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Cerebellar disease associated with anti-glutamic acid decarboxylase antibodies: review

  • Neurology and Preclinical Neurological Studies - Review Article
  • Published:
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Abstract

Several neurological syndromes have been recognized associated to GAD antibodies. Among those disorders, cerebellar ataxia (CA) is one of the most common, along with stiff-person syndrome. Patients with GAD associated CA present with a progressive pancerebellar syndrome, with a subacute or chronic evolution, along with other neurological manifestations such as stiffness, oculomotor dysfunction, epilepsy, and cognitive dysfunction. These symptoms may be preceded by the so-called “brainstem attacks”, where manifestations consistent with transient dysfunction of the brainstem may be observed. These patients frequently have extra-neurologic autoimmune manifestations such as diabetes mellitus type 1, polyendocrine autoimmune syndrome, pernicious anemia, vitiligo, etc. A proportion of patients may present with an underlying neoplasia, but the course is less aggressive than in those patients with classical paraneoplastic CA with onconeural antibodies. The diagnosis is based on the present of high serum and CSF titers of GAD antibodies, with intrathecal production of such antibodies. Treatment is aimed to decrease the immunological response with intravenous immunoglobulin, steroids, rituximab and oral immunosuppressive drugs. A subacute presentation and rapid initiation of immunotherapy seem to be the predictors of a favorable clinical response.

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Acknowledgements

We thank Ian Osuna for his help in Fig. 1.

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Authors and Affiliations

  1. Department of Neurology, Parkinson’s Disease Center and Movement Disorders Clinic, Baylor College of Medicine, Houston, TX, USA

    José Fidel Baizabal-Carvallo

  2. Department of Internal Medicine, University of Guanajuato, 20 de Enero no. 927, C.P. 37320, León, Guanajuato, Mexico

    José Fidel Baizabal-Carvallo

  3. National Polytechnic Institute, Mexico City, Mexico

    Marlene Alonso-Juarez

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  1. José Fidel Baizabal-Carvallo
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  2. Marlene Alonso-Juarez
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Contributions

Dr. Baizabal-Carvallo gathered the data, made the statistical analysis, conceptualized and wrote the first draft of the manuscript. Dr. Alonso-Juarez gathered the data, conceptualized and reviewed the manuscript.

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Correspondence to José Fidel Baizabal-Carvallo.

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The authors declare that they have no competing interests.

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Baizabal-Carvallo, J.F., Alonso-Juarez, M. Cerebellar disease associated with anti-glutamic acid decarboxylase antibodies: review. J Neural Transm 124, 1171–1182 (2017). https://doi.org/10.1007/s00702-017-1754-3

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  • DOI: https://doi.org/10.1007/s00702-017-1754-3

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