Neuromuscular Disorders
Volume 19, Issue 12 , Pages 853-855, December 2009

Isolated inflammatory myopathy with rimmed vacuoles presenting with dropped head

  • Hiroshi Kataoka

      Affiliations

    • Department of Neurology, Nara Medical University, Kashihara, Nara, Japan
    • Corresponding Author InformationCorresponding author. Address: Department of Neurology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan. Tel.: +81 744 29 8860; fax: +81 744 24 6065.
    • ,
  • Kazuma Sugie

      Affiliations

    • Department of Neurology, Nara Medical University, Kashihara, Nara, Japan
    • ,
  • Mari Terashima

      Affiliations

    • Department of Neurology, Nara Medical University, Kashihara, Nara, Japan
    • ,
  • Munehisa Koizumi

      Affiliations

    • Department of Orthopaedic Surgery, Nara Medical University, Kashihara, Nara, Japan
    • ,
  • Hirosei Horikawa

      Affiliations

    • Department of Neurology, Nara Medical University, Kashihara, Nara, Japan
    • ,
  • Ichizo Nishino

      Affiliations

    • Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
    • ,
  • Ikuya Nonaka

      Affiliations

    • Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
    • ,
  • Satoshi Ueno

      Affiliations

    • Department of Neurology, Nara Medical University, Kashihara, Nara, Japan

Received 12 May 2009; received in revised form 13 August 2009; accepted 19 August 2009.

Abstract 

We describe an unusual case of inflammatory myopathy with rimmed vacuoles associated with dropped head syndrome. Muscle biopsy in our patient revealed variations in fiber size with fiber necrosis and regeneration, accompanied by many rimmed vacuoles and areas of endomysial cell infiltration. Electron microscopy demonstrated autophagic vacuoles and tubulofilamentous inclusions. This myopathy can cause dropped head syndrome in a subgroup of patients.

Keywords: Dropped head, Inclusion body, Rimmed vacuoles, Paraspinal muscles

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PII: S0960-8966(09)00587-2

doi:10.1016/j.nmd.2009.08.006

Neuromuscular Disorders
Volume 19, Issue 12 , Pages 853-855, December 2009

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