Skip to main content

Advertisement

SpringerLink
Log in

Dropped head in systemic sclerosis: a case based review

  • Case Based Review
  • Published:
Rheumatology International Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Drain JP, Virk SS, Jain N, Yu E (2019) Dropped head syndrome: a systematic review. Clin Spine Surg 32:423–429

    Article  Google Scholar 

  2. Alhammad RM, Naddaf E (2020) Myopathies presenting with head drop: clinical spectrum and treatment outcomes. Neuromuscul Disord 30:128–136

    Article  Google Scholar 

  3. Akiyama M, Kaneko Y, Saito S, Matsumoto K, Kondo Y, Hanaoka H et al (2021) A case of dropped head syndrome due to focal myositis that worsened with cellulitis and improved only by treatment of cellulitis. Mod Rheumatol Case Rep 31:1–6. https://doi.org/10.1080/24725625.2021.1899381

    Article  Google Scholar 

  4. Kuwana M (2017) Circulating anti-nuclear antibodies in systemic sclerosis: utility in diagnosis and disease subsetting. J Nippon Med Sch 84:56–63

    Article  CAS  Google Scholar 

  5. Denton CP, Khanna D (2017) Systemic sclerosis. Lancet 390:1685–1699

    Article  Google Scholar 

  6. Yoshida T, Yoshida M, Mitsuyo K, Jonosono M, Higuchi I (2018) Dropped head syndrome and the presence of rimmed vacuoles in a muscle biopsy in scleroderma-polymyositis overlap syndrome associated with Anti-Ku antibody. Intern Med 57:887–891

    Article  Google Scholar 

  7. Fernández-Serna M, Arboleya L, Alonso S, Queiro R, Alperi M (2013) Dropped head syndrome in a patient with scleromyositis. J Clin Rheumatol 19:32–34

    Article  Google Scholar 

  8. Yoshinaga T, Miyazaki D, Fukushima K, Shimojima Y, Matsuda M, Ikeda S (2010) Dropped head sign and tongue atrophy in systemic sclerosis-associated myopathy: a case report. Rinsho Shinkeigaku 50:732–735

    Article  Google Scholar 

  9. Rojana-Udomsart A, Fabian V, Hollingsworth PN, Walters SE, Zilko PJ, Mastaglia FL (2010) Paraspinal and scapular myopathy associated with scleroderma. J Clin Neuromuscul Dis 11:213–222

    Article  Google Scholar 

  10. Garcin B, Lenglet T, Dubourg O, Mesnage V, Levy R (2010) Dropped head syndrome as a presenting sign of scleromyositis. J Neurol Sci 292:101–103

    Article  Google Scholar 

  11. Rosato E, Rossi C, Salsano F (2009) Dropped head syndrome and systemic sclerosis. Joint Bone Spine 76:301–303

    Article  Google Scholar 

  12. Gasparyan AY, Ayvazyan L, Blackmore H, Kitas GD (2011) Writing a narrative biomedical review: considerations for authors, peer reviewers, and editors. Rheumatol Int 31:1409–1417

    Article  Google Scholar 

  13. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A et al (2013) 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 72:1747–1755

    Article  Google Scholar 

  14. Katz JS, Wolfe GI, Burns DK, Bryan WW, Fleckenstein JL, Barohn RJ (1996) Isolated neck extensor myopathy: a common cause of dropped head syndrome. Neurology 46:917–921

    Article  CAS  Google Scholar 

  15. Muppidi S, Saperstein DS, Shaibani A, Nations SP, Vernino S, Wolfe GI (2010) Isolated neck extensor myopathy: is it responsive to immunotherapy? J Clin Neuromuscul Dis 12:26–29

    Article  Google Scholar 

  16. Toledano C, Gain M, Kettaneh A, Baudin B, Johanet C, Chérin P et al (2012) Aldolase predicts subsequent myopathy occurrence in systemic sclerosis. Arthritis Res Ther 14:R152

    Article  CAS  Google Scholar 

  17. Jung M, Bonner A, Hudson M, Pope JE, Canadian Scleroderma Research Group, (CSRG) Collaborators (2014) Myopathy is a poor prognostic feature in systemic sclerosis: results from the Canadian Scleroderma Research Group (CSRG) cohort. Scand J Rheumatol 43:217–220

    Article  CAS  Google Scholar 

  18. Koschik RW, Fertig N, Lucas MR, Domsic RT, Medsger TA (2012) Anti-PM-Scl antibody in patients with systemic sclerosis. Clin Exp Rheumatol 30:S12–S16

    PubMed  Google Scholar 

  19. D’Aoust J, Hudson M, Tatibouet S, Wick J, Canadian Scleroderma Research Group, Mahler M, Baron M, Fritzler MJ (2014) Clinical and serologic correlates of anti-PM/Scl antibodies in systemic sclerosis: a multicenter study of 763 patients. Arthritis Rheumatol 66:1608–1615

    Article  CAS  Google Scholar 

  20. Rozman B, Cucnik S, Sodin-Semrl S, Czirják L, Varjú C, Distler O et al (2008) Prevalence and clinical associations of anti-Ku antibodies in patients with systemic sclerosis: a European EUSTAR-initiated multi-centre case-control study. Ann Rheum Dis 67:1282–1286

    Article  CAS  Google Scholar 

  21. Mimura Y, Ihn H, Jinnin M, Asano Y, Yamane K, Tamaki K (2005) Clinical and laboratory features of scleroderma patients developing skeletal myopathy. Clin Rheumatol 24:99–102

    Article  Google Scholar 

  22. Allenbach Y, Benveniste O, Stenzel W, Boyer O (2020) Immune-mediated necrotizing myopathy: clinical features and pathogenesis. Nat Rev Rheumatol 16:689–701

    Article  CAS  Google Scholar 

  23. Bhansing KJ, Lammens M, Knaapen HK, van Riel PLCM, van Engelen BGM, Vonk MC (2014) Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and systemic sclerosis: a descriptive study on clinical features and myopathology. Arthritis Res Ther 16:R111

    Article  Google Scholar 

  24. Toru S, Kobayashi T, Akaza M, Yokota T, Mizusawa H (2012) Dropped head in polymyositis. Rheumatol Int 32:1105–1107

    Article  Google Scholar 

  25. Slobodin G, Rozenbaum M, Weller B, Boulman N, Rosneret I (2005) Dropped head: an unusual presentation of dermatomyositis. J Rheumatol 32:1174–1175

    PubMed  Google Scholar 

Download references

Author information

Author notes

  1. Tatsuya Shimada and Misako Higashida-Konishi contributed equally.

Authors and Affiliations

  1. Department of Connective Tissue Diseases, National Hospital Organization Tokyo Medical Center, Tokyo, 1528902, Japan

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

  2. Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 1608582, Japan

    Tatsuya Shimada, Mitsuhiro Akiyama, Hiroshi Takei & Keisuke Izumi

Authors
  1. Tatsuya Shimada
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Misako Higashida-Konishi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mitsuhiro Akiyama
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Satoshi Hama
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hiroshi Takei
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Keisuke Izumi
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Hisaji Oshima
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Yutaka Okano
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

TS, MHK, and MA wrote the manuscript and made the figures and a table; TS, MHK, MA, HS, HT, KI, HO, and OY were involved in the clinical management; MA and OY supervised the entire process.

Corresponding author

Correspondence to Mitsuhiro Akiyama.

Ethics declarations

Conflict of interest

All authors have no conflict of interest related to this case report.

Patient consent

Written informed consent for publication of this report was obtained from the patient.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Shimada, T., Higashida-Konishi, M., Akiyama, M. et al. Dropped head in systemic sclerosis: a case based review. Rheumatol Int 42, 1483–1489 (2022). https://doi.org/10.1007/s00296-021-04942-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-021-04942-z

Keywords

Search

Navigation