Acta Neuropathologica

, Volume 98, Issue 3, pp 313–316

Multiple episodes of thrombosis in a patient with Becker muscular dystrophy with marked expression of utrophin on the muscle cell membrane

  • I. Higuchi
  • Takahito Niiyama
  • Yuichi Uchida
  • Masaru Inose
  • Masanori Nakagawa
  • Kimiyoshi Arimura
  • Mitsuhiro Osame
Case report

DOI: 10.1007/s004010051086

Cite this article as:
Higuchi, I., Niiyama, T., Uchida, Y. et al. Acta Neuropathol (1999) 98: 313. doi:10.1007/s004010051086

Abstract

We previously reported a patient with Becker muscular dystrophy (BMD) who exhibited a benign clinical phenotype and marked expression of utrophin on the muscle cell membrane. The patient developed multiple episodes of thrombosis (middle cerebral and femoral arteries) in the course of the disease. We re-examined the biopsy muscle specimen from the patient immunohistochemically as to the expression of procoagulant or anticoagulant factors. We found a lower expression of thrombomodulin on the muscle cell membrane in the BMD patient compared with other BMD or Duchenne muscular dystrophy (DMD) patients. Although utrophin up-regulation in muscle is thought to prevent the muscle wasting in dystrophin-deficient DMD or BMD, the data obtained in the present study indicate that up-regulated utrophin may have an unexpected influence on the function of the vascular or coagulation system.

Key words UtrophinBecker muscular dystrophyThrombosis

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • I. Higuchi
    • 1
  • Takahito Niiyama
    • 1
  • Yuichi Uchida
    • 1
  • Masaru Inose
    • 1
  • Masanori Nakagawa
    • 1
  • Kimiyoshi Arimura
    • 1
  • Mitsuhiro Osame
    • 1
  1. 1.Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima 890-8520, Japan Tel.: +81-992-75-5332, Fax: +81-992-65-7164JP