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In this issue of Neurology®, Turner et al.1 present the case of a 28-year-old man with progressive encephalomyelitis with rigidity and myoclonus (PERM) in the presence of serum autoantibodies to the NMDA receptor (NMDAR) and the glycine receptor (GlyR). The patient died and underwent autopsy. The CNS neuropathology constitutes the core findings from the group that is well-known for contributions to the field of neuronal autoantibodies.
Why is one so curious to read the results of this report? It broadens our knowledge to read that in the presence of these autoantibodies PERM can occur in dramatic fashion and rapidly lead to death. Even more so, however, a dedicated neuropathologic analysis is, as in an earlier era, an essential part of defining inflammatory CNS syndromes.2 Autoantibody assays are now the primary method of making the diagnosis of immune-mediated disorders of the gray CNS matter. This has led to questionable developments. Even though neuropathology has made considerable technical advances and has contributed to our modern understanding of several neurologic disorders, little is known about the brain …
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