The Clusterheadache

The disease

AVFClusterhaedaches are violent kind of headache. It appears as violent painful crisis affecting just one side of the face, always the same side (just some exeptions). The pain is extreme, much more than migraine, constant, and is located at the orbit of and at eye-level. It spreads to the whole side of the head.

They are generally accompanied by at least two of the following symptoms on the painful side: red eye, eye-watering, dripping nose and the syndrome of Claude Bernard-Horner.

It's sometimes described as the most painful experience that a human being can experience.

“Cluster headache is probably the worst pain that humans experience. I know that’s quite a strong remark to make, but if you ask a cluster headache patient if they’ve had a worse experience, they’ll universally say they haven’t. Women with cluster headache will tell you that an attack is worse than giving birth. So you can imagine that these people give birth without anesthetic once or twice a day, for six, eight or ten weeks at a time, and then have a break. It’s just awful.”1

The crisis generally occurs with a frequency of 1 to 8 a day, sometimes more. The crises generally last about 1H00 but can last from 20 minutes to 3 hours and occur in clusters or bunches . The crises often occur at a fixed hours and have usually at night predominance.

Who and when?

This disease generally affects young men between 15 and 45 years old. More often around the age of 30 however, (but there are many exceptions to the rule.).

This disease also affects women, to a lesser degree (sex ratio 4/1 – 10/1 depending on the source), and often at a later age.

The majority of the patients are known as episodical (episodic clusterheadache), i.e. the crises episodically occur with episodes lasting from 15 days to a few months when the daily crises are interrupted by periods of remission, which themselves can last from 15 days to several years.

According to estimations, 10 to 20% of affected people have the chronic form, (chronic clusterheadache) i.e., the crises occur almost daily for months, years, even decades without any period of remission longer than 14 days.

Causes

The intense pain is caused by the dilation of blood vessels which creates pressure on the trigeminal nerve. While this process is the immediate cause of the pain, the etiology (underlying cause or causes) is not fully understood.

This is probably due to an hypotalamus disorder, and more precisly to the region involved in the circadian rhythme. A complex hormonal interaction probably take part in this phenomenon But a lot of questions on he exact causes and path of crisis remain unanswered.

Symptoms

The International Headache Society informs us about the following diagnostic criteria:

A- at least 5 crises answering to the criteria B and D;

B- unilateral severe orbital pains, supra-orbital or temporal pains lasting 15-180 minutes without treatment;

C- cephalgia associated with at least one of the following characteristics occurring on the painful side: conjunctival injection, eye-watering, nasal congestion, rhinorrhea, sweating of the forehead and face, myosis, ptosis, oedema of the eyelid;

D- frequency of the crises from 1 to 8 per day;

E- at least one of the following characteristics: the history, the physical and neurological examination do not suggest an organic disorder; an organic disorder exists but cluster headaches do not initially appear at the same time.The crises generally last 1H00 but can last from 20 minutes to 3 hours and occur in salvos or bunches (from whence the English name of cluster). The crises often occur at a fixed hour and have usually at night.

Some figures

Taking account of the meta-analyse by Fischera et al-Cephalalgia (2008), 1/1000 adults get this condtion, that means 6.800 men in Belgium , 1/6 are chronic, so about 1100 individuals en Belgium).

Cluster headaches are often lately or badly diagnosed, and is a rare disease.

1Peter Goadsby, Professor of Clinical Neurology at UniversityCollege, London

English