Paroxysmal hemicrania

Definition

Paroxysmal hemicrania is a rare form of headache that usually begins in adulthood. Patients experience severe throbbing, claw-like, or boring pain usually on one side of the face; in, around, or behind the eye; and occasionally reaching to the back of the neck.

Summary

• Attacks of paroxysmal hemicrania typically occur from 5 to 40 times per day and last 2 to 30 minutes.
• The hallmarks of this syndrome are the relative shortness of the attacks and the complete response to indomethacin therapy.

Forms

• The disorder has two forms: chronic, in which patients experience attacks on a daily basis for a year or more, and episodic, in which the headaches may remit for months or years.
• Episodic paroxysmal hemicrania patients have bouts lasting from 7 days to 1 year and separated by pain-free periods more than 3 months, whereas in chronic patients the pain-free period is less than 3 months.

Cause

The cause remains unclear.

Inheritance

Rare cases of familial PH have been reported.

Onset

The mean age of onset is 40 years (range 3-81 years).

Signs and symptoms

• PH patients experience purely unilateral, severe to very severe attacks of head pain.
• The pain is often in the temporal, orbital or supraorbital region.
• The pain has an abrupt start and cessation and is accompanied by ipsilateral autonomic features, including lacrimation, conjunctival injection, rhinorrhoea, nasal congestion, periorbital oedema, facial flushing, miosis and/or ptosis.
• PH attacks last 2-30 minutes and occur more than 5 times a day up to 40 times a day with a mean of 11 a day.
• Patients can have unilateral photophobia and phonophobia ipsilateral to the side of the attack.
• Osmophobia, nausea or vomiting during the attacks has been reported.
• Although attacks are most usually spontaneous, attacks can be triggered by neck movements, or pressure over the neck or greater occipital nerves.
• As this is a primary headache disorder, patients have normal neurological tests.

Diagnosis

• Diagnosis is based on clinical history and response to indomethacin.
• Patients with suspected paroxysmal hemicrania should undergo an oral indomethacin trial or placebo-controlled intramuscular indomethacin test.
• In adults, one could start with 25mg three times a day, titrating up to 75mg three times a day over the course of 3 weeks.
• With intramuscular indomethacin 100-200 mg could be used.

Treatment

• The nonsteroidal anti-inflammatory drug (NSAID) indomethacin often provides complete relief from symptoms.
• Other less effective NSAIDs, calcium-channel blocking drugs (such as verapamil), and corticosteroids may be used to treat the disorder.
• Patients with both paroxysmal hemicrania and trigeminal neuralgia (a condition of the 5th cranial nerve that causes sudden, severe pain typically felt on one side of the jaw or cheek) should receive treatment for each disorder.

Prognosis

• Many patients experience complete to near-complete relief of symptoms following physician-supervised medical treatment.
• Paroxysmal hemicrania may last indefinitely but has been known to go into remission or stop spontaneously.

NIH genetic and rare disease info

• NIH info on Paroxysmal hemicrania

Paroxysmal hemicrania is a rare disease.

Paroxysmal hemicrania Resources
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