Gary W. Jay, MD, FAAPM, DAAPM
The first issue to note when discussing this topic is nosological. That is, the term “complicated migraine” is not typically used by headache specialists, but it may be used by other physicians or by patients. The terms “complex migraine” or “atypical migraine” are now used in its stead. The National Headache Foundation (www. headaches.org) describes complicated/complex migraine as those migraine headaches associated with extended or exaggerated visual migrainous auras. Complex migraine has also been defined as referring to attacks of prolonged aura symptoms that could last hours to days, or situations in which patients develop ophthalmoplegic migraine, which is associated with partial paralysis of the cranial nerves used to move the eye. Still another definition equates a cerebral infarction with complex migraine. Given the existence of multiple definitions describing a range of clinical characteristics, it should be obvious that there is not a real consensus regarding this term. The reality is that atypical or complex migraine comes in several varieties, several major forms of which we’ll discuss in this article.
Soon after I started practice as a headache and pain specialist, I had a 24-year-old female patient with migraine with visual aura who smoked and took estrogenic birth control pills (BCP). She stroked out the upper-left quadrant of her visual fields. This experience changed the way I treated migraine with aura. Even though some headache specialists disagree, I would not treat a migraine-with-aura patient who smoked and took BCPs. Some physicians didn’t think the combination would create problems. I felt differently.
Basically, after an aura lasting 5 to 60 minutes or so, during which time a neurological aura begins, a severe headache starts.
There are several other types of what may be called migraine with complex/complicated aura:
• In children there are different types of migraine that are not necessarily “complicated migraine”, but what I would term “migraine equivalents.” These include abdominal migraine, characterized by bouts of pain in the abdomen lasting for hours, ending after the child falls asleep. It can be associated with nausea, occasional vomiting, flushing, and pallor. Triptans may help treat this condition if administered early enough.
• Vertigo is another migraine equivalent in children, although in my experience abdominal migraine is seen more frequently.
• Other forms of complex migraine include, as noted above, ophthalmoplegic migraine. In this disorder, patients may experience recurrent headache with migrainous characteristics, pain around the eye that can last for weeks or even months, associated with paresis (weakness) of one or more ocular cranial nerves, most commonly the third cranial nerve. There are no associated changes in MRI other than in the affected nerve. Some feel that this may be a recurrent demyelinating neuropathy rather than a variant of migraine.1
Familial hemiplegic migraine (FHM) is described as migraine with aura including motor weakness. For a diagnosis of FHM, the patient must have at least one first- or second-degree relative who has migraine with aura including motor weakness. FHM is a genetic problem; researchers have identified specific gene abnormalities associated with this condition, including mutations in the CACNA1A gene, the FHMI gene that encodes the pore-forming α 1A subunit of P/Q-type voltage-dependent neuronal calcium channels.2 A diagnosis of familial hemiplegic migraine requires that the patient has experienced at least two attacks that fulfill criteria 1 and 2:3
1. Aura consisting of fully reversible motor weakness and at least one of the following:
• Fully reversible visual symptoms, including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision)
• Fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness)
• Fully reversible dysphasic speech disturbance
2. And at least two of the following:
• At least one aura symptom develops gradually over ≥ 5 minutes and/or different aura symptoms occur in succession over ≥ 5 minutes
• Each aura symptoms lasts ≥ 5 minutes and < 24 hours.
• Headache lasting 4-72 hours that has two or more of the following characteristics: unilateral location, pulsating quality, moderate to severe pain intensity, and/or aggravation by or causing avoidance of routine physical activity. During the headache attack, the patient must experience at least one of the following symptoms: nausea and/or vomiting, photophobia and phonophobia. Headache attacks begin during the aura or follow the onset of aura within 60 minutes.
As mentioned, a diagnosis of FHM requires that the patient have at least one first-or second-degree relative who has also had attacks fulfilling these criteria. Lastly, a diagnosis requires that these symptoms not attributed to another disorder. Since the disorder was first described in 1920 by Clark,4 there have been more than 150 families reported in the literature. There is also a sporadic form of this migraine disorder known as sporadic hemiplegic migraine (SHM),5 which shares similar diagnostic criteria with FHM. With SHM, the patient must have no first- or second-degree relative who has migraine with aura including motor weakness.
Complex/atypical migraine can be very frightening to the patients who experience it.
When a patient has a first episode of a hemiplegic migraine, even if there may be a family history of the same, but especially if family history is not knowna full neurological evaluation should be done to rule out a cerebral vascular accident (CVA). Note that the FHM diagnostic criteria indicate a gradual onset of symptoms, while during a CVA or transient ischemic attack, the symptoms are sudden.
Another form of complex migraine, basilar-type migraine (also known as “Bickerstaff Migraine”), appears to go beyond just basilar artery involvement to include involvement of the posterior fossa. Echoing the prevalence seen across migraine types, basilar-type migraine occurs most often in young women. Findings suggest transient focal reduction of cerebral blood flow during the aura phase of this entity, but it has not been entirely determined if the vascular changes are the cause or the consequence of neuronal dysfunction. The diagnostic criteria for basilar-type migraine include:3
A. At least two attacks fulfilling criteria B through E
B. Fully reversible visual, and/or sensory, and/or speech aura but no motor weakness
C. Two or more fully reversible aura symptoms of the following types:
d. Decreased hearing (hypacusia)
e. Double vision (diplopia)
g. Decreased level of consciousness
h. Simultaneous bilateral visual symptoms in both the temporal and nasal field of both eyes
i. Simultaneous bilateral paresthesias
D. At least one of the following:
a. At least one aura symptom develops gradually over ≥5 minutes and/or different aura symptoms occur in succession over ≥5 minutes
b. Each aura symptom lasts ≥5 and ≤60 minutes
E. Headache lasting 4-72 hours that has two or more of the following characteristics: unilateral location, pulsating quality, moderate to severe pain intensity, and/or aggravation by or causing avoidance of routine physical activity. During the headache attack, the patient must experience at least one of the following symptoms: nausea and/or vomiting, photophobia and phonophobia. Headache attacks begin during the aura or follow aura within 60 minutes.
F. Not attributed to another disorder.
Basilar-type migraine is the only headache entity associated with syncope. Finally, to mention two other complex forms of migraine, there is indeed migrainous infarction as well as migraine-triggered epilepsy (or seizure) and retinal migraine, but space prohibits a more thorough discussion.
Prevention and treatment
One issue with the treatment of atypical forms of migraine is that some clinicians think that the best approach is to try to stop the migraine using a triptan, which involves medication-induced vasoconstriction. Because we know that migraine (and most probably the more complex or atypical forms of migraine) is neurological in nature with secondary vascular involvement, the use of a vasoconstrictor in a complicated disorder that manifests with neurological symptoms (hemiplegia, ataxia, ophthalmoplegia, etc) would not, in my opinion be appropriate. This treatment approach may indeed make a transient neurological problem more permanent.
Therefore, prophylactic treatment makes more sense. For this, patient education is mandatory. Patients should avoid their individual migraine triggers, including dietary factors, lack of sleep, bright lights, etc. Dietary management is important; patients should avoid chocolate, aged, sharp cheeses, red wine, walnuts, nitrates, nitrites, and other known or suspected triggers. In fact, there are more than a hundred foods/spices that may affect different individuals with migraine.
I would recommend avoidance of estrogenic birth control pills and hormone replacement therapy because they may trigger migraine in some patients. I have my patients learn relaxation techniques including biofeedback training. Meditation is also good. Some patients have reported benefits from practicing yoga.
What should patients do when a complex or atypical migraine attack occurs? This a major treatment-related issue. I do not typically use triptans or ergots secondary to vasoconstriction. Dodick, in the Mayo Clinic News, stated that triptans and ergots are frequently contraindicated in the patient with complex migraine.6 Some clinicians are of the opinion that if a patient catches the atypical migraine early enough, then a triptan can be used. My concern is that the patient may think they are early enough but in fact are not, and the activity of the triptan may worsen the problem. Nonetheless, these mediactions are often used in this patient population, as it is certainly better to stop (or try to stop) such a frightening problem as a hemiplegia or ataxia. However, if not used on time or correctly, making the problem worse should not be an option.
If patients are capable of early recognition of the signs of migraine, they may institute treatment—for example, rest in a cool, dark room. The use of a benzodiazepine to help “sleep off attacks” may be helpful in select patients. The use of pain medications such as aspirin, ibuprofen, naproxen, or acetaminophen may be initially helpful. Metoclopramide, domperidone, or prochlorperazine may alleviate migraine-associated nausea and vomiting. Opioids are not frequently for complex migraine, to prevent issues of overuse or rebound headache.
The real key is prophylaxis, which should include medications taken daily such as beta-blockers (eg, propranolol, metoprolol); amitriptyline (start with a low dose and increase slowly at night); and antiepileptics including valproate, topiramate (labeled for migraine prophylaxis), gabapentin, and others. Other medications that may be helpful include pizotifen and clonidine.
The bottom line is that complex/atypical migraine can be very frightening to the patients who experience it. It is imperative to teach them about this form of headache, and explain the positives and negatives of the use of various medications, so that they understand and can make informed choices about their care. The best treatment for difficult headache conditions is for the patient and the physician to work together to devise an individualized treatment plan that maximizes symptom relief and minimizes side effects.