Proper diagnosis and treatment requires that physicians and patients understand the symptoms and clinical presentation of this complicated migraine condition.
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
"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."
–Gary W. Jay, MD, FAAPM, DAAPM
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.