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. 2015 Sep 24;2(5):e151.
doi: 10.1212/NXI.0000000000000151. eCollection 2015 Oct.

Incomplete Susac syndrome exacerbated after natalizumab

Lana Zhovtis Ryerson  1 , Ilya Kister  1 , Matija Snuderl  1 , Cynthia Magro  1 , Bibiana Bielekova  1
Affiliations

Incomplete Susac syndrome exacerbated after natalizumab

Lana Zhovtis Ryerson et al. Neurol Neuroimmunol Neuroinflamm. .
No abstract available

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Figures

Figure
Figure. Radiologic and pathologic findings
Fluid-attenuated inversion recovery (FLAIR) sequence with a single large T2-hyperintense signal involving the left frontoparietal lobe (A) with gadolinium-enhanced (Gd+) T1-weighted imaging (T1WI) demonstrating a small central area of contrast enhancement (B). FLAIR sequence demonstrating ongoing new T2 lesions involving bilateral hemispheres (C) with evidence of blood-brain barrier opening, with Gd+ T1WI again corresponding to the left parietal lobe lesion (D). Preferential involvement of the central portion of the corpus callosum (known as “snowball” lesions) (E) as well as a central callosal hole (F). FLAIR sequence demonstrating extensive T2-hyperintense lesions (G) with corresponding postcontrast T1WI confirming leptomeningeal enhancement along the left parietal hemisphere (H) days after the patient's first natalizumab infusion. (I) Low-power Luxol fast blue (LFB) hematoxylin & eosin (H&E) stain demonstrates white matter with patchy myelin loss. Myelin loss was most prominent in the perivascular white matter, with greater perivenular than periarterial involvement. (J) High-power view of perivascular macrophages also contained small particles of LFB– and periodic acid-Schiff–positive material consistent with the myelin accumulation, which can be seen in demyelinating disorders. (K) Vascular deposits of C3d. (L) A venule infiltrated by macrophages and lymphocytes. There is evidence of vascular compromise characterized by marked red cell extravasation. (M) Neurofilament stain, an axonal marker, showed relative preservation of axons; however, axons were focally distended and showed evidence of axonal injury (yellow arrowheads).
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References

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