What are the advances in the management of cluster headache?

Cluster headaches are nasty, excruciatingly severe, headaches. They are not called suicide headaches without good reason. Cluster headaches are typically one-sided, localised around the orbit. The eye on the affected side classically turns red and waters. The nostril follows suit by either running or blocking up. The episodes last between 45 minutes to 3 hours during which the hapless victims pace up and down, feeling like smashing their heads against a concrete wall. Relief is short-lasting because the headache cycle repeats itself several times a day, for weeks and months on end. People with episodic cluster headaches may go several months without headaches, but those with the chronic form are not afforded this luxury.

Lego splitting headache. Matt Brown on Flikr. https://www.flickr.com/photos/londonmatt/15191073177
Lego splitting headache. Matt Brown on Flikr. https://www.flickr.com/photos/londonmatt/15191073177

Treatment of cluster headache is typically three-pronged: acute treatment with triptansintermediate prevention with oral steroids; and prevention with verapamil. OK, I over simplify. Each of these strategies has 2nd, 3rd, and 4th line options. Verapamil, the cornerstone of treatment, comes with significant risks to the heart. And in extreme cases, invasive measures are called upon to save the day.

By Hansjorn - το :Αρχείο:Poseidon sculpture Copenhagen 2005.jpg, CC BY-SA 3.0, Link
By Hansjorn – το :Αρχείο:Poseidon sculpture Copenhagen 2005.jpg, CC BY-SA 3.0, Link

Unfortunately all these treatments fail miserably more often than we like to admit. Even invasive treatments are not always successful in cluster headaches. Neurologists and patients alike are therefore always on the lookout for developments which will improve the understanding and management of cluster headaches. And, thankfully, there are a few.

A. Abnormal tyrosine metabolism and cluster headache

By No machine-readable author provided. Benjah-bmm27 assumed (based on copyright claims). - No machine-readable source provided. Own work assumed (based on copyright claims)., Public Domain, Link
By No machine-readable author provided. Benjah-bmm27 assumed (based on copyright claims). – No machine-readable source provided. Own work assumed (based on copyright claims)., Public Domain, Link

The sad fact about cluster headache is, nobody knows what causes it. We know it is due to some malfunction of the autonomic nervous system, and to the trigeminal, or fifth, cranial nerve. This is why it is called a trigeminal autonomic cephalalgia. We know that it favours men who smoke. Beyond this we are rather clueless. It is therefore with high hopes that I read about abnormal tyrosine metabolism in chronic cluster headache, in the journal Cephalalgia. The authors report that people with cluster headaches have high levels of the products of tyrosine metabolism in their blood, such as dopamine, noradrenaline, and tyramine. If this turns out to be confirmed, it may open the way to the development of newer and more effective treatments for this painful condition.

B. Heart monitoring on verapamil

https://pixabay.com/en/pulse-trace-healthcare-medicine-163708/
https://pixabay.com/en/pulse-trace-healthcare-medicine-163708/

The heart is at risk whenever people are put on verapamil. This is because it may induce abnormal and dangerous heart rhythms. It is therefore important to check the electrocardiogram (ECG) of people on verapamil. Guidelines suggest checking the ECG before starting, 10 days after starting, and before each dose increment. It was therefore disconcerting that a recent study, published in the journal Neurology, found that 40% of people on verapamil never had any form of heart monitoring. The paper, titled electrocardiographic abnormalities in patients with cluster headache on verapamil therapy, is an audit of >200 people with cluster headaches on high dose verapamil. In those who had cardiac monitoring, the authors found ECG abnormalities in more than 50%, some very significant and life threatening. A similar finding was reported in an older study published in the Journal of Headache and Pain, titled cardiac safety in cluster headache patients using the very high dose of verapamil (≥720 mg/day). Worrying! Time to take ECG monitoring more seriously in people on verapamil. 

C. New preventative drug options

By Stomac - Own work, CC BY-SA 2.0 fr, Link
By StomacOwn work, CC BY-SA 2.0 fr, Link

Besides verapamil, there are many other options for cluster headache prevention. The list is quite long, and this is the case whenever we are uncertain of what else really works. That is why I was relieved to see a recent guideline on treatment of cluster headaches touting new evidence to guide neurologists. Published in the journal Headache, it is titled Treatment of Cluster Headache: The American Headache Society Evidence-Based Guidelines. This guideline establishes that lithium is effective in preventing cluster headache, but valproate is probably ineffective. More importantly, the guidelines introduce new effective preventative agents such as civamide nasal spray, melatonin, and warfarin. For transitional prevention, occipital nerve injection comes through with glowing tributes. Progress, surely!

D. Neurostimulation for cluster headache

By C. Clark - NOAA Photo Library (direct), NOAA Central Library; OAR/ERL/National Severe Storms Laboratory (NSSL), Image ID: nssl0010, Public Domain, Link
By C. Clark – NOAA Photo Library (direct), NOAA Central Library; OAR/ERL/National Severe Storms Laboratory (NSSL), Image ID: nssl0010, Public Domain, Link

It is no longer surprising to find neurostimulation cropping up in the treatment of any neurological disorder. And cluster headache is no exception. The most effective agent, according to the latest guidelines, is sphenopalatine ganglion stimulation. It now ranks very high in the acute treatment of cluster headache, even if less effective than the good old, conventional acute treatments which are subcutaneous sumatriptan, intransal zolmitriptan, and 100% oxygen. Neurostimulation is also likely to play a future preventative role in cluster headaches, and the candidates here are invasive and non-invasive vagus nerve stimulation. We are waiting with bated breaths!

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For more on vagus nerve stimulation, you may check out my previous post titled Vagus nerve stimulation: from neurology and beyond!

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