case_images: PMC8578066_01
case_images: MRI findings in the first prolonged attack. MRI performed 5 days after attack onset demonstrates right temporo-occipital lobe hypointensity on T1-weighted image (A) and hyperintensity on T2-FLAIR image (B) with restricted diffusion on DWI (C). MRI performed 4 weeks after symptom onset demonstrated right temporal-occipital cortical hyperintensity on T1-weighted images (D).
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case_images: PMC8578066_01_fneur-12-748034-g0001.jpg
case_images: F1
case_images: Repeated MRI performed 4 weeks after symptom onset showed hyperintensity on T1-weighted image in right occipital-temporal cortex, suggestive of cortical necrosis (Figure 1).
case_images: MRI performed 10 days after onset of the second prolonged attack. Right temporal-occipital hyperintensity on T1-weighted images (A) with gliosis on T2-FLAIR images (B) suggests the chronic stage of cortical necrosis caused by first prolonged attack. Left temporal-occipital cortex hyperintensity on T2-FLAIR images (B) with gyriform enhancement (C), restricted diffusion (D), and normal apparent diffusion coefficient (ADC) (E) suggests the subacute stage of cortical necrosis caused by second prolonged attack. MRA demonstrated vasodilation of the branches of the left middle cerebral artery (MCA) and posterior cerebral artery (PCA) (F).
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case_images: PMC8578066_01_fneur-12-748034-g0002.jpg
case_images: F2
case_images: MRA demonstrated vasodilation of the branches of the left middle cerebral artery (MCA) and posterior cerebral artery (PCA) (Figure 2). Transthoracic echocardiography revealed the presence of PFO. Mitochondrial DNA and whole exon sequence of nuclear DNA were analyzed by targeted region capture and high-throughput sequencing, but failed to detect any significant variant.
case_images: MRI performed 15 days after the onset of the second prolonged attack. DWI showed extensive cortical edema with diffusion restriction involving the left hemisphere (a). T1-weighted images showed slight hyperintensity in left temporal-occipital lobe and volume loss of right temporal-occipital lobe, suggestive of subacute and chronic stage of cortical necrosis, respectively (b). Gadolinium-enhanced T1-weighted images showed cortical enhancement in the left temporal-occipital lobe (c). MRA showed dilation of left MCA and PCA (d). PWI showed increased cerebral blood flow (CBF) (e) and cerebral blood volume (CBV) (f) in the left hemisphere. Region of interest (ROI) placed on the left temporal-parietal area showed a mean relative CBF value of 486.1 compared with that of 123.1 on the right temporal-parietal area. ROI placed on the left temporal-parietal area showed a mean relative CBV value of 533.1 compared with that of 129.8 on the right temporal-parietal area.
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case_images: PMC8578066_01_fneur-12-748034-g0003.jpg
case_images: F3
case_images: MRI revealed aggravated cortical edema involving the entire left hemisphere. MRA showed prominent vasodilation of left MCA and PCA while PWI showed hyperperfusion in corresponding region (Figure 3).