1.2.4 Familial hemiplegic migraine (FHM)

Migraine with aura including motor weakness and at least one first- or second-degree relative has migraine aura including motor weakness.

B. Aura consisting of fully reversible motor weakness and at least one of the following:

1. fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision)

2. fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness)

1. at least one aura symptom develops gradually over 5 minutes and/or different aura symptoms occur in succession over 5 minutes

3. headache fulfilling criteria B-D for 1.1 Migraine without aura begins during the aura or follows onset of aura within 60 minutes

D. At least one first- or second-degree relative has had attacks fulfilling these criteria A-E

Case report

"I have been trying to figure out what areas are involved in my migraines. They have often had a very obvious right visual field upper/mid quadrant visual aura, so I have assumed that the origin is left temporal lobe. I usually get numbness of the left side of my face, and weakness on the left side of my tounge. On a few severe occassions I had hemiparesis and was unresponsive to reflex testing on my left arm and leg. I'm a neuroscientist and am frustrated that I don't know what is going on in my brain. What I find strangest is that I will often have an aphasia in which I can form words in my brain, can write or use sign language fluently, but cannot speak without EXTREME effort, and then only haltingly [i.e. dysphasic speech disturbance in familiar hemiplegic migraine]. This doesn't seem to match the Broca's or Wernicke's aphasias; is it possible that the language area itself isn't affected, but only its connection to verbal motor regions??? On a personal note, it has caused bad feelings when I don't respond to people, but my family now knows that if I stand there bobbing my head with my mouth shut, something is probably going on. - In fact, my migraines are probably familial hemiplegic, as my mom has very similar symptoms, including confusion, facial numbness and left side hemiplegia. She does not, however, appear to have speech or language disturbances."

(Amber Buhler, Email to Klaus Podoll, July 26, 2005)

References

International Headache Society. The International Classification of Headache Disorders, 2nd edition. Cephalalgia 2004; 24 (suppl. 1): 1-160.