Cluster headache: how to recognise and manage it?

By Cristiano Antonino On Jan 26, 2023

Sudden onset and extreme intensity: these are the main characteristics of cluster headache

Cluster headache is an uncommon form of primary headache that – although it affects less than 0.2 per cent of the general population – is the worst for the patient.

Cluster headache sufferers experience such acute forms of pain that they engage in extreme, borderline bizarre behaviour

How to recognise cluster headache? What medication to take and what behaviour to adopt to limit attacks? Are there differences between males and females in the manifestation of the disorder?

HOW TO RECOGNISE CLUSTER HEADACHE?

Cluster headache, a unilateral cyclic headache, tends to occur in the middle stages of life, between the ages of 20 and 40.

However, in women there appears to be a bimodal pattern with two peaks: an earlier one in their thirties and a later one in their sixties.

In contrast, there is no evidence of a correlation with ovarian function, unlike in migraine.

Cluster headache causes extreme and obvious suffering to the patient who finds no relief and finds himself in a kind of pain-related panic.

The main clinical manifestations are sudden onset intense pain in the eye, totally disabling, accompanied by agitation and restlessness.

Associated manifestations are lacrimation, reddening of the eye, swelling and drooping of the eyelid, together with nasal stuffiness and dripping from the nostril, as well as sweating and warmth.

The patient’s behaviour during a cluster headache attack, completely unheard of in other forms of acute pain, can be very extreme, bordering on the bizarre.

They may bang their head against the wall or on the floor, they may engage in self-destructive behaviour, for instance beating themselves up to the point of fracturing themselves.

They can also become violent with those who approach them trying to bring them comfort, which is why headache attacks can also become a problem in terms of family serenity.

The term cluster headache is well known and used, but where does it come from and what information can it give us about this disorder?

The most unmistakable clinical connotation of cluster headache is its cyclic pattern with active and remission phases.

It is precisely from this temporal pattern that the name ‘cluster’ derives as it refers to the grouping of crises in periods of activity, called ‘clusters’, of variable duration of weeks or months, during which the crises can also be multi-day, characteristically at fixed times of recurrence in the twenty-four hours, both at night and during the day.

The attacks have a variable spontaneous duration, ranging from a minimum of 15 minutes to a maximum of three hours, and may repeat up to eight times during the day.

Two different forms of cluster headache can be distinguished.

Episodic forms are those in which the remission period lasts from at least three months up to several years, and are the most frequent (about 90% of all cluster headaches).

Then there are the chronic forms where the remission period, if present, lasts less than three months.

Stability over time is recognised in about 60% of cases, with potential evolution, in a small proportion, from the chronic to the episodic form, and vice versa.

DIFFICULT DIAGNOSIS

Although the clinical presentation is rather striking, as we have seen, timely diagnosis is by no means a given.

Cluster headache, in fact, is largely unrecognised, with a diagnostic delay of up to a decade or more.

The acute pain disorder is often mistakenly associated with a variety of other causes such as odontalgia, sinusitis and neuralgia, which leads to multiple visits to the emergency room, multi-specialist evaluations, inappropriate procedures such as completely unnecessary dental avulsions or unsuitable pharmacological treatment with courses of antibiotics, steroids or anti-epileptics.

…ESPECIALLY FOR WOMEN

The diagnostic delay seems to affect women in particular, as evidenced by a study soon to be published in the journal Neurology involving a thousand patients.

Moreover, despite being a typically male pathology, research shows that women are more likely to experience severe forms, perhaps due to this diagnostic delay.

In fact, 18% of women, compared to 9% of men, were diagnosed with the chronic form.

The diagnostic delay can partly be explained by the presence of confounding migraine-like symptoms, especially in women, such as nausea, vomiting, hypersensitivity to environmental stimuli and the need for isolation.

CLUSTER HEADACHE, THE PATIENT’S IDENTIKIT

The phenomenon of cluster headache is quite complex, and each individual faces different symptoms, frequency and intensity.

But are there any traits that sufferers have in common?

Cluster headaches are typically experienced by individuals with a dynamic temperament, hyperactive, passionate, very determined, often successful in their field of employment, sometimes career managers burdened with numerous responsibilities, and therefore constantly under stress.

Apparently it is precisely this condition of hyperactivity that gives them relative protection from headache attacks.

The crises seem to be modulated by their own rhythms: a slowdown, a pause and a drop in tension are enough to give way to the triggering of the attack.

Typically it happens in the post-prandial phase or at the end of the day, if not during the night’s rest.

These individuals are often heavy users of caffeine, cigarettes, alcohol and sometimes even illicit substances: it is a compulsion set in place partly to support the high pace of hyperactivity and probably to buffer an emotional state that is always kept under control.

BEYOND CLUSTER HEADACHE

For cluster headache sufferers, unfortunately, painful and cyclic attacks are not the only health problem they face.

Another study to be published shortly in the journal Neurology, in fact, after analysing data from over 3,000 cluster headache sufferers, recognises a threefold greater likelihood of suffering from other pathologies than controls.

In particular, these are general disorders of the nervous system, mental and musculo-skeletal disorders, with a strong impact on lost working days and disability levels, which are always higher in the female sex.

Even within the limitations of the publication, which lacks the distinction between Episodic and Chronic forms, the greater risk of comorbidity clearly emerges.

In the subjects analysed, all under 64 years of age, many cardiovascular and metabolic risk factors, conditions of overweight, snoring and head injuries, linked to an unhealthy lifestyle, often punctuated by excesses in behaviour, are detected.

PHARMACOLOGICAL TREATMENT

Are there effective drugs to eliminate pain and perhaps prevent future attacks?

For acute attacks, precisely because of their sudden and violent onset, the active ingredient sumatriptan, discovered in the 1990s, is the only effective symptomatic drug, as it is the only existing triptan in an injectable subcutaneous formulation that is fast-acting within a few minutes.

High-flow inhaled oxygen may also be rapidly effective.

In both episodic and chronic forms of cluster headache, taking certain drugs may help to prevent or mitigate the onset of a new series of attacks, such as verapamil, lithium, and cortisone.

A new prophylactic therapy based on monoclonal antibodies capable of blocking the calcitonin gene-related peptide (CGRP), a substance involved in the disease, is currently in advanced trials.

Evidence of efficacy, which has earned FDA approval, has however only been observed in episodic forms of cluster headache.

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