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Migraine Comorbidity or Alternative Diagnosis — Hemicrania Continua

Think you have chronic Migraine? You might not…

There are times when diagnosing Migraine can be difficult. There are more than 300 different possible headache diagnoses. That’s a lot to consider for every patient walking into an office.

Our physicians always try to consider alternative diagnoses when patients come to their office with symptoms of Migraine Disease, and when unilateral chronic or intractable Migraine, chronic daily headache (CDH), new daily persistent headache (NDPH) or a trigeminal autonomic cephalalgia seems a possible culprit, it’s the wise physician who also considers and rules out a primary headache disorder called hemicrania continua.

If you have a current diagnosis of chronic or intractable Migraine, has your doctor talked to you about hemicrania continua? When hearing hoofbeats, sometimes it is not a horse but a zebra. Sometimes, it is even a unicorn.

What is Hemicrania Continua?

Hemicrania continua (HC) is a single-sided headache that does not go away and that rarely if ever changes sides, most often presenting with autonomic symptoms such as tearing or a runny nose that coincide with flares of increased pain activity. Unlike Migraine, patients often are agitated and active during their severe pain exacerbations, often even becoming verbally abusive. HC is said to be a cross-over between cluster headache (aka “suicide headaches”) and Migraine.

HC is uncommon, but cases of confusing headache diagnoses were presented and physicians were reminded at the 2011 Scottsdale Headache Symposium that HC is probably not nearly as rare as once thought, but actually very under-diagnosed.

In fact, in a recent study of 25 HC patients, not one had been appropriately diagnosed before going to the headache center, even though 85% of them had seen a physician within 6 months of the onset of their symptoms. Unfortunately, it takes the average HC patient years and several physicians to get an appropriate diagnosis.

Appropriate diagnosis is critical because hemicrania continua is very successfully treated with one particular medication — a prescription NSAID – which can be life changing for the patient who has most often endured years of nearly constant pain and disability.

The (IHS) International Headache Society’s ICHD-II (International Classification of Headache Disorders most recent version) uses the following criteria for hemicrania continua diagnosis:

Description:

Persistent strictly unilateral headache responsive to indomethacin.

Diagnostic criteria:

  1. Headache for >3 months fulfilling criteria B-D
  2. All of the following characteristics:
    1. unilateral pain without side-shift
    2. daily and continuous, without pain-free periods
    3. moderate intensity, but with exacerbations of severe pain
  3. At least one of the following autonomic features occurs during exacerbations and ipsilateral to the side of pain:
    1. conjunctival injection and/or lacrimation
    2. nasal congestion and/or rhinorrhoea
    3. ptosis and/or miosis
  4. Complete response to therapeutic doses of indomethacin
  5. Not attributed to another disorder

It is noted in ICHD-II that HC may rarely remit, but is usually continual.

There is also question whether the condition may be further subdivided. ICHD-III is due out in the next couple of years and it looks like change is in the air. It will be interesting to see if (how) the IHS changes the way we currently diagnose HC. Here is more information on changes that are being discussed.

Treating HC

HC is considered completely (100%) responsive to therapeutic doses of a drug called indomethacin. Indomethacin is a potent NSAID with powerful anti-inflammatory and other properties. Its chemical makeup is close to that of the brain chemical melatonin. In fact, some patients who may not tolerate the significant side effect profile of indomethacin may in fact be responsive to melatonin either in conjunction with decreased doses of, or in place of indomethacin.

It’s interesting to note that there can be a lag time after beginning treatment and before the indomethacin is effective, and physicians are encouraged not to be shy about using a higher than *normal* dosage to be sure the trial is sufficient before ruling HC out.

Because of HC’s responsiveness to indomethacin, many headache specialists choose a clinical trial of a therapeutic dose (often suggested to be 75 mgs) of the drug in patients they suspect may have HC or a similar presenting condition such as chronic Migraine or chronic daily headache.

Indomethacin is known to cause significant gastric and other side effects.

Additionally, for patients with gastric stasis symptoms, absorption may be a problem. Headache specialists will often suggest patients bypass the oral route normally used for indomethacin, prescribing compounded suppositories to use in place of oral tablets. Injections are also available, but much less frequently utilized often due to cost, availability and patient compliance. Bypassing the oral route unfortunately does not eliminate gastric side effects, but they can often be proactively treated with prescription and/or OTC medications when indomethacin treatment is started.

Start a conversation with your doctor

Migraine (and chronic Migraine) is a diagnosis of exclusion, and because of its disabling nature, excluding an indomethacin responsive headache disorder like HC is something that should be done to be sure patients are receiving appropriate treatment. Most headache specialists are aware of this and are prepared to help their patients try the drug, however non headache specialists are often too quick to diagnose other chronic conditions or psychological disorder in the absence of such a test, to the detriment of their often disabled patients.

Proactive patients with chronic, intractable (does not respond to treatment) unilateral (one side only, with no side shift) headache, especially with autonomic features such as tearing or runny nose, will want to be sure to educate themselves about HC and use this information to begin a conversation with their physician.

This article represents the opinions, thoughts, and experiences of the author; none of this content has been paid for by any advertiser. The Migraine.com team does not recommend or endorse any products or treatments discussed herein. Learn more about how we maintain editorial integrity here.

1 Newman, Larry, MD, FAHS. Tepper,Stewart J, MD. “Trigeminal Autonomic Cephalalgias and Other Primary Headaches “ Scottsdale Headache Symposium. November, 2011.2 Cittadini, Elisabetta, MD. Goadsby, Peter, MD, PHD, FAHS. “Update on Hemicrania Continua.” Curr Pain Headache Rep. 2011 Feb;15(1):51-6.Available at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3021804/?tool=pubmed 3Full ICHD-II Headache Classification Subcommittee of the International Headache Society. “The International Classification of Headache Disorders 2nd Edition.” Cephalalgia 2004;24:8-160.

Comments

  • travelape54
    5 years ago

    As a long time sufferer of Hemicrania, I want to like this article but I just cannot. Not all people with Hemicrania respond to Indomethacin, there are several people I know who have to have supplemental medications that help relieve the pain. Secondly, I have been to several headache specialists and none of them have suggested the suppository for the indo, and I have never heard of anyone taking it this way. Although Melatonin is a good supplement to take, Boswellia has more research and better results treating HC. Botox has also been know to be an effective treatment for HC but there is no cure for HC. Even if the indomethacin works most people cannot tolerate it because it is so difficult on their stomachs. I was able to take it for only three months, if it weren’t for the Boswellia and Botox, I wouldn’t be able to function.

  • Janet
    6 years ago

    ellen
    as always your articles are most informative and you choose the right ones to repost as well. i am doing better than before but have been slammed the last 13 days. same migraine…won’t let up. lots of stress…we are moving from las vegas to atlanta. i leave this monday morning and have the stress of 2 walk throughs on my own…as i did on this house 20 years ago. sold our home here 2 months ago and the buyer backed out 1 week ago. the hopes of selling before the end of the year are next ti nil. thus the migraine. i hope to stay in touch and keep informed on migraine progress in the doctor world and with the migraineur population.
    janet jones

  • Zev
    7 years ago

    Hmmm……don’t know what to say except that I’m overwhelmed. Having a dignoses of Chronic Migraines w/Clusters, Icepick headaches and Chronic Daily headache….it’s a bit much to deal with. I will take this to my Dr. and see what they say, certainly it’s worth discussing. My migraines seem to be under control yet, the other headaches (CDH, Cluster, etc) we’ll ….life just goes on. Thank you for the info.

  • taralane
    7 years ago

    Hi Ellen – I posted a rather long reply under the first blog article you posted, and I don’t want to be redundant. The symptoms still sound very much like my migraine symptoms, although now I can add aura and an occasional move to the opposite side, very rare, which was not present when I was first diagnosed with HC. I still have an ice-pick going down through my head, eye, and into my nose every day at varying pain levels, can have a runny nose and eye tearing, but indomethecin never worked on my migraine – the daily or acute attacks. Don’t know what to think of the new information. I’ll discuss it again with my doc., but am now, just confused.

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