Isolated inflammatory myopathy with rimmed vacuoles presenting with dropped head

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

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

b Department of Orthopaedic Surgery, Nara Medical University, Kashihara, Nara, Japan

c Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan

Corresponding 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.

PII: S0960-8966(09)00587-2

doi:10.1016/j.nmd.2009.08.006

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