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Cluster Migraines

Cluster headache or “suicide headache” is an extremely painful (has been called the most extreme pain a human can endure) and debilitating headache. Unlike migraine, cluster headache has a high male predominance (8 to 1) and usually occurs between the age of 20 to 50 and approximately 69 out of every 100K people experience the disorder. There is a genetic component with first degree relatives being more likely to experience this type of headache. Cluster patients tend to have a characteristic appearance ruddy complexion, multi-furrowed and thickened skin, and a broad, prominent chin: all contributing to a "leonine" facial appearance. The headaches tend to have a seasonal variation i.e. occur in the fall and early spring with most patients having one or two attacks per year. Some patients can go a number of years between episodes (especially older individuals). Attacks usually occur 2 times per day (range 1 to 6 per day) typically lasting 20 to 60 minutes (however can last up to 4 hours). Patients are often awoken (approximately 2 hours after falling asleep) from sleep. Attacks can be triggered by alcohol, nitrates (hot dogs), and naps. Interestingly most cluster patients are able drink alcohol, eat hot dogs and take naps without difficulty when not in their cluster cycle. A typical cluster cycle will last 6 to 8 weeks and approximately 10% of cluster patients will go on to develop chronic cluster headache. Many chronic migraine or new onset persistent daily headache patients are incorrectly diagnosed/labeled as having cluster headache.

Cluster headache pain has been described as lancinating or boring/drilling in quality and is located behind the eye (periorbital) or in the temple. Analogies frequently used to describe the pain are a red-hot poker inserted into the eye, or a spike penetrating from the top of the head, behind one eye, or a twisting knife or screwdriver in ones eye The pain can radiate to the neck or shoulder and often times will begin a a dull or burning sensation in the occipital region (i.e. back of the head). Cluster patients will often develop mild occipital pain without the severe pain early on in their cluster cycles. Cluster headache is associated what is termed autonomic symptoms which include lacrimation from the eye on the affected side (the most common associated symptom), blocked nasal passage (on the same side as the headache), rhinorrhea (runny nose), red eye, and sweating and pallor of the forehead and cheek are often found, but their absence does not exclude the diagnosis.

Physiologically cluster headaches are felt to originate in the hypothalamus which explains the circadian nature of the headache. Like migraine, serotonin appears to be a major player. However, serotonin alterations are more subtle in patients with cluster headache than in migraine. In addition, alterations in testosterone (see treatment), melatonin and cortisol and possibly histamine appear to play a role. In fact levels of these neurochemicals and neurohormones tend to return to baseline when the cluster cycle ends. Similar to migraine there appears to be dilation of meningial blood vessels, however enhanced pulsation of the blood vessels (some have termed vasospasm) occurs during cluster attacks but not during migraine attacks. The intracranial (part inside the head) portion of the carotid artery also appears to be involved which is not a component of migraine.

There is a multi-facet approach to treating cluster headache, which employs the use of abortive, transitional and preventative medications. As cluster headaches tend to come on quickly and are short lived, abortive medications need to be fast acting and involve the use of injectable and nasal preparations. The mainstay abortive treatment is Imitrex (Sumatriptan) injection, this medication typically aborts a cluster headache in 10-15 minutes and if taken early enough the patient may never experience severe pain. The nasal form of this medication along with nasal Zomig have been shown to be effective in other studies. Dihydroergotamine (DHE-45 injection and Migranal nasal spray) have also been shown to be effective however are not as fact acting as the Imitrex injections. One unique characteristic of cluster headache is its response to oxygen. Often times oxygen response will seal the diagnosis of cluster. Many physicians however are not versed in the proper use of oxygen in cluster headache which needs to be high flow (8 liters), using a non-rebreather mask and involves the patient placing their head between their legs and breathing deeply. Unlike migraine and because of the sever nature of the pain necessitating the need to prevent the headaches whenever possible. Cluster patients require transitional medications such as prednisone, long acting triptans, and methergine while preventative medications are titrated (brought up to) to a therapeutic dose. Prednisone is by far and way the most widely prescribe transitional medication and usually started at a high dose i.e. 60 mg and decreased slowly over a period of two to three weeks and is highly favored by a number of cluster patients. The medication is not without side effects (i.e. increased appetite, bloating, fluid retention, insomnia and agitation) and when given in multiple cycles can cause osteoporosis. The mainstay of preventative treatment is the calcium channel blocker verapamil. The medication is given in the sustained release formulation (Verapamil SR or Calan) and dosed twice a day. Cluster patient often require doses that are 2 to 3 times that used for high blood pressure, i.e. 720 to over 1000 mg per day. Side effects include constipation and sexual dysfunction. For patients who are not controlled with Verapamil or cannot tolerate the medication, a second medication may be added or substituted. Other preventatives used in cluster headache include Topamax, Depakote, Lithium, Methergine and Melatonin. Some patients also benefit from serial occipital nerve blocks which our physician has extensive experience with. There are also a number of cutting edge treatments on the horizon including Psilocybin which is derived from psychedelic mushrooms and being studied in Europe, and testosterone which in a recent study presented by Dr. Conidi at the International Headache Society meeting was found to be 85% effective in terminating the cluster cycle with one or two injections.

Unfortunately and as stated above almost 10% of cluster patients will go on to develop chronic cluster headache. These patients are at high risk for suicide and it is essential that they are seen by a headache specialist with experiencing in managing chronic cluster. There are a few medical and interventional procedures currently being employed and studied and include; testosterone replacement therapy (our facility), Psilocybin (Europe), occipital and dual neurostimulation (see our headache management program) and deep brain stimulation (Italy and a few centers in the US).