Need answers!

I am a 31yr old female and have severe migraine w/ aura.  I was DX with coarctation of aorta when I was 11 with no previous symptoms. I then had my conduit repair.  Five days later I had my first migraine.  In 1999, I had my balloon procedure, another in 2000, then finally repaired in 2001 by stents.  I still had debilitating headaches, but then became pregnant.  During pregnancy, I still had headaches, but no aura.  After delivery, it was a rainstorm of head pain.  Doc's assumed a spinal headache, went for a patch.  Headaches still horrible every since including very dilated pupils, total loss of sight, and an out of body feeling with numb limbs.  Im on beta blockers, calcium channel blockers, anti-siezure meds, antidepression meds to name a few.  My neurologist was thinking AVM's of the cerebellum, but it didn't show on the MRI.  What should I do now?

[Neuro Forum]

Thanks for using the forum. I am happy to address your questions, and my answer will be based on the information you provided here. Please make sure you recognize that this forum is for educational purposes only, and it does not substitute for a formal office visit with your doctor.

Without the ability to examine you and obtain a history, I can not tell you what the exact cause of your symptoms is. However I will try to provide you with some useful information.

It sounds like you have severe headaches despite multiple medications. In the medical literature, an association between severe migraines and coarctation has been reported.

There are several causes of headaches. Headaches can be divided into primary and secondary. Primary headache disorders are headaches without a direct cause. Secondary headache disorders are due to an underlying problem, such as a tumor, medication side effects, central nervous system infections, clots in the veins in the brain etc. In the presence of a normal MRI, a vascular malformation is exceedingly unlikely. Dilated pupils can occur with migraine.

One condition that comes to mind with severe chronic headaches is benign intracranial hypertension, also called pseudotumor cerebral, or idiopathic intracranial hypertension. It is a disorder characterized clinically by headaches and vision loss, and sometimes tinnitus (ringing in the ear that is synchronous with the pulse). Dizziness may also be a symptom. The headache is usually global (all over the head) and pressure-like; the headache presentation can be variable. BIH predominantly occurs in people who are overweight, women, and those taken specific medications such as retin-A (the acne medication), vitamin A, and tetracycline antibiotics.

It is diagnosed based on clinical findings and the finding of a high cerebrospinal fluid pressure on lumbar puncture. Other tests may include specific types of brain MRIs and an MRV. The treatment is usually with medications to decrease the amount of fluid around the brain, and these are mainly diuretics though other medications are also used. Patients who do not respond to medications or who have vision loss require procedures to relieve the cerebrospinal fluid pressure or at least the pressure around the nerves to the eye (to prevent vision loss). If a problem with the veins in the brain (cerebral venous stenosis is found), treatment for this may be indicated as well. Monitoring by an opthalmologist (eye doctor) for papilledema (swelling of the nerve head) is important as vision loss can occur.

The other condition that comes to mind with intractable migraines associated with systemic symptoms is called pheochromocytoma, which is a type of tumor that over-produces catecholamines, similar the chemical substance commonly known as amphetamine. This tumor is not usually cancerous, but can cause excretion of these hormones, leading to symptoms. This tumor is can be diagnosed with urine and blood tests. Please understand I am not suggesting you have this disorder but rather it is one possibility to consider and discuss with your physician.

Another potential cause of chronic headaches that would need to be ruled out is

If the migraines are deemed to be primary (meaning no known cause), then continued trials of different medications will be necessary. As I'm sure you know by now, in treating chronic headaches such as in yourself, the treatment should include two types of medications: preventative therapy and abortive therapy. Preventative therapy is a medication that would be taken every day regardless of whether or not a headache is prevent. This type of medication is used to prevent headaches from occurring, and there are several types including but not limited to beta blockers such as propranolol, calcium channel blockers such as verapamil, and others including topamax, depakote, elavil, etc. A lot of these medications were invented for other uses and are used not only for headache but also epilepsy and depression. They have proven very effective in preventing headaches. The second medication is abortive, meaning it is used when a headache is coming on. The medication used depends on the nature of a headache. If it is a migraine type headache, a group of medications called triptans can be used. And so on. However, with frequent use of abortive medications including triptans, tylenol, advil, and others, medication overuse headache occurs. This requires a specific treatment in which the over-used medications are slowly stopped and replaced with more long-acting medications. Tylenol with caffiene, and similar medications, can cause medication overuse headache if used too frequently. Lyrica, which is used for fibromyalgia, can be used for chronic headaches too.

A common cause of headaches in people with chronic headaches is called medication rebound or medication overuse headache: medications as simple as tylenol or advil and particularly narcotics if used too much can cause rebound headaches that are even worse then the headaches for which the medications were started for.

If you find that your symptoms are worse around menses, then hormonal therapy is sometimes useful. Also, some patients with chronic headaches benefit from magnesium supplementation.

It sounds like you have tried various medications, but continued trials of different combinations may still prove effective. If you have not yet been evaluated by a headache specialist (a neurologist specialized in headache), this may be beneficial to you. Sometimes, infusion therapy at a headache center may be helpful.

Thank you for this opportunity to answer your questions, I hope you find the information I have provided useful, good luck.