PMC4531751_01
Axial T2-weighted (3,000/85/2) image at the level of middle cerebellar peduncle showed ill-defined hyperintense lesions (left). After treatment with prednisolone, these lesions nearly disappeared (right).
kjim-15-1-89-16f1.jpg
PMC4531751_01_kjim-15-1-89-16f1.jpg
f1-kjim-15-1-89-16
Brain MRI demonstrated diffuse, patchy increased signal intensities involving the brain stem, both basal ganglia and right thalamus on T2-weighted axial images, which were suggestive of multiple ischemic events related to vasculitis (Fig. 1).
Sural nerve biopsy showed thickened blood vessels with marked inflammatory cell I infiltration in the epineurium and perineurium (left, x40), and fibrinoid necrosis in the entire vessel wall (right, x100). Hematoxylin and eosin.
kjim-15-1-89-16f2.jpg
PMC4531751_01_kjim-15-1-89-16f2.jpg
f2-kjim-15-1-89-16
Sural nerve biopsy specimens demonstrated thickened vessel walls with mononuclear inflammatory cell infiltration in epineurium and perineurium (Fig. 2).
Muscle biopsy of vastus lateralis showing mild mononuclear inflammatory cell infiltration in endomysial perivascular area. Hematoxylin and eosin (x100).
kjim-15-1-89-16f3.jpg
PMC4531751_01_kjim-15-1-89-16f3.jpg
f3-kjim-15-1-89-16
Muscle biopsy of vastus lateralis showed muscle fiber necrosis and degeneration, variation in fiber size with internal nuclei and mononuclear inflammatory cell infiltration in endomysial and perimysial perivascular areas (Fig. 3).
Liver biopsy demonstrated microscopic features consistent with primary biliary cirrhosis early stage: inflammatory cell infiltration, absence of bile duct in the portal area (left) and biliary piecemeal necrosis (right). Hematoxylin and eosin (x100).
kjim-15-1-89-16f4.jpg
PMC4531751_01_kjim-15-1-89-16f4.jpg
f4-kjim-15-1-89-16
Needle aspirated liver biopsy specimen noted findings consistent with the early change of primary biliary cirrhosis (Figure 4).