Advertisement

The Cerebellum

, Volume 18, Issue 5, pp 976–982 | Cite as

Stroke-Like Presentation of Paraneoplastic Cerebellar Degeneration: a Single-Center Experience and Review of the Literature

  • Alberto VogrigEmail author
  • Andrea Bernardini
  • Gian Luigi Gigli
  • Elisa Corazza
  • Alessandro Marini
  • Samantha Segatti
  • Martina Fabris
  • Jérôme Honnorat
  • Mariarosaria Valente
Short reports
  • 93 Downloads

Abstract

Paraneoplastic cerebellar degeneration (PCD) is usually thought to have a subacute progression over several weeks. We report herein incidence and clinical features of hyperacute onset PCD, a vertebrobasilar stroke mimic. We performed a retrospective analysis of all suspected PCD cases referred to the Udine University Hospital between 2009 and 2017. Our center provides the only neuroimmunology laboratory for three provinces of the Friuli-Venezia Giulia region, Italy (983,190 people as of January 1, 2017). Inclusion criteria were (1) abrupt onset of neurological symptoms; (2) initial consideration of a vascular etiology; (3) final diagnosis of “definite PCD.” We also carried out a systematic review of the literature in order to identify previous stroke-like PCD cases. Between 2009 and 2017, 24 patients received a final diagnosis of PCD. The age-standardized incidence rate of PCD was 0.22/100,000 person-years. Two cases (8.3%) had a stroke-like onset, with an incidence of 0.02/100,000 person-years. Additionally, 10 previously reported stroke-like PCD cases were identified. Among all cases (n = 12), 67% were female; median age was 51 years (range, 22–69). An associated cancer was discovered in all cases. Brain imaging was normal in most (75%) of the patients. Cerebrospinal fluid (CSF) analysis showed inflammatory alterations in 73% of the cases. Cancer treatment was more effective than immunotherapy in improving the neurological syndrome. Typical patients with hyperacute PCD are middle-aged women with normal brain imaging, inflammatory markers in CSF, and cancer. Surgery of the underlying cancer is probably the best treatment. PCD must be considered in the differential diagnosis of acute-onset ataxia and/or vertigo.

Keywords

Paraneoplastic syndromes Incidence Epidemiology Stroke Differential diagnosis Ataxia 

Notes

Author Contributions

Drs. Vogrig and Bernardini designed and conceptualized the study, collected and interpreted the data, and wrote the manuscript. Drs. Corazza, Marini, Segatti, and Fabris collected and analyzed the data. Prof. Honnorat analyzed and interpreted the data and provided critical revision of the manuscript. Prof. Gigli and Prof. Valente interpreted the data, revised the manuscript, and supervised the study.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Ethical Standard

This study followed the tenets of the Declaration of Helsinki and was performed according to the guidelines of the Institutional Review Board of University of Udine Medical School.

Supplementary material

12311_2019_1075_MOESM1_ESM.docx (31 kb)
ESM 1 (DOCX 31 kb)

References

  1. 1.
    Choi J-Y, Lee S-H, Kim J-S. Central vertigo. Curr Opin Neurol. 2018;31:81–9.CrossRefPubMedGoogle Scholar
  2. 2.
    Burton TM, Luby M, Nadareishvili Z, Benson RT, Lynch JK, Latour LL, et al. Effects of increasing IV tPA-treated stroke mimic rates at CT-based centers on clinical outcomes. Neurology. 2017;89:343–8.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Nam K-W, Kim CK, Kim TJ, An SJ, Oh K, Ko S-B, et al. Intravenous thrombolysis in acute ischemic stroke with active cancer. Biomed Res Int. 2017;2017:4635829.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Weeda ER, Bohm N. Association between comorbid cancer and outcomes among admissions for acute ischemic stroke receiving systemic thrombolysis. Int J Stroke. 2019;14:48–52.CrossRefPubMedGoogle Scholar
  5. 5.
    Shireman TI, Howard PA, Kresowik TF, Ellerbeck EF. Combined anticoagulant-antiplatelet use and major bleeding events in elderly atrial fibrillation patients. Stroke. 2004;35:2362–7.CrossRefPubMedGoogle Scholar
  6. 6.
    Mitoma H, Adhikari K, Aeschlimann D, Chattopadhyay P, Hadjivassiliou M, Hampe CS, et al. Consensus paper: neuroimmune mechanisms of cerebellar ataxias. Cerebellum. 2016;15:213–32.CrossRefPubMedGoogle Scholar
  7. 7.
    Graus F. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004;75:1135–40.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Anderson NE, Budde-Steffen C, Rosenblum MK, Graus F, Ford D, Synek BJ, et al. Opsoclonus, myoclonus, ataxia, and encephalopathy in adults with cancer: a distinct paraneoplastic syndrome. Medicine (Baltimore). 1988;67:100–9.CrossRefGoogle Scholar
  9. 9.
    Pradat PF, Uchuya M, Fontaine B, Lubetzki C. Pure cerebellar syndrome associated with anti-Hu antibodies. Rev Neurol (Paris). 1995;151:341–3.Google Scholar
  10. 10.
    Bhatia R, Prabhakar S, Lal V, Khurana D, Das CP. Acute onset paraneoplastic cerebellar degeneration in a patient with small cell lung cancer. Neurol India. 2003;51:244–5.PubMedGoogle Scholar
  11. 11.
    Ypma PF, Wijermans PW, Koppen H. Paraneoplastic cerebellar degeneration preceding the diagnosis of Hodgkin’s lymphoma. Neth J Med. 2006;64:5.Google Scholar
  12. 12.
    Bonakis A, Papageorgiou SG, Mandellos D, Galani E, Kalfakis N. Acute onset paraneoplastic cerebellar degeneration. J Neuro-Oncol. 2007;84:329–30.CrossRefGoogle Scholar
  13. 13.
    Hauspy J, Nevin A, Harley I, Mason W, Quirt I, Ghazarian D, et al. Paraneoplastic syndrome in vaginal melanoma: a case report and review of the literature. Int J Gynecol Cancer. 2007;17:1159–63.CrossRefPubMedGoogle Scholar
  14. 14.
    Ammar H, Brown SH, Malani A, Sheth HK, Sollars EG, Zhou SX, et al. A case of paraneoplastic cerebellar ataxia secondary to renal cell carcinoma. South Med J. 2008;101:556–7.CrossRefPubMedGoogle Scholar
  15. 15.
    Park JE, Liang T-W. Reversible cerebellar ataxia due to ovarian teratoma. J Clin Neurosci. 2014;21:1467–9.CrossRefPubMedGoogle Scholar
  16. 16.
    Pavolucci L, Giannini G, Giannoccaro MP, Foschini MP, Lang B, Avoni P, et al. Paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenia in a patient with merkel cell carcinoma and voltage-gated calcium channel antibodies: short reports. Muscle Nerve. 2017;56:998–1000.CrossRefPubMedGoogle Scholar
  17. 17.
    Enríquez-Marulanda A, Beltrán-Osorio LD, Escobar LA, Granados AM, Velásquez-Lasprilla F, Orozco JL. Anti-Yo-associated paraneoplastic cerebellar degeneration manifesting as acute cerebellitis with posterior cranial fossa hypertension. World Neurosurg. 2018;112:117–22.CrossRefPubMedGoogle Scholar
  18. 18.
    Vernino S. Paraneoplastic cerebellar degeneration. Handb Clin Neurol. 2012;103:215–23.CrossRefPubMedGoogle Scholar
  19. 19.
    Yshii LM, Gebauer CM, Pignolet B, Mauré E, Quériault C, Pierau M, et al. CTLA4 blockade elicits paraneoplastic neurological disease in a mouse model. Brain. 2016;139:2923–34.CrossRefPubMedGoogle Scholar
  20. 20.
    Panja D, Vedeler CA, Schubert M. Paraneoplastic cerebellar degeneration: Yo antibody alters mitochondrial calcium buffering capacity. Neuropathol Appl Neurobiol. 2019;45:141–56.CrossRefPubMedGoogle Scholar
  21. 21.
    Vogrig A, Pauletto G, Belgrado E, Pegolo E, Di Loreto C, Rogemond V, et al. Effect of thymectomy on refractory autoimmune status epilepticus. J Neuroimmunol. 2018;317:90–4.CrossRefPubMedGoogle Scholar
  22. 22.
    Kato N, Hashida G, Konaka K. Rehabilitation for a patient with anti-Yo antibody-positive paraneoplastic cerebellar degeneration caused by breast cancer: a case report and literature review. Medicine. 2017;96:e8468.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Navi BB, Reiner AS, Kamel H, Iadecola C, Elkind MS, Panageas KS, et al. Association between incident cancer and subsequent stroke. Ann Neurol. 2015;77:291–300.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Mehndiratta M, Pandey S, Nayak R, Alam A. Posterior circulation ischemic stroke-clinical characteristics, risk factors, and subtypes in a north Indian population: a prospective study. Neurohospitalist. 2012;2:46–50.CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Peterson K, Rosenblum MK, Kotanides H, Posner JB. Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients. Neurology. 1992;42:1931–7.CrossRefPubMedGoogle Scholar
  26. 26.
    Shams'ili S, Grefkens J, de Leeuw B, van den Bent M, Hooijkaas H, van der Holt B, et al. Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. Brain. 2003;126:1409–18.CrossRefPubMedGoogle Scholar
  27. 27.
    Gilmore CP, Elliott I, Auer D, Maddison P. Diffuse cerebellar MR imaging changes in anti-Yo positive paraneoplastic cerebellar degeneration. J Neurol. 2010;257:490–1.CrossRefPubMedGoogle Scholar

Copyright information

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

Authors and Affiliations

  • Alberto Vogrig
    • 1
    • 2
    • 3
    • 4
    Email author
  • Andrea Bernardini
    • 5
  • Gian Luigi Gigli
    • 1
    • 5
    • 6
  • Elisa Corazza
    • 5
  • Alessandro Marini
    • 5
  • Samantha Segatti
    • 5
  • Martina Fabris
    • 7
  • Jérôme Honnorat
    • 2
    • 3
    • 4
  • Mariarosaria Valente
    • 1
    • 5
  1. 1.Clinical Neurology UnitSanta Maria della Misericordia University HospitalUdineItaly
  2. 2.French Reference Center on Paraneoplastic Neurological Syndrome and Autoimmune Encephalitis, Hospital for Neurology and Neurosurgery Pierre WertheimerLyon University HospitalLyonFrance
  3. 3.SynatAc Team, NeuroMyoGene InstituteLyonFrance
  4. 4.University Claude Bernard Lyon 1, University of LyonLyonFrance
  5. 5.Neurology Unit, Department of Medicine (DAME)University of Udine Medical SchoolUdineItaly
  6. 6.Department of Mathematics, Informatics and Physics (DMIF)University of UdineUdineItaly
  7. 7.Institute of Clinical Pathology, Department of Laboratory MedicineSanta Maria della Misericordia University HospitalUdineItaly

Personalised recommendations