A Case of Anti-Caspr2 Autoimmune Encephalitis Associated with Adenocarcinoma of the Lung
  • Fábio Pé D’Arca Barbosa
    Internal Medicine Department, Hospital Garcia de Orta, Almada, Portugal
  • Francisco Vara-Luiz
    Gastroenterology Department, Hospital Garcia de Orta, Almada, Portugal
  • Ana Albuquerque
    Internal Medicine Department, Hospital Garcia de Orta, Almada, Portugal
  • Ana Valada Marques
    Internal Medicine Department, Hospital Garcia de Orta, Almada, Portugal
  • Vanda Spencer
    Internal Medicine Department, Hospital Garcia de Orta, Almada, Portugal

Keywords

Autoimmune encephalitis, paraneoplastic syndrome, anti-Caspr2, lung adenocarcinoma

Abstract

Autoimmune encephalitis (AIE) is an inflammatory brain condition with multiple aetiologies but is mainly associated with paraneoplastic syndromes. Several antibodies described in AIE are being investigated in relation to different cancers, including antibodies against Contactin-associated protein-like 2 (Caspr2), which have been associated with thymoma but very rarely with lung cancer. The authors present the case of a 72-year-old man with cardiovascular risk factors, who presented with a 3-week history of left hemichorea following a first unprovoked seizure the week before, with no other signs or symptoms. The patient was submitted to extensive aetiological investigation, testing positive for anti-Caspr2 antibodies in the cerebrospinal fluid. AIE was diagnosed after other possible causes had been excluded. A PET scan showed signs of abnormal metabolism, with a lung biopsy confirming lung adenocarcinoma. This case highlights a very rare association and the importance of a thorough aetiological investigation for neurological complaints.

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References

  • Patterson K, Dalmau J, Lancaster E. Mechanisms of Caspr2 antibodies in autoimmune encephalitis and neuromyotonia. Ann Neurol 2018;83(1):40–51.
  • van Sonderen A, Ariño H, Petit-Pedrol M, Leypoldt F, Körtvélyessy P, Wandinger K, et al. The clinical spectrum of Caspr2 antibody-associated disease. Neurology 2016;87(5):521–528.
  • Qin X, Yang H, Zhu F, Wang Q, Shan W. Clinical character of CASPR2 autoimmune encephalitis: a multiple center retrospective study. Front Immunol 2021;12:652864.
  • Boyko M, Au K, Casault C, de Robles P, Pfeffer G. Systematic review of the clinical spectrum of CASPR2 antibody syndrome. J Neurol 2020;267(4):1137–1146.
  • Graus F, Vogrig A, Muñiz-Castrillo S, Antoine J, Desestret V, Dubey D, et al. Updated diagnostic criteria for paraneoplastic neurologic syndromes. Neurol Neuroimmunol Neuroinflamm 2021;8(4):e1014.
  • Abboud H, Probasco J, Irani S, Ances B, Benavides D, Bradshaw M, et al. Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry 2021;92(7):757–768.
  • Dalmau J, Geis C, Graus F. Autoantibodies to synaptic receptors and neuronal cell surface proteins in autoimmune diseases of the central nervous system. Physiol Rev 2017;97(2):839–887.
  • Cervera R, Piette J, Font J, Khamashta M, Shoenfeld Y, Camps M, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002;46(4):1019–1027.
  • Seluk L, Taliansky A, Yonath H, Gilburd B, Amital H, Shoenfeld Y, et al. A large screen for paraneoplastic neurological autoantibodies; diagnosis and predictive values. Clin Immunol 2019;199:29–36.
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    Published: 2022-11-02
    Issue: 2022: Vol 9 No 11 (view)


    How to cite:
    1.
    Pé D’Arca Barbosa F, Vara-Luiz F, Albuquerque A, Valada Marques A, Spencer V. A Case of Anti-Caspr2 Autoimmune Encephalitis Associated with Adenocarcinoma of the Lung. EJCRIM 2022;9 doi:10.12890/2022_003544.

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