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Clinical Pathological Conference |

Blind and Confused

Vibhash D. Sharma, MD1; Patrick Malafronte, MD2; Nicole De Simone, MD2; Benjamin M. Greenberg, MD, MHS1
[+] Author Affiliations
1Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas
2Department of Pathology, University of Texas Southwestern Medical Center, Dallas
JAMA Neurol. 2013;70(7):932-936. doi:10.1001/jamaneurol.2013.3105.
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A 62-year-old man developed confusion and was diagnosed as having encephalitis. The etiology was not identified. He continued to have cognitive impairment but remained clinically stable. Five months later, he woke with bilateral vision loss. On neurological examination, he had no light perception bilaterally. The remainder of the neurological examination results were normal. Magnetic resonance imaging of the brain revealed multiple brain lesions. He was treated with steroids and plasmapheresis, with mild improvement in vision. He was then transferred to a long-term care facility, where he developed increasing confusion and ultimately died. An autopsy was performed; the differential diagnosis, neuropathology, and final diagnosis are discussed here.

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Figure 1.
Brain Magnetic Resonance Image, T2-Weighted Fluid-Attenuated Inversion Recovery

Hyperintensities in the bilateral hemispheres (A), basal ganglia (B-C), hypothalamus (D), mesial temporal lobes (D-F), optic chiasm/tract (E), periaqueductal gray matter, and tectum (E-H).

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Figure 2.
Images and Stainings of the Brain

A, Ill-defined hemorrhagic/necrotic lesion in the left basal ganglia and temporal lobe isthmus. B, Tan gliotic-appearing optic chiasm. C, Poorly delineated destructive lesion with macrophages and marked perivascular chronic inflammation with prominent eosinophils (hematoxylin and eosin stain; original magnification ×400). D, Loss of normal aquaporin 4 immunostaining around the vessels within the lesion (aquaporin 4; original magnification ×200). Destructive lesion of the optic chiasm (E, hematoxylin and eosin stain; original magnification ×100), with prominent macrophages containing periodic acid-Schiff–positive digested myelin material (F, Luxol fast blue–Periodic acid-Schiff; original magnification ×400).

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