Therapeutic considerations in a case of progressive encephalomyelitis with rigidity and myoclonus

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is an inflammatory disorder of the stiff-person syndrome spectrum that presents subacutely with trunk and limb rigidity, muscle spasms, brainstem dysfunction, and autonomic instability [1,2]. PERM has been strongly associated with IgG1 and IgG3 autoantibodies against the glycine receptor alpha-1 subunit (GlyR α1) [3,4]. Glycine receptors facilitate inhibitory signaling in the brainstem and spinal cord. Autoantibodies against GlyRα1 show pathogenic characteristics in vitro such as complement activation and receptor internalization [4].

Source: Journal of the Neurological Sciences - June 17, 2020 Category: Neurology Authors: Lauren Gluck, Amanda L. Hernandez, Sarah F. Wesley, Robert K. Fulbright, Erin E. Longbrake, Panos Stathopoulos Tags: Letter to the Editor Source Type: research