Neuronal injuries evidenced by transient cortical magnetic resonance enhancement in hemiplegic migraine: A case report

Abstract

Background

Magnetic resonance imaging abnormalities in hemiplegic migraine have been described previously but were limited to a cortical thickening and biphasic alternation of hypoperfusion and hyperperfusion. Our report reveals possible blood-brain barrier disruption during migraine.

Case

We present the first demonstrated case of regressive diffuse hemispheric cortical enhancement in sporadic hemiplegic migraine, with histological correlation revealing neuronal lesions similar to ischemic lesions. This is probably due to the severity of the attack as indicated by the left hemiplegia and transient altered consciousness in our 43-year-old male patient.

Conclusion

Cortical contrast enhancement on 3D T1 images may suggest migraine severity and be predictive of neuronal loss.

A 43-year-old male patient, with previous migraine history, was examined in the neuro-intensive care unit of our institution for a second episode of left hemiplegia that had appeared 5 days before. It was rapidly associated with a right unilateral headache and altered consciousness. Brain magnetic resonance imaging (MRI) was performed and showed diffuse cortical enhancement of the right hemisphere associated with a slight hyperintensity on axial FLAIR (fluid-attenuated inversion-recovery) and diffusion-weighted imaging (DWI) images and an intense cortical hyperperfusion on non-contrast arterial spin labeling (ASL) perfusion images (Figure 1, upper row).

Figure 1.

Upper row: Brain MRI of a 43-year-old male patient performed after the onset of transient left hemiplegia and headache, showing a blood-brain barrier (BBB) disruption with diffuse cortical enhancement of the right hemisphere on contrast enhanced 3D T1-spin echo weighted images (a) associated with a slight hyperintensity on axial T2-FLAIR (fluid-attenuated inversion-recovery) (b) and diffusion-weighted imaging images (c); also, an intense cortical hyperperfusion with increased cerebral blood flow (CBF) on non-contrast arterial spin labeling (ASL) perfusion images (d). Lower row: MRI abnormalities’ regression a few weeks later on the same sequences ((e), (f), (g), (h)).

Several diagnostic hypotheses were raised such as encephalitis, vasculitis, post-epileptic abnormalities or infections, but were ruled out by an extensive work-up including plasma studies: Blood culture; polymerase chain reaction (PCR) human immunodeficiency virus (HIV); hepatitis B virus (HBV); hepatitis C virus (HCV); hepatitis E virus (HEV); herpes simplex virus-1 (HSV-1); herpes simplex virus-2 (HSV-2); varicella-zoster virus (VZV); cytomegalovirus (CMV); Epstein-Barr virus (EBV); human herpes virus-6 (HHV-6); human herpes virus-8 (HHV-8); parvovirus B19; Treponema pallidum haemagglutination/Venereal Disease Research Laboratory (TPHA/VDRL); Lyme-Borreliosis; PCR West Nile virus; Dengue fever; Zika virus; Chikungunya virus; Bartonnella; serum protein electrophoresis (SPE); angiotensin-converting enzyme (ACE); anti-nuclear, anti-double stranded deoxyribonucleic acid (anti-dsDNA); anti-Smith; anti-Sjogren’s syndrome A (anti-SSA) and anti-Sjogren’s syndrome B (anti-SSB) antibodies; anti-neutrophil cytoplasmic antibodies (ANCA); anti-ganglioside antibodies; anti-neuronal, anti-N-methyl-D-aspartate receptor (anti NMDAR) and voltage gated kalium channel (VGKC) antibodies. Cerebrospinal fluid studies were also performed including bacteriological analysis, PCR listeria, PCR HSV 1-2, VZV, CMV, EBV, HHV-6, acid-fast bacilli research, cryptococcal antigen, Lyme-Borreliosis, PCR West Nile virus, Dengue fever, Zika virus, Chikungunya virus, JC virus, immunophenotyping, interleukin-6 (IL-6) and interleukin-10 (IL-10) measures. A cerebral angiography found no arterial caliber abnormality. Consecutive electroencephalograms, performed during symptoms, found high amplitude delta waves in the right hemisphere, without any epileptic pattern and with a normal background activity. A biopsy of the right frontal lobe was even performed, revealing advanced neuronal suffering, ballooned cells, neoangiogenesis with fibrohyalinosis suggestive of non-inflammatory vasculopathy.

The left deficit lasted about three weeks with a gradual and spontaneous improvement. MRI abnormalities regressed within a few weeks (Figure 1, lower row). Despite negative molecular genetic analysis and the absence of any family headache history, as no other etiology could be evidenced, the diagnosis of sporadic hemiplegic migraine was considered the most likely.

So far, only a few reports have depicted MRI changes in hemiplegic migraine such as cortical thickening (1) and biphasic neurological changes with alternation of hypoperfusion and hyperperfusion (2). To our knowledge, one earlier case of familial hemiplegic migraine showed blood-brain barrier (BBB) breakdown, similarly to ours (3); however, the cortical enhancement appears much less demonstrative in this previous report. Most recent studies have shown there was no BBB disruption during attacks of migraine with aura (4); however, its occurrence may be relative to the severity of the attack. Of note, in both reports with BBB disruption, the patients had to be transferred to intensive care units. Furthermore, cytotoxic oedema and brain atrophy following hemiplegic migraine attacks have been reported (5). This is coherent with our findings of slight atrophy and diffuse hypoperfusion at late follow-up. Lastly, our report is the sole study to have correlated the MRI findings with histological lesions.