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

Research output: Contribution to journalReview articlepeer-review

2 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)1483-1489
Number of pages7
JournalRheumatology International
Volume42
Issue number8
DOIs
Publication statusPublished - 2022 Aug

Keywords

  • Anti-topoisomerase I antibody
  • Dropped head syndrome
  • Inflammatory myopathy
  • Myositis
  • Systemic sclerosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology

Fingerprint

Dive into the research topics of 'Dropped head in systemic sclerosis: a case based review'. Together they form a unique fingerprint.

Cite this