Iliopsoas Hematomas in a Patient with Progressive Encephalomyelitis with Rigidity and Myoclonus

Intern Med. 2021 Aug 1;60(15):2475-2477. doi: 10.2169/internalmedicine.6604-20. Epub 2021 Feb 15.

Abstract

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and severe syndrome characterized by rigidity of the limb and truncal muscles, brainstem signs, myoclonus, and hyperekplexia. Iliopsoas hematoma is a serious complication of bleeding disorders that occurs most commonly in patients with hemophilia and also in association with anti-coagulant drug treatment. We herein present a case of PERM complicated with bilateral iliopsoas hematomas. His neurological symptoms improved after immunotherapy, and thereafter the iliopsoas hematomas disappeared. Neurologists should consider iliopsoas hematomas as a serious potential complication of PERM.

Keywords: PERM, progressive encephalomyelitis with rigidity and myoclonus; glycine receptor antibodies; hypercontraction; iliopsoas hematomas.

Publication types

  • Case Reports

MeSH terms

  • Encephalomyelitis*
  • Hematoma / diagnosis
  • Hematoma / diagnostic imaging
  • Humans
  • Muscle Rigidity
  • Myoclonus* / diagnosis
  • Myoclonus* / etiology
  • Receptors, Glycine

Substances

  • Receptors, Glycine

Supplementary concepts

  • Progressive Encephalomyelitis with Rigidity