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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | NMO (Neuromyelitis Optica) | NMO Spectrum Disorder | 5A0B Case 5 History - 2
Autopsy results - The external surface of the brain was unremarkable. Horizontal brain sections revealed gray discoloration and cystic change in periventricular areas and the posterior internal capsule, thalamus, globus pallidus, and putamen bilaterally. Several similar lesions, 0.3-2.5 cm, were noted primarily in the right frontal lobes and pons, spanning gray-white matter or confined to white matter. The cerebral and middle cerebellar peduncles and pons were soft and swollen. No abnormalities were noted in the cranial nerves, including the optic nerves or chiasm. The spinal cord was diffusely atrophic with poor delineation of gray and white matter. Histological examination disclosed myelin loss in the lesions, with CD68 positive macrophages, rare lymphocytes and occasional Creutzfeldt cells. While the autopsy case was being worked up, a report of a positive serum antibody to aquaporin-4 was received from Mayo Clinic, at a titer of 1:15,360. The lesions did not stain with AQ4 immunohistochemistry which is usually immunoreactive in multiple sclerosis.