Paroxysmal Hemicrania: What It Is and How It Is Managed

Written by Medicover Team and Medically Reviewed by Dr Krishna Haskar Dhanyamraju , Neurologists

Paroxysmal hemicrania is a rare primary headache disorder that causes repeated attacks of severe pain on one side of the head, usually around or behind the eye, temple or forehead. The attacks are short but can occur many times throughout the day and are often accompanied by symptoms such as tearing, eye redness or nasal congestion on the same side as the pain.

Paroxysmal hemicrania belongs to a group of headache disorders called trigeminal autonomic cephalalgias. A characteristic feature is the complete response of the attacks to therapeutic doses of indomethacin, under medical supervision.

What Are the Types of Paroxysmal Hemicrania?

Paroxysmal hemicrania is generally classified according to how long the disorder continues and whether symptom-free periods occur.

Chronic Paroxysmal Hemicrania

Attacks occur for at least one year without remission or with remission periods lasting less than three months.

Episodic Paroxysmal Hemicrania

Attacks occur in periods separated by symptom-free remissions lasting at least three months.

What Are the Symptoms of Paroxysmal Hemicrania?

The main symptom is a sudden, severe headache affecting one side of the head. Typical features include:

The pain usually remains on the same side during individual attacks. Unlike migraine, attacks are typically much shorter and occur much more frequently.

What Causes Paroxysmal Hemicrania?

The exact cause of paroxysmal hemicrania is not known. It is considered a primary headache disorder, meaning that the headaches are not usually caused by another underlying disease.

Researchers believe that abnormalities involving pain pathways and the autonomic nervous system may contribute to the condition. In some people, head or neck trauma has been reported before the onset of symptoms, but a clear cause cannot be identified in most cases.

When Should You See a Doctor for Paroxysmal Hemicrania?

See a doctor if you experience frequent, severe or recurring one-sided headaches, particularly when the attacks occur several times a day or are associated with eye or nasal symptoms.

Medical evaluation is particularly important if you experience:

A neurologist, particularly a doctor specializing in headache disorders, can evaluate the symptoms and distinguish paroxysmal hemicrania from other conditions such as cluster headache, migraine and other trigeminal autonomic cephalalgias.

Seek urgent medical care for a sudden, extremely severe headache, especially if it reaches maximum intensity within seconds or is accompanied by neurological symptoms, fever, stiff neck, loss of consciousness or significant vision changes.

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How Is Paroxysmal Hemicrania Diagnosed?

Diagnosis is based primarily on the pattern of headache attacks, associated symptoms, neurological examination and response to treatment. Doctors may also perform tests to exclude other causes of one-sided headaches.

Medical History and Headache Diary

Your doctor may ask about the location and severity of pain, duration of each attack, number of attacks per day, associated eye or nasal symptoms and possible triggers. Keeping a headache diary can help document these patterns.

Neurological Examination

A neurological examination can help identify signs suggesting another neurological condition and determine whether additional investigations are necessary.

Brain Imaging

An MRI or other imaging study may be recommended, particularly when headaches are new, atypical or associated with concerning findings. Imaging helps rule out structural causes that can produce similar symptoms.

Indomethacin Response

A complete response to an appropriate therapeutic trial of indomethacin is an important feature of the diagnosis. Because indomethacin can cause significant gastrointestinal, kidney and cardiovascular side effects, an indomethacin trial should only be performed under medical supervision.

What Are the Treatment Options for Paroxysmal Hemicrania?

Paroxysmal hemicrania is highly responsive to treatment when the diagnosis is correct. The main treatment is indomethacin, a nonsteroidal anti-inflammatory drug (NSAID).

Indomethacin

Indomethacin is the standard treatment and can completely prevent attacks in people with paroxysmal hemicrania. The dose and duration are determined by the treating physician based on the individual's response and risk of side effects.

Because prolonged indomethacin use can cause gastrointestinal bleeding, kidney problems and other adverse effects, regular medical monitoring may be necessary.

Alternative Medicines

If indomethacin cannot be tolerated or is contraindicated, a specialist may consider alternative medications in selected cases. These may include other NSAIDs, COX-2 inhibitors or certain other headache medicines. However, these alternatives generally do not have the same established effectiveness as indomethacin.

Managing Triggers

Some people notice that particular activities, such as bending or turning the head or applying pressure to the neck, can trigger attacks. Identifying and avoiding individual triggers may help reduce episodes, although medication remains the mainstay of treatment.

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What Are the Risk Factors for Paroxysmal Hemicrania?

Paroxysmal hemicrania can affect people of different ages, although it is more commonly recognized in adults. It is rare, and women may be affected more often than men.

Some reported factors or associations include:

Having one of these factors does not mean that a person will develop paroxysmal hemicrania.

What Is the Outlook for People With Paroxysmal Hemicrania?

Paroxysmal hemicrania is generally treatable, and many people experience complete or substantial control of their headaches with appropriate treatment. Some people require long-term medication, while others may experience periods of remission depending on the subtype.

Regular follow-up can help monitor treatment effectiveness, adjust medication and identify potential side effects.