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Washington University Experience | NEURODEGENERATION | Anti-NMDA Receptor (Paraneoplastic) Encephalitis | 1R0 Case 1 Denouement
1R Case 1 Denouement ---- This case of autoimmune-mediated encephalitis showed evidence of symptoms related to all three autoantibodies: cerebellar degeneration (Hu), cognitive impairment and myelopathy (CRMP-5), and acute psychoses and mutism (NMDAR). The patients lack of sustained response to immunotherapy and characteristic neuropathological findings (neuronal cell loss and gliosis, perivascular cuffing and parenchymal invasion of CD8-positive lymphocytes, microglial nodules, and dorsal root ganglionopathy) suggest that Hu-mediated encephalitis was the primary driver behind the patient’s presentation. With respect to this patient’s adrenal adenoma, in review of the patient’s medical record, there was no clinical history of Cushing’s disease, hyperaldosteronism, or Conn’s syndrome to indicate that this was a functional adenoma, thus favoring the diagnosis as incidental. While the autopsy, both grossly and microscopically, did not reveal a source of his questionable “paraneoplastic disease”, specifically cancer in the lung or elsewhere, we cannot completely exclude the existence of an occult malignancy. (see the published case, PMID: 29494437)
