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... Show moreOBJECTIVE 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]). CONCLUSION 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. Show less
Stam, A.H.; Louter, M.A.; Haan, J.; Vries, B. de; Maagdenberg, A.M.J.M. van den; Frants, R.R.; ... ; Terwindt, G.M. 2011
