Partially Reversible Cortical Metabolic Dysfunction in Familial Hemiplegic Migraine With Prolonged Aura

Abstract

We report a SPECT and PET voxel-based analysis of cerebral blood flow and metabolic rate for glucose in a 23-year-old woman with familial hemiplegic migraine (FHM) caused by ATP1A2 gene mutation. In comparison with healthy subjects, a PET scan showed brain glucose hypometabolism, controlaterally to the hemiplegia, in the perisylvian area early in the attack (Day 1), without any SPECT perfusion abnormalities. Decrease in metabolic rate was only partially reversible at Day 78, concordant at this time with a remaining hemisensory loss. These findings provide further evidence for a primary cortical metabolic dysfunction in FHM.

Neurovascular changes in prolonged migraine aura in FHM with a novel ATP1A2 gene mutation

“…There are only a few reports on CBF in genetically determined FHM 13 15. We confirmed unilateral hyperperfusion in the affected hemisphere corresponding to prolonged aura symptoms in repeated attacks of HMPA.…”

Prolonged Migraine Stuttering Aura: Structural, Functional, and Video Neuroimaging Study of an Atypical Migraine Aura. A Case Report

“…However, unlike functional neuroimaging studies in which brain perfusion is evaluated, performing a PET to analyze glucose consumption during a migraine aura is exceptional. Only 7 cases have been reported in the entire literature (Table ), 4 patients with persistent auras and 3 patients suffering from familial hemiplegic migraine (FHM) . In the first group, significant changes were found in just 1 patient with persistent aura who exhibited a left occipital hypometabolism contralateral to aura symptoms .…”

“…Only 7 cases have been reported in the entire literature (Table 1), 4 patients with persistent auras 12-14 and 3 patients suffering from familial hemiplegic migraine (FHM). [15][16][17] In the first group, significant changes were found in just 1 patient with persistent aura who exhibited a left occipital hypometabolism contralateral to aura symptoms. 12 FDG-PET studies performed in FHM patients [15][16][17] revealed reduced glucose uptake at different brain areas in all of them, with bilateral occipital hypometabolism in 2 subjects.…”

“…[15][16][17] In the first group, significant changes were found in just 1 patient with persistent aura who exhibited a left occipital hypometabolism contralateral to aura symptoms. 12 FDG-PET studies performed in FHM patients [15][16][17] revealed reduced glucose uptake at different brain areas in all of them, with bilateral occipital hypometabolism in 2 subjects. 16,17 Bilateral involvement, as shown by our patient, is also consistent with 2 previous PET studies of cerebral blood flow during migraine without aura attacks which showed bilateral hypoperfusion at occipital cortex spreading to temporal and parietal lobes.…”

Familial hemiplegic migraine with prolonged global aura: Follow-up findings of subtraction ictal SPECT co-registered to MRI (SISCOM)

“…Consecutive SPECT images at two different times, during and after a migraine with persistent aura, show different hyperperfusion foci in the affected hemisphere causing neurological symptoms (Figure 1). [1][2][3] …”