Dropped head in systemic sclerosis: a case based review

Tatsuya Shimada, Misako Higashida-Konishi, Mitsuhiro Akiyama, Satoshi Hama, Hiroshi Takei, Keisuke Izumi, Hisaji Oshima, Yutaka Okano

研究成果: Review article査読

1 被引用数 (Scopus)


Dropped head syndrome is a rare disease entity characterized by severe weakness of the cervical para-spinal muscles, resulting in a chin-on-chest deformity. Systemic sclerosis is one of the causes of dropped head syndrome, but its characteristics and prognosis remain unclear due to the extreme rarity of this condition. We present a case of dropped head which occurred in systemic sclerosis. He presented with severe dropped head and relatively mild weakness of the proximal limb muscles. Serum level of creatine kinase was elevated, myopathic change was observed in electromyography, and gadolinium enhancement was found in magnetic resonance imaging of his posterior neck muscles. Anti-topoisomerase I antibody was positive, while other autoantibodies such as anti-PM/Scl and anti-Ku antibodies were negative. Since his dropped head acutely progressed, high dose of glucocorticoid therapy was initiated, which successfully improved dropped head, serum level of creatine kinase, and gadolinium enhancement in magnetic resonance imaging. Our present case and literature review suggest that dropped head occurring in systemic sclerosis can be treatable with immunosuppressive therapy. It is important to recognize this rare but treatable involvement of systemic sclerosis because early diagnosis and treatment initiation are crucial to prevent the irreversible organ damage and the significant decrease of daily activities.

ジャーナルRheumatology International
出版ステータスPublished - 2022 8月

ASJC Scopus subject areas

  • 免疫アレルギー学
  • リウマチ学
  • 免疫学


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