The Cerebellum

, Volume 16, Issue 4, pp 868–871 | Cite as

Low-Titre GAD Antibody-Associated Late-Onset Cerebellar Ataxia with a Significant Clinical Response to Intravenous Immunoglobulin Treatment

  • Timotej PetrijanEmail author
  • Marija Menih
Case Report


Antiglutamic acid decarboxylase antibody-associated cerebellar ataxia (GAD-Abs CA) is a rare, but increasingly detected, autoimmune neurological disorder characterized by the clinical presence of a cerebellar syndrome concomitant with positive GAD-Abs levels in serum and cerebrospinal fluid (CSF). It represents 3% of all immune-mediated sporadic CAs. Low-titre GAD-Abs CA is an even rarer subtype of GAD-Abs CA. We report on a 68-year-old woman with a 3-year history of progressive gait ataxia. In addition to the modified Rankin Scale (mRS), we used two other objective scales to evaluate CA severity, i.e. the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for Assessment and Rating of Ataxia (SARA). Series of CT and MRI showed atrophy of the cerebellum. Except for the glycated haemoglobin (HbA1c) levels, all other routine laboratory examinations were within normal limits. Autoimmune laboratory examinations showed positive (25.8 U/mL) serum GAD-Abs levels. The GAD antibody index was <1.0. The CSF analysis showed no oligoclonal immunoglobulin bands. Intravenous immunoglobulin (IVIg) therapy was started and significant improvement was observed. The diagnosis of low-titre GAD-Abs CA was established.


Anti-GAD Autoantibodies Autoimmunity Late-onset cerebellar ataxia Low-titre GAD-Abs CA 


Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Fogel BL, Perlman S. An approach to the patient with late-onset cerebellar ataxia. Nat Clin Pract Neurol. 2006;2(11):629–35.CrossRefPubMedGoogle Scholar
  2. 2.
    Mitoma H, Hadjivassiliou M, Honnorat J. Guidelines for treatment of immune-mediated cerebellar ataxias. Cerebellum & Ataxias. 2015;2:14.CrossRefGoogle Scholar
  3. 3.
    Hadjivassiliou M, Boscolo S, Tongiorgi E, Grunewald RA, Sharrack B, Sanders DS, et al. Cerebellar ataxia as a possible organ specific autoimmune disease. Movement Disord. 2008;23:1270–377.CrossRefGoogle Scholar
  4. 4.
    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
  5. 5.
    Saiz A, Blanco Y, Sabater L, González F, Bataller L, Casamitjana R, et al. Spectrum of neurological syndromes associated with glutamic acid decarboxylase antibodies: diagnostic clues for this association. Brain. 2008;131:2553–63.CrossRefPubMedGoogle Scholar
  6. 6.
    Graus F, Saiz A, Dalmau J. Antibodies and neuronal autoimmune disorders of the CNS. J Neurol. 2010;257:509–17.CrossRefPubMedGoogle Scholar
  7. 7.
    Ariño H, Gresa Arribas N, Blanco Y, Martínez Hernández E, Sabater L, Petit Pedrol M, et al. Cerebellar ataxia and glutamic acid decarboxylase antibodies: immunologic profile and long-term effect of immunotherapy. JAMA Neurol. 2014;71:1009–16.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Nanri K, Niwa H, Mitoma H, Takei A, Ikeda J, Harada T, et al. Low-titer anti-GAD-antibody-positive cerebellar ataxia. Cerebellum. 2013;12:171–5.CrossRefPubMedGoogle Scholar
  9. 9.
    Honnorat J, Saiz A, Giometto B, Vincent A, Brieva L, Andres C, et al. Cerebellar ataxia with anti-glutamic acid decarboxylase antibodies: study of 14 patients. Arch Neurol. 2001;58:225–30.CrossRefPubMedGoogle Scholar
  10. 10.
    Leypoldt F, Wandinger KP. Paraneoplastic neurological syndromes. Clin Exp Immunol. 2014;175:336–48.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Planche V, Marques A, Ulla M, Ruivard M, Durif F. Intravenous immunoglobulin and rituximab for cerebellar ataxia with glutamic acid decarboxylase autoantibodies. Cerebellum. 2013;13(3):318–22.CrossRefGoogle Scholar
  12. 12.
    Nanri K, Okita M, Takeguchi M, Taquchi T, Ishiko T, Saito H, et al. Intravenous immunoglobulin therapy for autoantibody-positive cerebellar ataxia. Intern Med. 2009;48(10):783–90.CrossRefPubMedGoogle Scholar
  13. 13.
    Lauria G, Pareyson D, Pitzolu MG, Bazzigaluppi E. Excellent response to steroid treatment in anti-GAD cerebellar ataxia. Lancet Neurol. 2003;10:634–5.CrossRefGoogle Scholar
  14. 14.
    Bonnan M, Cabre P, Olindo S, Signate A, Saint-Vil M, Smadja D. Steroid treatment in four cases of anti-GAD cerebellar ataxia. Rev Neurol. 2008;164(5):427–33.CrossRefPubMedGoogle Scholar
  15. 15.
    Bayreuther C, Hieronimus S, Ferrari P, Thomas P, Lebrun C. Auto-immune cerebellar ataxia with anti-GAD antibodies accompanied by de novo late-onset type 1 diabetes mellitus. Diabetes Metab. 2008;34(4, pt 1):386–8.CrossRefPubMedGoogle Scholar
  16. 16.
    Vulliemoz S, Vanini G, Truffert A, Chizzolini C, Seeck M. Epilepsy and cerebellar ataxia associated with anti-glutamic acid decarboxylase antibodies. J Neurol Neurosurg Psychiatry. 2007;78(2):187–9.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Abele M, Weller M, Mescheriakov S, Bürk K, Dichgans J, Klockgether T. Cerebellar ataxia with glutamic acid decarboxylase autoantibodies. Neurology. 1999;52(4):857–9.CrossRefPubMedGoogle Scholar
  18. 18.
    McFarland NR, Login IS, Vernon S, Burns TM. Improvement with corticosteroids and azathioprine in GAD65-associated cerebellar ataxia. Neurology. 2006;67(7):1308–9.CrossRefPubMedGoogle Scholar
  19. 19.
    Virgilio R, Corti S, Agazzi P, Santoro D, Lanfranconi S, Candelise L, et al. Effect of steroid treatment in cerebellar ataxia associated with anti-glutamic acid decarboxylase antibodies. J Neurol Neurosurg Psychiatry. 2009;80(1):95–6.CrossRefPubMedGoogle Scholar
  20. 20.
    Pedroso JL, Braga Neto P, Dutra LA, Barsottini OG. Cerebellar ataxia associated to anti-glutamic acid decarboxylase autoantibody (anti-GAD): partial improvement with intravenous immunoglobulin therapy. Arq Neuropsiquiatr. 2011;69(6):993.CrossRefPubMedGoogle Scholar
  21. 21.
    Nociti V, Frisullo G, Tartaglione T, Pantanella AK, Iorio R, Tonali PA, et al. Refractory generalized seizures and cerebellar ataxia associated with anti-GAD antibodies responsive to immunosuppressive treatment. Eur J Neurol. 2010;17(1):e5.CrossRefPubMedGoogle Scholar

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© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Department of NeurologyUniversity Medical Centre MariborMariborSlovenia

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