Progressive encephalomyelitis with rigidity responsive to plasmapheresis and immunosuppression

Ann Neurol. 1996 Sep;40(3):451-3. doi: 10.1002/ana.410400315.


The stiff-man syndrome is reported in a 55-year-old woman who also had signs of severe bulbar musculature hypercontraction. These clinical features correspond to the progressive encephalomyelitis with rigidity syndrome. Diazepam was minimally beneficial, but after treatment with plasmapheresis and corticosteroids, she was still asymptomatic when seen 5 years later.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Disease Progression
  • Encephalomyelitis / complications*
  • Encephalomyelitis / physiopathology
  • Encephalomyelitis / therapy*
  • Female
  • Humans
  • Immunosuppression*
  • Middle Aged
  • Muscle Rigidity / etiology*
  • Plasmapheresis*


  • Adrenal Cortex Hormones