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Chronic Paroxysmal Hemicrania

Chronic paroxysmal hemicrania (CPH) is a unique headache syndrome that falls into a category known as "indomethacin responsive headaches." It is very similar to cluster headache but is less common. In fact, some authors have labeled CPH as "atypical cluster headache."

CPH patients have multiple headaches per day (on average greater than 5), more than what is seen in cluster headache. Each attack is usually short-lived, with most lasting between 5 and 30 minutes. The pain is always one-sided, very severe, and seems to be concentrated around the eye, temple, and forehead region. There are associated symptoms of watering of the eye and eye redness; even drooping of the eyelid has been reported.

Unlike cluster headache, CPH is usually seen in females. Children are not immune to this condition-it has been reported to occur in girls as young as six years old. Although patients affected may wake up with an attack, this does not seem to occur as regularly as it might in persons with cluster headache. In addition, the nausea and vomiting which seem to be so prevalent for patients with migraine are not seen in CPH.

Individuals who experience CPH may have remissions of their attacks and pain, at times lasting many months. They may also not be able to identify specific triggers for onset of another attack.

While we have not yet identified the specific pathophysiology (underlying physical cause) behind CPH, it seems that the trigeminal nerve and hypothalamus is involved. Unfortunately, many treatments that are effective in treating other forms of pain caused by a similar mechanism do not seem to work when treating CPH. One medication that will always eliminate CPH is indomethacin; hence the term "indomethacin responsive headaches." For patients who are unable to use this medication, other treatment strategies are being evaluated. However, other headache medications, such as triptans, usually do not have an effect on CPH.

Although MHNI physicians are usually able to make a diagnosis of CPH based on a person's history and clinical symptoms, there are underlying lesions in the brain that can lead to similar symptoms and attacks (e.g., tumors, arteriovenous malformation). Because of this, various tests of the brain are recommended to exclude structural abnormalities.

Key clinical features

  1. Pain is one-sided and usually localized to the eye, ear, temple, forehead, or lower jaw region.

  2. A range of pain forms (throbbing, boring, stabbing, etc.) is possible, with moderate to severe intensity. Persisting pain between acute attacks is reported.

  3. Attacks of pain last 2-25 minutes, averaging 10-15 minutes (range 2-120 minutes). (Cluster headache attacks have a mean duration of 45 minutes.)

  4. Patients generally prefer quiet and rest; some patients pace, as in cluster headache.

  5. Attack frequency may be up to 40 per day but average 10-20 attacks per day.

  6. In the episodic form, the headache phase is 2 days to 4.5 months. Remission is 1-30 months.

  7. Nighttime awakening occurs.

  8. Same side symptoms occur, similar to those in cluster headache:

    • eye watering/nasal congestion or discharge/eye congestion or redness;

    • forehead perspiration;

    • mild contraction of the pupils;

    • eyelid swelling;

    • light sensitivity;

    • nausea;

    • slow/fast heart rate; and

    • eyelid drooping.

  9. Headache can be provoked by movement in 10% of cases; pressure on cervical region may precipitate an attack.

  10. Provocation by alcohol.

Patients will frequently resort to excessive aspirin or over-the-counter (OTC) ibuprofen to control pain, perhaps due to the similarity in effect of indomethacin.

Some authorities, including Dr. Saper and the medical staff at MHNI, believe that the neck is sometimes involved as a cause, and neck treatments of various types can be helpful. Abnormalities around the pituitary gland can also mimic CPH, and this area must be examined by MRI scans.