Figure Disease course and neuropathology of CASPR2 encephalitis(A) Summary of the most important changes during the disease course. Disease course and.

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Presentation transcript:

Figure Disease course and neuropathology of CASPR2 encephalitis(A) Summary of the most important changes during the disease course. Disease course and neuropathology of CASPR2 encephalitis(A) Summary of the most important changes during the disease course. Shown are consecutive cerebral fluid-attenuated inversion recovery (FLAIR) MRI scans in relation to therapeutic treatment, clinical symptoms, and CASPR2 titer. The swollen hippocampus (HC) and amygdala (AM) were resected in January 2013. Note the progressive global atrophy of the brain (from August 2012 to July 2013), which largely stopped in October 2013. The left HC, however, remained FLAIR intense in October 2013. (B) Changes in CASPR2 antibody (ab) titers in serum (dots) and CSF (squares) during the disease course. The graph shows a decline of the titers in serum and CSF after prolonged administration of cyclophosphamide and oral prednisolone (indicated by the gray bars on top). The star indicates the time point of the biopsy. (C–K) Neuropathology of the right hippocampus. (C) Double staining for CD3 and CD8 shows moderate numbers of T cells (CD8+ T cells are blue, CD3+CD8− [CD4+] T cells are brown). (D) CD68 staining shows moderately activated microglial cells. (E) Staining for CD20 shows the presence of B cells in a perivascular cuff. (F) Staining for CD138 shows the presence of some plasma cells. (G) Staining for immunoglobulins (Ig) reveals strong leakage into the parenchyma. Deposition of immunoglobulin on neuronal membranes is indicated by the arrowheads. The arrow in the upper corner points at a degenerating neuron with nuclear changes. The inset shows an additional degenerating neuron (arrow) with a condensed nucleus. (H) Staining for TUNEL (black) and MAP2 shows the absence of degenerating cells in the amygdala of this patient. (I) In the hippocampus, a single TUNEL-positive neuron is indicated by the arrowhead. The insets show an enlargement of this neuron (left side) and a second MAP2+ TUNEL+ neuron. (J, K) Staining for complement C9neo (end complex) reveals a neuron with minor deposition, depicted by arrows (J), and a neuron with major deposition (K). CP = cyclophosphamide; MP = methylprednisolone. Peter Körtvelyessy et al. Neurol Neuroimmunol Neuroinflamm 2015;2:e75 © 2015 American Academy of Neurology