Paroxysmal Hemicrania, Hemicrania Continua: the little cousins of cluster headache
My physician has told me that I might have Paroxysmal Hemicrania (PH) or Hemicrania Continua (HC). I never heard about those before.
PH and HC are very rare. Even headache specialists do not see them very often. They are in the family of trigemino-autonomic cephalalgias, like cluster headache (See this post).
Key characteristics of paroxysmal hemicrania and hemicrania continua
The pain is on one side of the head only: we call these side-locked headaches, or strictly unilateral headaches.
There are other symptoms, on the side of the pain: droopy eyelid, teary eye, runny nose, small pupil. We call those «autonomic» as they are mediated by autonomic reflexes.
To read more, see the International Classification Website
TABLE : Key differences between these headaches:
| Cluster headache | Paroxysmal hemicrania | Hemicrania Continua | SUNCT | |
| Attack duration | 15-180 min | 2-30 min | Pain is continuous, with flare ups | 5-240 sec |
| Attack frequency | 1/ 2 days to 8/ days | 5-40/ day | Pain is continuous with flare ups | 3-200/ day |
| Periodicity | Seasonal Bouts and remissions | Episodic or chronic | Continuous | Episodic or chronic |
| Treatment | Verapamil, Oral steroids GON block Lithium | Indomethacin Occipital nerve blocks Gliacin | Indomethacin Occipital nerve blocks Glicain | Anticonvulsants Lamotrigine Topiramate Gabapentin |
What type of workup should I have if I present those symptoms?
Any patient who presents with these symptoms should see a neurologist. The usual workup will include an MRI. The MRI is expected to be normal. In very rare cases, a benign tumor of the pituitary gland can be linked with TACs. A blood workup could be considered by your physician. It is quite surprising to see such clear symptoms with normal imaging, but remember that migraine and cluster headache are common neurological diseases in which the MRI is also normal.
What is indomethacin and why does it help with these headaches?
Indomethacin is an anti-inflammatory. It has particular effects, including the decrease in the brain pressure and the constriction of blood vessels. At present time science cannot explain why it is so effective to treat certain headaches like paroxysmal hemicrania and hemicrania continua and be relatively ineffective for others like cluster headache and SUNCT.
How should I try the indomethacin?
Your physician will give you the exact instructions, as protocols might vary from one doctor to the other. Usually the dose will start lower (50 mg twice a day) and be increased (up to 200 or even 300 mg per day). Indomethacin is known to cause gastro-intestinal side effects like heart burn and diarrhea. If these side effects are tolerable, it’s best to keep going and at least see if there is a clear improvement that would confirm the diagnosis.
The most important thing to do for a good indomethacin trial is to hold a headache diary and keep count of the daily attacks at baseline and during the treatment. If the effect is 90%, the diary might not be needed, but as the dose increases, a more subtle effect can be observed with the diary.
What will happen to me on the long term if I have a diagnosis of PH or HC?
These headaches can last over decades but may also stop and go into remission. If indomethacin is tolerated, then it will become a long-term treatment, at the lowest dose possible. Sometimes the drug can be stopped for a while (drug holiday). If nerve blocks are helpful, they can be used to spare indomethacin.
If you do develop complications of indomethacin (gastric irritation or ulcers, high blood pressure), you may have to stop it and discuss other options with your physician.
REFERENCES
Burish MJ, Rozen TD. Trigeminal Autonomic Cephalalgias. Neurol Clin. 2019;37(4):847-69.
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I have tried all the medications are my hemicrania continua and the only thing that has worked for me or didn’t give me bad side effects is the botox. It is decreased my headaches and therefore lowered my Tylenol intake. My insurance will not pay for the botox for my HC. I’m wondering if the treatment is done in Canada or if I can buy the botox in Canada and have my doctor in Washington administer it
Disclaimer *** This message does NOT replace a medical opinion from a qualified health care provider after a full analysis of your case. These are generic comments that could not apply to your specific case.
Dear Cheryl,
there are many similar symptoms between hemicrania continua and chronic migraine. Sometimes, people with migraine can present autonomic symptoms. I do not want to question your diagnosis, but have you had a conversation with your physician about the possibility that you have a particular type of chronic migraine that looks like hemicrania continua (which, in theory, should respond exclusively to indomethacin, but of course it’s not 100% of people and this drug is also poorly tolerated)? This could allow a revision of diagnosis and coverage of Botox. Also, a recent article suggests a benefit of Botox for hemicrania continua (see below) so your physician could use this to justify the therapy.
Hopefully these ideas could lead to productive discussions with your physician to find a way to get the treatment you need.
Dr Leroux
J Headache Pain. 2015 Mar 5;16:19. doi: 10.1186/s10194-015-0502-z.
OnabotulinumtoxinA for hemicrania continua: open label experience in 9 patients.
Miller S1, Correia F2,3, Lagrata S4, Matharu MS5.
Author information
Abstract
BACKGROUND:
Hemicrania continua is a strictly unilateral, continuous headache, typically mild to moderate in severity, with severe exacerbations commonly accompanied by cranial autonomic features and migrainous symptoms. It is exquisitely responsive to Indomethacin. However, some patients cannot tolerate treatment, often due to gastrointestinal side effects. Therapeutic alternatives are limited and controlled evidence lacking.
METHODS:
We present our experience of nine patients treated with OnabotulinumtoxinA for hemicrania continua. All patients were injected using the PREEMPT (Phase 3 REsearch Evaluating Migraine Prophylaxis Therapy) protocol for migraine.
RESULTS:
Five of nine patients demonstrated a 50% or more reduction in moderate to severe headache days with OnabotulinumtoxinA with a median reduction in moderate to severe headache days of 80%. Patient estimate of response was 80% or more in five subjects. The median and mean duration of response in the five responders was 11 and 12 weeks (range 6-20 weeks). Improvements were also seen in headache-associated disability
CONCLUSIONS:
OnabotulinumtoxinA adds a potential option to the limited therapeutic alternatives available in hemicrania continua.
I have hemicrania continua, no doubt. My doctor had to put that code on my Medicare claim and they refused it because it is not being used for the HC. He is a headache doctor and cant change the code which does not coninside with the treatment of botox. . The Indomethacin and all the other drugs I have tried either dont work or make me woozy and have loss of balance. I also am inclined to gastritis with NSAIDS. The Botox treatment is working. He tried to give these explanations for the use of the Botox but we were told by Medicare that they dont preapprove any ailment so they never saw the letter he wrote them. Do you know if they do this treatment for HC in Canada?
Dear Cheryl, it seems you are well taken care of. This situation is rare and falls «between the cracks». It is a question of expert opinion, and at present time your physician is the best qualified to help. This being said, access to medications for rare conditions and particular situations is now on the radar for Migraine Canada. You are probably not the only one dealing with this. Hopefully this situation will be resolved fairly so you can get the treatments that work for you! Dr Leroux
Most of my migraines are one sided, however, my droopy eye is on the opposite side! I very often have the dripping eye and nostril but I’ve never really thought of which side those are on. I’m going to have to watch for that!