Making Sense of Complex Confusional Migraine
Complex Confusional Migraine (aka Acute Confusional Migraine - ACM) is rare type of migraine that is said to occur roughly between 5-10% of pediatric population (occasionally in teenagers as well). It is considered a variant of migraine with auras. They typically occur in children who already have diagnosis of migraines. This variant usually has a distinct trigger mainly of prior head injury (even small bump in the head) in at least ½ of those diagnosed. Those with family history of migraine are highest at risk.
Why is it given this name?
The reason this terminology is used is because of its unclear underlying pathophysiology. Hence, this is a diagnosis of exclusion due to the many similarities between migraines and seizures both of which are episodic and have an aura. Also both can exhibit headaches and have confusion during, before, and after an attack and exhibit sleepiness as well. Furthermore, both conditions are extremely common in children. Thus it is extremely important to rule out a seizure as well as other neurologic and medical conditions that can mimic these symptoms (e.g. strokes and low blood sugars). Before diagnosis is given an MRI of the brain, blood work, as well as an EEG need to be done as part of work up.
What are the symptoms?
The headache is distinguished by its pronounced state of mental confusion thus the name. The confusion can last anywhere from 10 minutes to 20 hours usually ending in deep sleep. Average episode last about 5 hours. Occasionally these can occur without a headache (acephalgic migraine) making the diagnosis much more confusing especially in absence of good history. Along with the confusion there can be problems in speech such as slurring along with agitation, anxiety (a common pediatric presentation of migraines) and even amnesia.
What is prognosis and treatment?
The evolution of this type of headaches into adulthood is not well understood. However, in the majority of cases a single attack is the norm while multiple attacks are extremely rare. Since the chance of recurrence is very low most ACM are treated as a migraine disorder. Only 1 in 4 will have more than one episode.
- Keep diary if more than one episode occurs as to onset, duration, and triggers.
- Have a 24 hour EEG if persistent episodes of ACM occur
- During an acute attack especially if loss of consciousness occurs go to ER or call 911; otherwise treat as typical migraine with triptans, OTC pain killers, and other ergotamine’s such as DHE (nasal or injectable-IV).
- Anti-seizure medicines such as IV Valproic acid (Depakote) have been shown to improve acute episodes; but other anti-seizure medicines (e.g. Keppra, and Topamax) may also be used as well. these may also serve as prophylaxis to prevent episodes. Other preventive medications are similar to those used in typical migraines (e.g. calcium channel, beta blockers and tricyclic medications).
- Have your doctor write a prescription to keep in school at nurse’s station to be used in case of an episode.
- If child has several episodes causing falls and injuries due to falls – consider wearing a helmet to prevent further head injuries until symptoms are under control.
- During an acute attack high flow oxygen can also be used – so have an oxygen tank available if have multiple ACM episodes.
- IV prochlorperazine (Compazine) which also comes in suppositories that can be used at home or in school during an attack.
So although this sounds like an ominous diagnosis chances are that after the first scare you won't have to deal with again. Just like in seizures everyone is entitled to one without much repercussions.
How much has your migraine disease changed or evolved over time?