A long-term follow-up study of 18 patients with sporadic hemiplegic migraine.
OBJECTIVE
Our objective was to study the long-term prognosis of sporadic hemiplegic migraine (SHM).


METHODS
We performed a longitudinal follow-up study in 18 patients who were diagnosed with SHM between 1993 and 1996. Follow-up time between the first and second survey ranged from nine to 14 years. These patients were included as part of a genetic study in which we systematically analysed the role of the three known familial hemiplegic migraine (FHM) genes.


RESULTS
In 12 out of 18 patients the clinical diagnosis was unchanged. In two of the six remaining patients the attacks were no longer associated with hemiplegia; one of them had an ATP1A2 gene mutation (E120A). In the four other patients, the diagnosis changed into FHM, because a family member had developed hemiplegic migraine since the initial diagnosis was made. In two of the four patients a mutation was demonstrated (CACNA1A [R583Q] and ATP1A2 [R834X]).


CONCLUSIONS
This study shows that the diagnosis of SHM changes into FHM in a considerable percentage of patients (22% [4 of 18]), almost a decade after the initial diagnosis. This indicates that a careful follow-up of SHM patients and their families is advisable for optimal care and counseling. Diagnostic screening of FHM genes in SHM patients can be of value. Our genetic and clinical follow-up studies reinforce the evidence that FHM and SHM are part of the same spectrum of migraine.
Leiden University Medical Centre, The Netherlands.
Anine H
AH
Stam
Mark A
MA
Louter
Joost
J
Haan
Boukje
B
de Vries
Arn M J M
AM
van den Maagdenberg
Rune R
RR
Frants
Michel D
MD
Ferrari
Gisela M
GM
Terwindt
D000293:Adolescent; D000328:Adult; D015220:Calcium Channels / Q000235:genetics; D002648:Child; D018450:Disease Progression; D005192:Family Health; D005260:Female; D005500:Follow-Up Studies; D005820:Genetic Testing; D006429:Hemiplegia / Q000175:diagnosis / Q000209:etiology / Q000235:genetics; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D008881:Migraine Disorders / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D010375:Pedigree; D011379:Prognosis; D000254:Sodium-Potassium-Exchanging ATPase / Q000235:genetics; D055815:Young Adult; D020864:Calcium Channels, N-Type
D015220:Calcium Channels; D000254:Sodium-Potassium-Exchanging ATPase; C105656:CACNA1A protein, human; D020864:Calcium Channels, N-Type; C498531:ATP1A2 protein, human