Cluster Headaches and Misdiagnoses

The Spanish Society of Neurology (SEN) has warned that more than 57 percent of cluster headache patients initially receive erroneous diagnoses, since the symptoms can be confused with other more common pathologies, such as sinusitis, glaucoma or other types of headache.

The SEN has highlighted that the delay in detection can exceed three years. In some studies carried out in Spain, delays of close to five years have even been described from the appearance of the first symptoms to obtaining a correct diagnosis.

“Cluster headache is the most common trigemino-autonomic headache,” explained the Coordinator of the Headache Study Group of this scientific society (GECSEN), Dr. Roberto Belvis, who added that this “is characterized by recurrent episodes of extremely severe headache, generally located on one side of the head, especially in the region of the eye and forehead.”

As he explained, “the pain appears abruptly, reaches its maximum intensity in a few minutes and usually last between 15 minutes and three hours. “Due to the intensity of the pain, cluster headaches have been described on numerous occasions as one of the most serious pains that humans can experience,” he indicated, with 50,000 people suffering from it in Spain.

This is a rare, but extremely disabling neurological pathology, of which Every year around 1,000 new cases are diagnosed. It usually debuts mainly between the ages of 20 and 40 and is more common in men. Traditionally, a ratio with women has been described as close to 4:1, although recent studies suggest that this difference is reducing and could currently be around 3:1.

The pain attacks of this type of headache are grouped in periods called ‘clusters’, in which they can be repeated several times a day for weeks or months, followed by other complete remission in which the patient remains symptom-free. These attacks often have a marked pattern, as many patients experience attacks at the same time of day – usually at night – and at certain times of the year.

In any case, the most common form is episodic cluster headache, in which patients have attacks followed by pain-free periods. However, approximately up to 20 percent of patients develop chronic forms of the disease, cases in which attacks occur for more than a year without remission or with pain-free periods of less than three months.

“The pain of a cluster headache is usually accompanied by other symptoms on the same side pain, such as watery eyes, red eyes, stuffy or runny nose, drooping of the eyelid, or facial sweating,” Belvís reported, adding that, “during crises, patients usually show a lot of restlessness or agitation and feel the need to move, which contrasts with other headaches, such as migraine, in which the patient usually prefers to remain at rest.”

Another aspect highlighted by the SEN is that this pathology generates a high burden of disability, More than 75 percent of patients present significant limitations in their daily life and up to 45 percent associate depression. Different national investigations show that up to 36 percent have lost their jobs due to the disease and that around 32 percent have had to reduce their work activity by at least half.

Treatment

Regarding the treatment, Belvís has stated that “it is based on three pillars”, which are crisis, transition preventive and maintenance preventive. However, “although there are effective treatments, cluster headaches continue to be undertreated,” he stressed, given that studies carried out by the SEN confirm that more than 50 percent of patients do not receive adequate preventive treatment and that more than 30 percent do not have access to effective symptomatic treatments, especially oxygen therapy, which is one of the most optimal for aborting attacks.

In fact, in patients with chronic forms refractory to pharmacological treatment, advanced surgical strategies can be considered, such as neuromodulation techniques performed in accredited units such as the Centers, Services and Reference Units (CSUR). In Spain, it is estimated that there could be between 500 and 1,000 patients with refractory chronic cluster headache, who are those with the greatest disability and require follow-up in specialized units.