An unusual cause of acute ischemic stroke: Adult Onset Still’s Disease

  • Yihui GohEmail author
  • Venetia Yunjia Wong
  • Wei Lin Tan
  • Benjamin Yong-Qiang Tan
  • Hock Luen Teoh
Letter to the Editor


Adult Onset Still's Disease (AOSD) is a systemic inflammatory disease of unknown aetiology. The usual manifestations of AOSD are spiking fevers, arthritis, and an evanescent salmon-pink rash, with neurological manifestations occasionally described. Stroke is a rare manifestation of AOSD and the exact mechanism for stroke in AOSD remains unknown, although it has been hypothesized to be secondary to thrombocytosis or vasculitis. We present a case where acute ischemic stroke secondary to a floating internal carotid artery thrombus was an early manifestation of AOSD. The patient also had prolonged high spiking fevers, significant leucocytosis, arthralgias and transaminitis. He responded well to a high dose of oral corticosteroids and was eventually started on anticoagulation for secondary stroke prevention. To our knowledge, this is the first described case of arterial thrombosis associated with AOSD. We postulate that thrombocytosis, vasculitis and hypercoagulability from the underlying inflammatory state may have contributed to the ischemic stroke.


Adult Onset Still's Disease Stroke Arterial thrombosis Hypercoagulability 




Compliance with ethical standards

Conflict of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Supplementary material

Supplementary material 1 (MOV 3116 kb)

Extracranial doppler findings showing mobile right internal carotid artery thrombus

Supplementary material 2 (MOV 11720 kb)


  1. 1.
    Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P (2014) Adult-onset still’s disease. Autoimmun Rev 13(7):708–722CrossRefGoogle Scholar
  2. 2.
    Liu Z, Lv X, Tang G (2015) Clinical features and prognosis of adult-onset still’s disease: 75 cases from China. Int J Clin Exp Med. 8(9):16634–16639PubMedPubMedCentralGoogle Scholar
  3. 3.
    Ichiki H, Shishido M, Nishiyama S (1992) Two cases of adult onset of Still’s disease in the elderly. Nihon Ronen Igakkai Zasshi 29(12):960–964CrossRefGoogle Scholar
  4. 4.
    Kurasawa M, Kotani K, Kurasawa G, Shida K, Yamada S, Tago T (2007) Adult-onset Still’s disease in a patient over 80 years old successfully treated with low-dose methotrexate therapy. Age Ageing 36(1):104–106CrossRefGoogle Scholar
  5. 5.
    Giacomelli R, Ruscitti P, Shoenfeld Y (2018) A comprehensive review on adult onset Still’s disease. J Autoimmun 93:24–36CrossRefGoogle Scholar
  6. 6.
    Yamaguchi M, Ohta A, Tsunematsu T et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19(3):424–430PubMedGoogle Scholar
  7. 7.
    Desai SS, Allen E, Deodhar A (2002) Miller Fisher syndrome in adult onset Still’s disease: case report and review of the literature of other neurological manifestations. Rheumatology 41(2):216–222CrossRefGoogle Scholar
  8. 8.
    Kadavath S, Efthimiou P (2015) Adult-onset Still’s disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med 47(1):6–14CrossRefGoogle Scholar
  9. 9.
    Choi JK, Seo YJ, Kim DY (2018) Adult-onset still’s disease as the first manifestation of cerebral infarction: a case report. Brain Neurorehabil 11(2):e13CrossRefGoogle Scholar
  10. 10.
    Islam MMSU, Sarker MN, Hossain MI, Hossain MI, Kamruzzaman M (2016) Adult onset still’s disease. Faridpur Med Coll J 11(2):86–89CrossRefGoogle Scholar
  11. 11.
    Yanai H, Furutani N, Yoshida H, Tada N (2009) Myositis, vasculitis, hepatic dysfunction in adult-onset still’s disease. Case Rep Med 2009:504897CrossRefGoogle Scholar
  12. 12.
    Salamon L, Salamon T, Morovic-Vergles J (2009) Thrombotic microangiopathy in adult-onset still’s disease: case report and review of the literature. Wien Klin Wochenschr 121(17–18):583–588CrossRefGoogle Scholar
  13. 13.
    Efthimiou P, Kadavath S, Mehta B (2014) Life-threatening complications of adult-onset Still’s disease. Clin Rheumatol 33(3):305–314CrossRefGoogle Scholar
  14. 14.
    Calborean V, Gheorman V, Constantin C, Istrătoaie O (2018) Venous thromboembolism secondary to adult-onset still’s disease: a case report. J Cardiovasc Emerg 4(2):101–105CrossRefGoogle Scholar
  15. 15.
    Morita H, Nishiwaki H, Nagayama Y, Yoshimura A (2009) Portal vein thrombosis in adult-onset Still’s disease: a case report and literature review. Rheumatol Int 29(12):1515–1518CrossRefGoogle Scholar
  16. 16.
    Gallistl S, Mangge H, Neuwirth G, Muntean W (1998) Activation of the haemostatic system in children with juvenile rheumatoid arthritis correlates with disease activity. Thromb Res 92(6):267–272CrossRefGoogle Scholar
  17. 17.
    Merashli M, Jawad A, Ames PR (2015) Venous thromboembolism as initial manifestation of adult onset Still’s disease. Scand J Rheumatol 44(4):340–341CrossRefGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Neurology, Department of MedicineNational University Health SystemSingaporeSingapore
  2. 2.Department of MedicineNg Teng Fong General HospitalSingaporeSingapore
  3. 3.Department of Medicine, Yong Loo Lin School of MedicineNational University of SingaporeSingaporeSingapore

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