Familial Hemiplegic Migraines

Pediatric Familial Hemiplegic Migraines

by Eleanore
(Denver, CO)

Boy With Migraine

I am the mother of a 3 1/2yr old boy who we believe is suffering from what we believe is FHM(familial hemiplegic migraine).

He was initially diagnosed with epilepsy at 14mos. It was found this past summer after he spent 3 days in and out of a coma that he does not in fact have epilepsy. It is now believed that he has FHM or Alternating Hemeplegia of Childhood.

I am having a really hard time finding someone to treat him. We've managed to reduce the number and severity of episodes by decreasing calcium in his diet but it's not enough. We are searching for a FHM Specialist. At this point we will travel anywhere in the country, I just need to find someone to help my little boy.

Eleanore

Answer:

Hi Eleanore,

I understand your frustration, especially as a parent.

I am glad your son is having less attacks. Calcium may play a role both in vascular tissue and in nerve conduction when it comes to the pathology of migraines.

Familial hemiplegic migraine as you know is a childhood migraine variant which is accompanied by fluctuating weakness that can last from minutes to hours. In children, the headache may not be as prominent as the weakness, nausea and vomiting.

I spoke to the neurologists that I work with and we all agree with what is currently accepted in the treatment of this condition:

At the present time there are two ways to treat for children:

Acute treatment: Anti-nausea drugs (reglan), mild sedatives, and pain relievers are generally safe (non-narcotic). Triptans, ergots and vasocontrictors of any type should not be used to abort familial hemiplegic migraine.

Prevention: There are several possibilities here. You did not mention what your son is taking so I will list each of them.

Low dose Elavil is quite good and dose is adjusted to his weight as are all medications. Low dose anti-convulsants that are used in regular migraine prophylaxsis and finally low dose calcium channel blockers are good. Calcium channel blockers are generally the drug of choice in this condition. There is a drug called Flunarizine, which is one of the most effective drugs for true alternating hemiplegia of childhood. It is a calcium channel blocker but unfortunately it is not available in the United States.

Alternative headache treatments included riboflavin and feverfew and magnesium supplementation. Watch the dosing on magnesium as its side effect can be diarrhea.

What you really need is a pediatric neurologist who is willing to work with you on dosing for your son. Many times we see that children tend to respond faster and best on alternative therapies first. This may be due to the fact that their brains are still "plastic" or growing. but there is no migraine research yet to support this. His brain will be finished growing when he is about 20 years old. He has a higher chance of growing out of this than girls do so don't give up hope!

Here is an excellent article on childhood migraine variants that you can discuss with your doctor.

I wish you the best of luck!
Mary Kay Betz MS RPA-C

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Familial hemiplegic migraines

by Vicki
(Lashmeet, West Virginia)

Hemiplegic Migraine

I am a 40 year old female trying to find a new migraine specialist to treat my chronic daily headaches and familial hemiplegic migraines. There are no specialists in the area where I live so I know I will have to travel but I do not know if I would benefit from a place where I am likely to be admitted to an inpatient program or if a practice like yours is what I need. I am a complicated case because I have low blood pressure, orthostatic syncope and hemiplegic migraines so triptans, ergots, beta blockers and calcium channel blockers are all contraindicated. I am basically limited to preventive and rescue medications, no abortive medications.

I am very much aware of my triggers: weather changes, insomnia, stress release and hormone fluctuations. I changed my diet years ago to remove food triggers. In order to help avoid rebound headaches, I do not take pain meds more than 9 days a month. I have made major changes in my life to try and make it less stressful.

I had my first migraine with aura when I was 23 and continued to have 1 to 2 a month for the next 10 years. The headaches stopped in June of 2002 and I did not have another one until January 2004 following a miscarriage.

On September 10 2006, I was 4 months postpartum, and when I awoke that morning I noticed weakness and heaviness on the left side and slurred speech. I was taken to the ER by ambulance thinking I had had a stroke. As soon as they laid me flat for the CT, the pain hit! I then knew I was having a migraine. Until the pain hit, migraine never crossed my mind because I didn't have my usual vision disturbances, photophobia, phonophobia and nausea. The pain from this migraine was worse than any one I had ever had before and it lasted for 3 days. From that day on I had a headache everyday for 7 months, not a migraine but the pain was bad enough.

I was referred to a headache specialist/neurologist who was the first person to tell me about Familial Hemiplegic migraine. I did not get an appointment with him until the end of Jan. 2007 and had been having 3-4 of these migraines a month and I had moved from Va Beach to Southeast West Virginia. I was put on Topamax as a preventive and Stadol NS as a rescue ( this is the only pain med that works for me). The Topamax took away the daily headaches and had me down to 2-3 migraines a month once I reached 200mg daily. Because of this, I gladly did the 12 hour round trip to see the doctor. In January 2008 my care was turned over to my primary care physician when the specialist stopped seeing patients and went into research full-time.

Now, over a year later, my doctor is referring me out because my daily chronic headaches are starting to return and I am having 3-4 migraines a month.

I am trying to figure out where I need to go next. Anything you can suggest will be greatly appreciated.

Answer:

Hi Vicki:

You are right..you do have a complex history, but I have seen and treated far worse, so don't get discouraged.

First of all, make sure you are doing everything you can in regards to lifestyle changes that will improve your headache profile. Patients who improve are the ones who actively participate in their own care. I suspect you already are doing most of this..go to bed and get up same time every day (even weekends!) for six months. Don't skip meals..no smoking! Start yourself on vitamin therapy and after 3 months, increase the magnesium to a total of 800 mg per day.

Aerobic exercise three times a week raises serotonin in the brain and over time reduces headache severity and pain.

Severe headaches and chronic daily headaches need a specialist as your PMD says..so I found a few in your area (had to look you up on a map!) Here they are:

Kentucky:
Tarvez Tucker, MD (neurologist)
740 S. Limestone St.
Lexington, KY
859-323-6702

Ohio:
Claude Hobeika, MD (neurologist)
6527 Colerain Ave,
Cinncinnati, OH
513-385-5000

Virginia:
Lawrence Varner, MD (primary care)
400 Lexington Ave
Farmville, VA
804-392-6101

Stuart Stark, MD (neurologist)
Neuro & Headache Treatment ctr
4660 Kenmore Ave
Alexandria, VA
703-212-0700

I would actually recommend if you can travel that you consider Dr. Stark as he is at a headache center. They may be able to offer inpatient treatment or at the very least, a series of IV infusions to break your headaches.

Finally...the orthostatic syncope sounds suspicious for neurocardiogenic syncope which is seen in women with migraine. If you have not had a tilt table test done, ask for one. There is medication for this one!

Most of our patients are on multiple medications at a time, and the vitamins and two or three things at the onset of the headache. If you come to Buffalo, you are more than welcome to see us at Dent..716-250-2000.

Good luck!
Mary Kay Betz MS RPA-C

COMMENTS:

Since I have family members and friends with CADASIL, Vicki's story sounds all too familiar to me. Familial hemiplegic migraines have been linked to Chomosome 19, the same chromosome as CADASIL. CADASIL is not as rare as it was thought to be when it was first discovered. It's prevalence is unknown because it's been misdiagnosed or underdiagnosed in the past. I hope that Vicki is informed of CADASIL. More information on CADASIL can be obtained at www.cadasilfoundation.org

Answer

Thank you! You have a valid point, although I must say that in almost 10 years of seeing migraine all day long, I have only seen 2 cases of CADASIL.

I have posted your comment with a live link to the foundation so others can read more about this condition.

Mary Kay

So much of this story by vicki sounds very similar to a genetic disorder called CADASIL. One of the predominate symptoms is migraines. 85% of those with CADASIL suffer strokes. This might be something for Vicki to look in to.

Seizures do exist with hemiplegic migraines. I was recently diagnosed with having Hempiplegic Migraines, shortly after my attack I started having seizure like symptoms. I was told by the doctors that they were not seizures because I could remember some of the event occurring. I am recently learning that seizures can happen and you know somewhat that they are happening. No doctor has performed an EEG or anything. I am also at the age where I know my hormones are causing the attacks but still the doctors seem not to be listening. What can I do?

Answer:

Hi,
I will assume you are female because of the comment about hormones.

Partial seizures do happen and with a form of them (absence seizure) the patient is aware of surroundings. Absence seizures, however, usually occur in children and adolescents. If suspected, I usually order a 24 hour EEG or even a video monitored 24 hour EEG. That requires a more specialized practice that has an overnight sleep lab and video monitoring. Some hospitals also have this.

There is about a 5% overlap between seizure and migraine. In other words, 5% of seizure patients have migraine and vice versa.

You do not say where you are, but I would get to a board certified headache specialist who can sort things out for you.

Good luck!
Mary Kay

Here is a link to more information about the genetics of Familial Hemiplegic Migraine that was prepared by our genetic counselor and which has links to some useful resource for those dealing with this condition: Genetics of Familial Hemiplegic Migraine. There is also a number listed for anyone who wants to speak to a genetic counselor by phone. I hope it helps. Thanks, AccessDNA

Answer
Hi,
Thanks so much! I have post the link here and will add it to my resources page..

Mary Kay
Thank you so much for the fast and helpful response.

I did have a tilt table test when I was 29 and the official diagnosis was Neurally Mediated Hypotensive Orthostatic Syncope. I am on a high salt diet and take Proamatine for this but it causes me to stay awake for 24 hours after just 1 dose so I take it very sparingly.

I do ride my exercise bike 5 times a week and eat regular meals but sleep is the hard one. I have had insomnia since about 6 months before the migraines started in 1992. I have tried Elavil, Trazadone, Ambien, Roserem and Xanax in the past and Xanax is the only one that did much good. These days, I consider it a good night if I get 4 hours of sleep a night.

After reading about herbs and vitamins on your web site, I started thinking about the time period when my migraines stopped. During that time I was undergoing fertility treatments or pregnant and taking prenatal vitamins and extra folic acid and I wonder if that had anything to do with the pain stopping? I am going to talk to my Dr. at my next appointment to make sure there are no interference issues with my daily meds and then start the vitamins you recommended.

Lastly, can you give me an idea of what to expect if I traveled to the Dent Clinic? Based on what you know of my history, should I expect to need to be in town more than 1 day, are there tests I could have done here to expedite things, how frequently will I need to come back for follow-ups, will I be seeing you or someone else? I know you will be guessing on some of this and that is ok.

Thank you so much for all of the information and hope you provide.

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Familial hemiplegic migraines

by Tim
(Hudson, Florida, USA)

I'm 50 years old and for the past 4 years I have suffered with these symptoms and have found through talking with my family that it is a family inherent condition that my immediate blood line have had as well.

I have been being treated through the veterans administration for the past four years. They ran extensive test on me from EEG's, ct scans, echo cardiogram, and some I can't remember I do know that in the EEG stress test they invoked a seizure and measured weakness on my right side of my body as that happens every time I have one. Something on my right side of my body goes weak with tingling feeling, I also go to a state of unconscious for a brief time and stop breathing during these episodes as far as I have been told. Any ways, I also have a disorientation and two years ago I had one that wiped out nearly twenty years of my memory.

I have been tested also by the psychology department and measured with a degree of Dementia equal to a person in later years this was after the episode that I lost so much memory. So as of this last week they told me to use more Alprazolam when I feel I will be having one of these attacks. For them however as recent I saw a so called migraine doctor there whom explained to me that my seizures that accompany my symptoms is just anxiety and that is the latest findings of the medical field. They tried to give me two different Triptan medications both had very bad effects on me where I felt I was going to die.

They do have me on Divalproex 250 mg twice a day that helps reduce the number of attacks I have but it doesn't stop them. I have all the typical symptoms that are associated to this rare and definitive illness but they refuse to understand it nor treat it in the right manner. I know this is a sore subject among the medical field however the only thing I have found that helps my migraines and brings them to bay is Oxycodone, I have gone to pain management and they refuse to treat with this drug as well.

So I guess my question now is what now? How do I get the proper treatment for this so I don't have to keep suffering everyday with this. One note also is that I was diagnosed with PTSD from the military and this is what haunts me in the VA because to have this is to have anxiety attacks and this seems to be the theme and the treatment they lean towards for everything. I do know the difference between them, and it took nearly 20 plus years before they finally released my records so I would know that I even suffered with it, but before I even had the records as soon as I was finally excepted into the VA they diagnosed me with the PTSD right off the bat, so that's so you can maybe understand all that I have dealt with through the VA.

I just would like to find someone that has a good handle on this condition and can treat it correctly and not talk to me like I'm an idiot about what is happening to me and my body I live in here and I do know what go's on and I do read and search out information about it however I just don't seem to be able to get anyone else to understand what these is all about. Thank you for your time and any help you can give.

Tim

Answer

Hi Tim,
Well first of all you are not an idiot. You have a couple of conditions which are intimately intertwined from the neurological point of view. Any type of migraine whether an everyday sort of migraine or familial hemiplegic has an 85% of the time association with anxiety. Unfortunately, a condition such as PTSD can worsen the anxiety, and when anxiety flares, so do the migraines. Conversely, when you are having a full blown migraine, your anxiety will get worse.

So the answer here is to treat both conditions at the same time and realize that they are both part of the larger picture...chronic illness.

Divalproex (Depakote) is a good choice for you but from my professional point of view you are under-dosed. While we start people at 250 mg per day, generally I taper them up to 750mg or 1000mg over the course of 4-6 weeks and then leave the dose alone for 2-3 months. Most of my patients are between 500mg and 1500mg per day for treatment of their migraines. Based on what you have said, I think the dose could go higher. Main side effect is sedation and because of this at increasing doses you might ask them to switch you to the extended release version (depakote ER) which you can take at night. This is generic now so the VA should be able to get it.

Another benefit is that at a slightly higher dose, this anti-seizure medication can have a beneficial effect on mood, so you might get some benefit there.

Don't underestimate lifestyle changes when it comes to headache treatment. You must give any changes a full 90 days to have an affect on the migraines.

Finally, if you cannot tolerate the triptans (and yes they can provoke anxiety and panic) ask for Migranol nasal spray which doesn't cause this reaction. Generally the fast acting triptans, Imitrex and Maxalt cause this problem. The slower ones, Amerge and Axert...not so much. But Migranol is still a good option. You can combine either a triptan or Migranol with naprosyn at the onset of the migraine which is better than taking either alone.

Best of luck..
Mary Kay Betz MS RPA-C

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Hemiplegic Migraine and Numbness

by carol k
(scottish borders)

I am female, aged 60,diagnosed with hemiplegic migraine 4 years ago. I take amitryptiline to block attacks but last attack 2 years ago seems to have left me with permanent numbing around face and some days left leg.

All the doctors i have asked say this is NOT permanent, but today its bad and its 2 years now. can you advise??? do these attacks lessen with age?

thank you

Carol K.

Answer:

Hi Carol,

Generally speaking, migraines do lessen with age. Unfortunately, hemiplegic migraines are associated with numbness. This can even be worse than the migraine itself. It can be part of the migraine headache, but is also a sign of anxiety, which goes hand in hand with migraine.

Talk to your doctors about a small dose of an anti-seizure medication to take in addition to amitryptiline. This is our most common combination at our clinic.

Good luck
Mary Kay Betz MS RPA-C

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