Table 2 Imaging studies in familial and sporadic hemiplegic migraine (FHM/SHM) to detect an inflammatory phenotype
FHM/SHM studies which reported on dural uptake | |||||||
Reference | Population (SHM/FHM) | Modality and contrast agent/tracer | Assumptions of technique | Delay after migraine onset | Comparisons | Results | Comments |
Dreier et al. [22] 2005 Neurology | FHM (ATP1A2 mutation carrier) (spontaneous) N = 1 | MRI w/ Gadolinium (Gd-DTPA) | Gadolinium passes a disrupted BBB | 1, 3, and 9 days after admission. Interictal scan after 17 months | FHM (aura phase) vs. FHM (interictal) | • Meningeal enhancement and BBB opening on the left hemisphere during aura phase • Pronounced contralateral cortical edema at day 9 on T2-weighted images | • Case report • Concomitant fever and neck stiffness • Lumbar puncture unremarkable • Neuropsychiatric deficits persisting for 3 months |
FHM/SHM studies which did not report on dural uptake | |||||||
Reference | Population (SHM/FHM) | Modality and contrast agent/tracer | Assumptions of technique | Delay after migraine onset | Comparisons | Results | Comments |
Cha et al. [23] 2007 Cephalalgia | FHM (spontaneous) N = 2 | MRI w/ Gadolinium PET w/ 18-FDG | Gadolinium enhancement increases with perfusion and a disrupted BBB 18-FDG uptake increases with cerebral metabolism | During hemiplegia | Symptomatic hemisphere vs. Asymptomatic hemisphere Before hemiplegic episode vs. During hemiplegic episode | • Cortical occipital edema contralateral and to a lesser extend ipsilateral to headache, persisting 9 days into attack in one case • Gadolinium enhancement in right posterior gyri • 18-FDG uptake increased in contralateral temporal, insular, and occipital lobes | • Case reports • Twins • One case with reduced level of consciousness and followed by lasting neurological deficits • CSF with elevated protein, otherwise unremarkable • A right temporal lobe and dural biopsy showed reactive lymphocytes and astrogliosis • Uptake of 18-FDG increases with neuronal activity and is not specific for inflammation |
Iizuka et al. [24] 2011 Journal of Neurology, Neurosurgery, and Psychiatry | FHM (spontaneous) N = 2 (6 attacks w/ contrast, 2 attacks w/o contrast) | MRI FLAIR w/ Gadolinium (unspecified) CBF-SPECT w/ HMPAO or IMP | Gadolinium passes a disrupted BBB HMPAO and IMP measures perfusion | Day 1-4 from aura onset | Ictal FHM (symptomatic hemisphere) vs. Ictal FHM (asymptomatic hemisphere) | • Early mild unilateral cortical edema at FLAIR (for 1/5 attacks in 1/2 patients) • Late cerebrospinal fluid enhancement on FLAIR in affected cortex in 1/3 attacks suggesting BBB leakage • Hyperperfusion in symptomatic hemisphere in 5 attacks and hypoperfusion in 3 attacks | • Gadolinium contrast may be unable to extravasate during minor decreases in BBB function • ATP1A2 mutation carriers • Early and late enhanced FLAIR was conducted 5-10 min and 2 post-contrast infusion, respectively |
Iizuka et al. [25] 2006 Cephalalgia | SHM (spontaneous) N = 1 | MRI (FLAIR w/ contrast): Gadolinium-based (Gd-DTPA)CBF-SPECT w/ HMPAO | Gadolinium passes a disrupted BBBHMPAO and IMP measures perfusion | Day 4 | SHM (aura phase) vs. SHM (interictal) | • BBB permeability increased in left posterior cortex • Increased CBF (hyperperfusion) of left hemisphere • Reduced blood flow (hypoperfusion) of right cerebellum | • Case report • Concomitant aphasia, right-sided hemiplegia, confusion, and agitation (right-handed patient) |
Pellerin et al. [26] 2019 Cephalalgia | SHM (spontaneous) N = 1 | MRI contrast enhanced T1-spin echo weighted images, FLAIR, and DWI (contrast unspecified) | Gadolinium passes a disrupted BBB | NA (during aura) | Ictal FHM (symptomatic hemisphere) vs. Ictal FHM (asymptomatic hemisphere) FHM (ictal) vs. Ictal FHM (interictal) | • Diffuse cortical enhancement of right hemisphere on T1-spin echo weighted images • Slight hyperintensity on FLAIR • Slight hyperintensity on DWI • Hyperperfusion of right hemisphere on non-contrast ASL | • Case report • Left-sided hemiplegia with headache and altered consciousness • Right frontal lobe biopsy with advanced neuronal suffering, ballooned cells, neoangiogenesis and fibrohyalinosis |