PMC5645167_01
MRI images at presentation. A: Brain MRI FLAIR axial image showing a focal area of hyper intensity in the right peri ventricular white matter and corona radiata. B: Brain MRI FLAIR axial image showing cortical and subcortical hyper intensity in the left parietal lobe. C: Brain MRI T1 post contrast coronal image showing corresponding cortical enhancement of the left parietal lesion.
gr1.jpg
PMC5645167_01_gr1.jpg
f0005
The images were thought to be radiologically suggestive of inflammatory vasculitic lesions or ictal hypoxemia. (Fig. 1).
Cerebral angiography. Sagittal view of conventional angiography showing no focal area of stenosis or beading appearance to raise a suspicion of vasculitis.
gr2.jpg
PMC5645167_01_gr2.jpg
f0010
CT scan of the chest, abdomen and pelvis was done and revealed no abnormalities. Cerebral angiogram was done as part of the workup for CNS vasculitis and showed no evidence of small vessel disease. (Fig. 2) Despite extensive investigation no identifiable cause could be delineated.
MRI images at presentation and 3 months follow-up. A: Brain MRI at presentation. B: Brain MRI at 3-months follow-up. Axial FLAIR image showing confluent peri-ventricular white matter hyper-intensity sparing the subcortical U fibers.
gr3.jpg
PMC5645167_01_gr3.jpg
f0015
His repeated brain MRI showed dramatic worsening which was attributed most probably to the tapering of corticosteroid (Fig. 3).
Pathology slides. A: H & E stain Vessel wall showing inflammation of all the layers of the vessel wall. B: Vessel wall with lymphocytic infiltration. Lymphocytes are small and mature. C: Iron indicating damage of the vessel wall. D: Normal meninges.
gr4.jpg
PMC5645167_01_gr4.jpg
f0020
The biopsy was composed of many cortical and white matter fragments showing many focal angiocentric mononuclear inflammatory cell infiltrates composed almost exclusively of lymphocytes, predominantly T cells (Fig. 4).