Progressive encephalomyelitis with rigidity and myoclonus after thymectomy in a woman with myasthenia gravis.

ABSTRACT: Progressive encephalomyelitis with rigidity and myoclonus (PERM), part of the spectrum of stiff-person syndrome (SPS), is a rare neurological disorder characterized by axial and limb rigidity, painful muscle spasms, hyperekplexia, brainstem signs, and autonomic dysfunction. Here, we present the case of a 44-year-old woman with a history of myasthenia gravis (MG) who had previously undergone a thymectomy. She presented with a 20-day history of startle-induced episodes of generalized rigidity and painful spasms affecting her face, trunk, and limbs. Her symptoms began gradually, initially with numbness and pain on the right side of her face, followed by sudden episodes of myoclonus and jerking, predominantly in axial muscles, triggered by auditory stimuli and light touch. Laboratory tests rev

SUBMITTER: Qin L 

PROVIDER: S-EPMC12040685 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature