Inclusion body myositis with neck muscle weakness and positive effect of immunoglobulins - A case report

Authors:

• Edvard Ehler,
• A. Meleková,
• J. Latta,
• P. Mandysová,
• P. Vojtíšek,
• J. Zámečník

Abstract

Inclusion body myositis is the most frequent inflammatory myopathy in patients aged 50 years and above. There is a chronic-progredient course of the disease without attacks and remissions. Typical clinical findings consist of proximal and distal weakness of extremity muscles and of axial muscles. Deep finger flexors of the upper extremities, quadriceps and muscles of the anterior compartment of the shin are severely affected. Dysphagia is a typical sign. Weakness of the neck muscles with a tilting head represents a rare and atypical form. We report a case of a 59-year old woman with progredient muscle weakness and atrophy with head tilting forward and dysphagia. Prednisone therapy was ineffective. After repeated intravenous administration of large immunoglobulin doses (60 g every four weeks), the posture of head a neck improved and dysphagia disappeared. One year into the treatment, immunoglobulin therapy is still highly effective.