by nausea and increased sensitivity to bright light and loud noises. They began when the patient was in her early 20s.

The patient has a regular menstrual cycle and denies any association between her headaches and menses. Her maternal grandmother had migraines, and her mother's nephew has newly diagnosed chronic daily headache.

Results of a neurologic and physical examination were within normal limits. MRI and magnetic resonance angiographic images of the brain taken 2 months earlier were also normal.

Currently the patient is taking sumatriptan or zolmitriptan—8 or 9 pills per month on average. Both medications eliminate or significantly decrease the intensity of the headaches (those behind her eye and those that involve severe pain) about 80% of the time.

• Is this patient experiencing a single type of headache?
• What clues point to the cause(s)?

THE DIALOGUE:
Primary care doctor: Is this patient experiencing a single type of headache?

Headache specialist: This patient presented with “frequent severe headaches” and did not distinguish between those that manifest behind her eye and those that involve half or all of her head. The clinical features of her headaches vary with the site of pain; this suggests the presence of separate headache entities.

Most patients with frequent or chronic headaches have difficulty in differentiating one type of headache from another. Most simply report frequent or daily headaches.

Consider the clues this patient has offered:

• The duration of her headaches varies. Some attacks last up to an hour; others may last up to 3 days.
• The short-lasting headaches occur behind her left eye.
• Symptoms vary, depending on the site of pain. The short-lasting headaches are usually associated with lacrimation and nasal congestion. The prolonged headaches are accompanied by nausea, photophobia, and phonophobia.

Primary care doctor: What diagnoses would you consider?

Headache specialist: There is no question that the longlasting headache that is associated with nausea, photophobia, and phonophobia is migraine without aura.1 Migraines can manifest on different sides of the head. This diagnosis is supported by the patient’s family history of migraines and by the normal results of the physical examination and diagnostic imaging.

Diagnosis of the second type of headache is more problematic. Short-lasting, strictly unilateral headaches associated with unilateral autonomic symptoms (eg, lacrimation, nasal congestion, conjunctival injection, or ptosis) are typically manifestations of a group that includes cluster headaches, episodic and chronic paroxysmal hemicrania, and the SUNCT (Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing) syndrome.

It is crucial to distinguish among these types of headaches because treatments can vary.

Primary care doctor: This patient’s symptoms could be manifestations of any of these short-lasting headache entities. What specific factors should be weighed in the differential diagnosis?

Headache specialist: It’s true, the majority of this patient’s symptoms are somewhat nonspecific, and they can occur in many different headache disorders. The only unique symptom is the duration of the short-lasting headache.^2,3

• Headaches associated with the SUNCT syndrome last between 5 seconds and 4 or 5 minutes.³
• Attacks of chronic or episodic paroxysmal hemicrania usually last between 2 and 30 minutes.
• Cluster headaches may last up to 3 hours.

Given this patient's symptoms, it would be reasonable to consider a diagnosis of cluster headache. However, there are some atypical (and thus potentially confusing) features.

First, cluster headaches almost always affect men. The male-to-female ratio is 6:1. Paroxysmal hemicrania, especially the chronic form, is more common in females. There is a dearth of information regarding the distribution of the SUNCT syndrome.

A second atypical feature of this patient's short-lasting headaches is the severity of the pain. Cluster headaches are more painful than any other type of headache. A typical cluster attack manifests with sharp, stabbing, extremely severe pain so intense that the patient cannot remain still. (Most prefer to pace or rock back and forth.) Pain associated with paroxysmal hemicrania and the SUNCT syndrome varies from moderate to severe.

Considering all the information that has been gathered, I suspect that the patient is probably suffering from migraine without aura with coexisting cluster headaches.^4,5

Primary care doctor: The migraine diagnosis seems clear, but the diagnosis of cluster headaches seems less clear. As mentioned, cluster headaches affect men almost exclusively and present as an excruciating, severe pain. This patient, a woman, rates the intensity of her short-lasting headaches as a 7 or 8 on a 10-point scale. Thus, the pain associated with these headaches is less severe than that from migraine.

Headache specialist: The clinical reality is that cluster headaches do affect females—although with far less frequency than they do males. Remember that the duration of the headache attack is the only unique feature in these otherwise similar headache disorders. This patient complains of headache that usually lasts between 40 and 50 minutes, which points to cluster headache.

When you see an unusual presentation of a headache disorder (in this case, cluster headaches in a woman), be prepared to see atypical symptoms. For example, this patient describes the pain as moderate to severe—which is atypical of cluster headache. Also, the patient does not exhibit classic cluster-type behavior during an attack (eg, pacing, rocking). Nor does she present with the full range of autonomic symptoms (ptosis or partial Horner syndrome, lacrimation, conjunctival injection, nasal congestion) that are common manifestations of cluster headache.

Occasionally, cluster headaches manifest on different sides of the head. Such was the case with this patient.

Another observation—women with cluster headaches almost always have coexisting migraine. From this standpoint, this case is a classic presentation of a cluster headache in a female.

Primary care doctor: Is treatment of cluster headache gender-dependent?

Headache specialist: Treatment is the same for men and women. For details on therapy, we refer readers to several good published reports.^1,2,4,5

The take-home point: Do not ignore atypical symptoms. They may herald an unusual presentation of an atypical headache.