PMC4925902_01
Axial T2 MRI FLAIR sequences with multifocal hyperintensities in the right temporal lobe (A), left anterior insula (B), and left occipital lobe (B, C). Blooming artifact over the left occipital region showing micro hemorrhages on the gradient echo sequences (D).
gr1.jpg
PMC4925902_01_gr1.jpg
f0005
Magnetic resonance imaging (MRI) revealed left occipital white matter and cortical T2 hyperintensity with mild focal mass effect, without enhancement but with petechial hemorrhages on gradient echo (GRE) imaging and with a second smaller focus of T2 hyperintensity in the left anterior insula (Fig. 1).
Relapsing appearance of the left posterior quadrant lesion at 1 month, 3 months, 6 months, and 9 months of symptom onset (A, B, C, D, respectively).
gr2.jpg
PMC4925902_01_gr2.jpg
f0010
However, a repeat MRI performed in March 2015 in preparation for the biopsy showed significant improvement of the left occipital and left anterior insular lesions (Fig. 2B). The patient was again discharged home, but follow-up MRI in June 2015 showed worsening of the left occipital T2 hyperintensity (Fig. 2C) and new left frontal T2 hyperintensities, as well as new enhancement of the lesion in the right anterior temporal lobe (not shown). Repeat imaging in September of 2015 showed marked improvement of all T2 hyperintense lesions and complete resolution of all enhancement (Fig. 2D).
Hematoxylin and eosin (H&E) staining showed reactive changes including vacuolization suggestive of edema and astrocytosis/gliosis (A). Glial fibrillary acidic protein (GFAP) staining highlighted reactive astrocytes (B). H&E staining also demonstrated thickened blood vessels with perivascular hemosiderin deposition (C), and beta-amyloid immunohistochemistry revealed beta-amyloid deposition in blood vessel walls (D). These findings were consistent with cerebral amyloid angiopathy.
gr3.jpg
PMC4925902_01_gr3.jpg
f0015
The biopsy showed reactive changes and thickened blood vessels with beta-amyloid deposition in vessel walls (Fig. 3).
Continuous lateralized periodic discharges with overlying fast activity (LPD + F) over the left posterior quadrant (maximal at O1 > P3/T5) (A), evolving into 3-4 electrographic seizures per hour, consisting of 9-Hz rhythmic sharp activity (B), without clinical return to baseline between seizures.
gr4.jpg
PMC4925902_01_gr4.jpg
f0020
An EEG was obtained, revealing continuous lateralized periodic discharges with overlying fast activity over the left posterior quadrant (Fig. 4A), evolving into 3-4 electrographic seizures per hour (Fig. 4B), consisting of 9-Hz rhythmic sharp waves, without clinical return to baseline between seizures.