Hemiplegic migraines

[paula__0__0]

  Please,  I am interested in any information that you can give me on Hemiplegic Migraines.  I have a patient that was diagnosed with this diease and I cannot find much on it.  I have some information from the physican as on how to care for him, but I need something that I could use in helping to deal with the parents.  As you can imagine thier fear and concern when the doctor told them of the slim chance of their son having a stroke, and  the frequency of these "spells" are nothing to be overly concerned about and that they have to live with this and there is nothing they can do.  The patient has been having frequent "spells" at least q.i.d. lasting form anywhere from 15 minutes to 1hour.  He has some severe enough to have left sided paralysis and drooping of the mouth, and some as mild as just dozing in class.  He is still in school but with the frequency and longer duration it is becoming  difficult to keep to a normal routine as possible.  Please give me some information that I could pass on to the parents so they can adjust to their sons diagnosis.
Dear Paula:
I am sorry that your patient has Hemiplegic Migraine.  The best of the studies for hemiplegic migraine comes from an old study by Bradshaw and Parsons (1965) Q.J. Med 133:65-85.  In their series they found that 47% of the patients had a family history of migraine, and 18% has a family history of hemiplegic migraine (more recent stuff on this later).  Attacks of hemiplegic migraine are frequently precipated by minor head trauma (ice hockey is definitely a no-no).  They found that 23% of their patients had a change in consciousness from confusion to frank coma.  The hemiplegia onset may be abrupt and last for days or weeks and stimulate a stroke (again more on this later).  All of their patients had associated paresthesias and 88% had visual auras and 44% had speech problems associated with their migraines.  The headache can be generalized (29%), contralateral (47%), or epsilateral (22%) to the hemiparesis.  This is not a progressive disorder and is not associated with dystonia or retardation.  There is some new data that indicates that this order is genetic and inherited as an autosomal dominant mutation.  Studies by Joutel et al. in Am J Hum Genet (1994) 55:1166; May et al. in Hum Genet (1995) 96:604, and Ophoff et al in Genomics (1994) 22:21 have shown that hemiplegic migraine is clearly part of the migraine spectrum and not epilepsy.  Of the reported FHM (familial hemiplegic migraine, as it is called now), half have been assigned to chromosome 19p13 and four to 1q; some familes do not link to either locus.  Cerebellar atrophy and ataxia is found in half of the 19p13-linked familes but none of the unlinked.  Ophoff and colleagues reported missense mutations in the CAVNa1A gene at 19p13 encoding the alpha 1A subunit of the brain specific P/Q type voltage-dependent calcium channel.  
In the differential diagnois of hemiplegic migraine, one has to rule out epilepsy and MELAS (a mitochondrial disorder).  If the patient does have stroke-like events, and lactic acidosis one should think of MELAS.  However, it sounds like your patient has had these ruled out.  I hope that this helps you.  Let me know if you need any other information.
Sincerely,
CCF Neuro:Pediatrics MD, RPS