PMC6746545_01
Head computed tomography (CT) and magnetic resonance imaging (MRI) findings. These images were obtained 8 months after the disease onset. Clinically, facial mimicry and pathological laughing were remarkable at this stage. a: CT images show edematous and swelling findings in the right cerebral hemisphere, particularly in the temporal and parietal lobes (arrows). In the right temporal and parietal cortices, the sulcus shows narrowing and the corticomedullary junction is generally indistinct. b: Diffusion weighted MRI shows widespread gyriform hyperintensity in the right cerebral hemisphere and left parietal and occipital lobes, except in the medial occipital regions. c: T2-weighted images show hyperintense regions with swelling (arrowheads), and the regions correspond to those that were hyperintense on diffusion weighted MRI. R, right side.
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Head computed tomography showed swelling in the right cerebral hemisphere (Figure 1a). Diffusion-weighted (DW) magnetic resonance imaging (MRI) showed extensive gyriform hyperintensity of the cerebral cortex (Figure 1b). The hyperintense regions on DW imaging (DWI) were also hyperintense with edematous swelling on T2-weighted (Figure 1c) and fluid-attenuated inversion recovery (FLAIR) images and showed cerebral blood flow decrease on single photon emission computed tomography (Figure 2).
SPECT findings. 99mTc-ECD-SPECT images obtained 5 months after the onset of symptoms revealed decreased regional cerebral blood flow in the right cerebral hemisphere, particularly in the parietal lobe. The regions with decreased cerebral blood flow almost corresponded to the regions that were hyperintense on diffusion weighted magnetic resonance imaging. 99mTc-ECD-SPECT, technetium-99m ethyl cysteinate dimer single photon emission computed tomography; R, right side.
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The hyperintense regions on DW imaging (DWI) were also hyperintense with edematous swelling on T2-weighted (Figure 1c) and fluid-attenuated inversion recovery (FLAIR) images and showed cerebral blood flow decrease on single photon emission computed tomography (Figure 2).
Expression of facial mimicry of the patient. These photographs were obtained 8 months after disease onset. The physician faced the patient and sequentially showed several facial expressions to the patient. The physician gazed at the patient (a). Then, sequentially, the physician widely opened the eyes (b) and showed a smiling expression (joy) (c), sadness (tragedy) (d), astonishment (surprise) (e), and a wry expression (grimacing)(f).
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PSWCs. Pathological laughing and startle reaction appeared 7 months after symptom onset, and subsequently, she showed facial mimicry; she imitated the facial expressions of the person who faced her, including members of her family and of the medical staff, such as sadness, wide opening of the eyes or mouth, surprise, smiling, tearful expressions, and grimaces (Figure 3).