Childhood dermatomyositis associated with intracranial tumor and liver cysts

Nina Barišić, Jasminka Jakić-Razumović, Miroslav Harjaček, Marina Fanin, Hanns Lochmüller, Ivan Lehman, Corrado Angelini

Research output: Contribution to journalArticlepeer-review

Abstract

We present a girl with dermatomyositis, liver cysts and choroid plexus papilloma who was treated and followed for 7 years. Muscle histology revealed an inflammatory muscle disease and similar changes were detected in a brain tumor that was surgically removed at onset. Western blot analysis of the muscle revealed severely reduced calpain-3 protein. She was treated with pulse methylprednisolone treatment (800 mg i.v. for 4 days) followed by oral prednisone treatment (16 mg on alternate day) for 14 months, which improved muscle strength. Moreover, the cystic liver formations disappeared during steroid treatment. This is an unusual association of muscular disorder, steroid-responsive liver cysts, intracranial tumor and secondary calpain-3 deficiency. We speculate that this association is not coincidental, but mediated by an autoimmune attack against an antigen that is shared among the target tissues.

Original languageEnglish
Pages (from-to)76-80
Number of pages5
JournalEuropean Journal of Paediatric Neurology
Volume11
Issue number2
DOIs
Publication statusPublished - Mar 2007

Keywords

  • Calpain-3 deficiency
  • Child
  • Choroid plexus papilloma
  • Dermatomyositis
  • Liver cysts

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Neurology

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