PMC8634645_01
Neuroimaging: Axial T2-Fluid attenuated inversion recovery (FLAIR) (A), coronal T2-weighted (B), coronal diffusion-weighted images (C), and with ADC map (D) from the initial MRI scan. Focal bilateral parietal cortical/subcortical high signal intensities with mild swelling were visible on T2-FLAIR and T2-weighted images (A,B), on diffusion weighted images (C), the affected areas showed largely no restriction, only vasogenic edema. Only small cortical sections were hypointense on ADC (D) indicative of cytotoxic edema.
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F2
The cranial MRI (cMRI) performed on the first day of admission was consistent with PRES showing multiple spot-like, partly confluent cortical/subcortical hyperintensities predominantly in the bilateral parietal region on FLAIR/T2-weighed images with slight focal swelling (Figures 2A,B). Four days after the seizure, a non-tender purpuric rash appeared on the lower extremity and buttocks/forearms/and on the trunk in loco typico for IgAV (Figure 2).
Skin lesions: Rash, appearing within the first 5 days after onset of the neurological impairment with hives, with small red spots or bumps on the buttocks, lower legs (A,C), knees, feet (B,C), elbows, and hands. The rash affected both sides of the body equally and did not turn pale on pressing. Occasionally, classical purpura was noted. The lesions completely resolved within 2 weeks.
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F3
Within the first 5 days of admission, the patient showed progressive petechial bleeding signs, which progressed to the classical and diagnostically groundbreaking palpable purpura (Figure 3), and suffered from another short episode of confusion and blurred vision.
The clinical phenotype of IgAV-patients in this cohort (A) Fraction of affected patients relative to the whole cohort of patients of whom information was available with different clinical manifestations depicted on the Y-axis. (B) Absolute numbers of patients who are not affected by different clinical manifestations are depicted on the left (open white bars), while the absolute number of reported patients affected are depicted to the right side (solid gray bars). gen. seizure, generalized seizure; loss/disturb. vision, loss/disturbed vision; foc. Seizure, focal seizure; cer. vasc., cerebral vasculitis; neuro sequelae, neurological sequelae.
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F4
The clinical spectrum of the given IgAV cohort is summarized in Figure 4.
PMC8634645_02