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Indomethacin Responsive Headaches
Paroxysmal Hemicrania – Hemicrania Continua – Triggers – Symptoms – Treatment – Prognosis
Indomethacin responsive headaches are less common female counterparts of cluster headache. They belong to the group of trigeminal autonomic cephalgias. Their symptoms share many common features with cluster headaches, such as type of pain and similar associated symptoms. What makes them unique is 100% response to the treatment with Indomethacin. No matter how alike the pain is with Hemicrania Continua or Paroxysmal Hemicrania, lack of pain relief in response to the treatment with Indomethacin rules out either diagnosis.
Pain Characteristics in Paroxysmal Hemicrania
Paroxysmal hemicrania is a rare headache syndrome. It is characterized by brief and frequent headache attacks. The headache is severe, pulsating, boring, claw-like pain behind the eye or around the ear on one side.Sides may switch from time to time. Pain may radiate to the shoulder. A typical attack lasts from 2 to 30 minutes with mild residual pain in between the attacks in some patients. Unlike cluster headache, headache in paroxysmal hemicrania recur multiple times per day without any specific time preferences.
Associated symptoms of paroxysmal hemicrania in the order of decreasing frequency are tearing, stuffed nose, eye redness, runny nose, and droopy eyelid. Light and noise sensitivity are common. All of the associated symptoms (including sensitivity to light and noise) are limited to the side of pain. Some patients pace back and forth like in cluster headaches, others feel more comfortable in a quiet environment like in migraine.
Triggers are not essential for this headache syndrome but about a third of patients do report specific triggers. About 10% of paroxysmal hemicrania attacks are provoked by mechanical triggers – specifically, by the neck flexion, extension, rotation, or applying pressure on the side of the upper or mid neck. Alcohol triggers attacks only in about 1/5th of the patients. Birth control pills and pregnancy do affect the severity of paroxysmal hemicrania, but the pattern is unpredictable.
Paroxysmal Hemicrania Additional Facts
Paroxysmal hemicrania is a rare condition. It is about 100 times less common than cluster headache. Long thought 3:1 female predominance was questioned in the recent study but I have not seen a single male with this headache type. Headaches can start at any age; most commonly during the third decade. About one third of the patients have episodic paroxysmal hemicrania. In a typical episodic paroxysmal hemicrania, a bout of headache lasts from weeks to months with remission from one month to three years. The rest have a chronic form of paroxysmal hemicrania characterized by lack of any remissions for more than a year or remissions lasting for less than a month. Natural history is not very well known in this disorder. It is not clear if it is lifelong or a self-limiting syndrome. The mechanism (pathophysiology) of paroxysmal hemicrania is poorly understood. Paroxysmal hemicrania appears to be produced by the hypothalamus in a manner similar to cluster headaches.
Treatment of Paroxysmal Hemicrania
Paroxysmal hemicrania has another name – Indomethacin responsive headache. So, by definition, it is unconditionally responsive to Indomethacin. Indomethacin dose is ranging from 75 mg to 225mg per day, divided in 3 doses. Response to Indomethacin is usually long lasting. Considering chronic nature of paroxysmal hemicrania, large doses of Indocin over a long period of time may be challenging for the stomach and kidneys. Preventive treatment with Verapamil offer relief to some patients. Triptans, Topamax, Celebrex, Occipital nerve lidocaine/steroid injections offer some benefit to a small percentage of patients.
Headache Characteristics in Hemicrania Continua
Headache is typically localized to the eye and temple areas. Pain is mild-to-moderate, constant, 24/7 and is limited to one side, which is only rarely switched to the opposite one. Besides modest above mentioned constant pain, there is episodic exacerbation of pain in the same area. During headache exacerbation attacks milder pain escalates to moderate-to-severe. Frequency of headache exacerbations is variable, from multiple per week to once in a few months. Periods of headache exacerbation may last from hours to days.
Escalation of headache intensity is associated with similar to cluster headache or paroxysmal hemicrania symptoms, such as tearing, droopy eyelid, runny and stuffy nose. Other typical symptoms are eyelid swelling, eyelid twitching, sensation of sand in the eye, and an “ice-pick” headache (jolt of a sudden brief headache). Migraine-specific associated symptoms like nausea, vomiting, light and noise sensitivity, and aura are not unusual either.
Statistics and Prognosis of Hemicrania Continua
Hemicrania continua is a rare primary headache with female predominance. Natural history and age of onset of hemicrania continua are not clear. Eighty five percent have a chronic form without any remissions. This condition is often diagnosed as chronic daily headache, so its actual incidence is probably underestimated. Chronicity of the symptoms raises concerns about conditions such as temporal arteritis, brain tumor, carotid dissection or a blood clot in the brain venous sinuses. Brain MRI and MRA, Neck MRA, Brain MRV, Blood tests for ESR and CRP are essential. Natural history of hemicrania continua is not known. Some patients were able to stop treatment without symptoms recurrence.
Treatment of Hemicrania Continua
The hallmark of hemicrania continua is a dramatic and universal resolution of pain after the first (sometimes second) dose of Indomethacin. All associated symptoms and auras are completely controlled as well. The effect of this treatment is long lasting. Headaches come back within a couple of days after Indomethacin is stopped. Minimal effective dose of Indomethacin is variable. I had a patient who was doing well on one 25 mg pill every other day but this is rather an exception. No alternative therapy comparable with Indocin is available. A single patient of mine had an definite complete pain control on high dose of Melatonin after living in pain for 5 years.