Familial Hemiplegic Migraine: Follow-up Findings of Diffusion-Weighted Magnetic Resonance Imaging (MRI), Perfusion-MRI and [99mTc] HMPAO-SPECT in a Patient with Prolonged Hemiplegic Aura

Abstract

Familial hemiplegic migraine (FHM) is a rare inherited autosomal dominant disorder. Migraine aura may last up to several weeks and then resolve without sequel. We report a 21-year-old male with FHM since the age of 3 years. Diffusion-weighted magnetic resonance imaging (DWI), perfusion-MR imaging (P-MRI) and [99mTc] hexamethyl-propyleneamine-oxime-single photon emission tomography (HMPAO-SPECT) were performed on day 2, when he was somnolent with right-sided hemiplegia, on day 9 when a mild hemiparesis was still present and on day 24 after recovery. The right central region showed normal findings in DWI, whereas P-MRI and SPECT revealed hyperperfusion on day 2, less marked on day 9, and normal findings on day 24. In conclusion, this case report indicates for the first time, by means of SPECT, P-MRI and DWI studies, that even extremely long-lasting migraine aura is not associated with cerebral ischaemia. Therefore, it supports the revised International Headache Society criteria where the term 'persistent' aura is proposed.

Acute-Onset Migrainous Aura Mimicking Acute Stroke: MR Perfusion Imaging Features

“…16 Some published studies report hyperperfusion; however, this is during the headache stage, 6 -24 hours after the onset of symptoms. 2,17,[19][20][21]24 Others report hypoperfusion during the migrainous aura. 2,14,18,22,23,27 These findings emphasize that early MR imaging examination is crucial to detect the perfusion abnormalities.…”

“…12,13 MR imaging, nuclear medicine, and CT perfusion studies have demonstrated that migrainous aura may be associated with perfusion abnormalities; however, all articles are limited to a small number of selected patients. [14][15][16][17][18][19][20][21][22][23][24] To date, no data are available from a larger series of consecutive patients.…”

Migraine Associated Cerebral Hyperperfusion with Arterial Spin-Labeled MR Imaging

“…The vascular theory proposes that the migraine aura is associated with vasoconstriction and the subsequent headache is associated with vasodilation and hyperperfusion. 3,4 The second theory is that a contiguous wave of neuronal depression spreads through the cortex causing the aura symptoms. [5][6][7][8][9][10] The vascular changes have been evaluated with nuclear medicine studies including single-photon emission CT and positron-emission tomography as well as blood oxygen level-dependent functional MR imaging and T2* dynamic susceptibility contrast (DSC) MR perfusion imaging.…”

“…3,8,9,[11][12][13] These methods are limited in the evaluation of the patient with migraine because of the dynamic nature of the perfusion abnormalities.…”

“…[5][6][7][8][9][10] The vascular changes have been evaluated with nuclear medicine studies including single-photon emission CT and positron-emission tomography as well as blood oxygen level-dependent functional MR imaging and T2* dynamic susceptibility contrast (DSC) MR perfusion imaging. 3,8,9,[11][12][13] These methods are limited in the evaluation of the patient with migraine because of the dynamic nature of the perfusion abnormalities.…”