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The Cerebellum

, Volume 18, Issue 6, pp 1137–1142 | Cite as

Neurochondrin Antibody Serum Positivity in Three Cases of Autoimmune Cerebellar Ataxia

  • Zhang Weihua
  • Ren Haitao
  • Fang Fang
  • Yang Xunzhe
  • Wang Jing
  • Guan HongzhiEmail author
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Abstract

To report three cases of autoimmune ataxia patients with positive neurochondrin (NCDN) antibodies. Patients with unknown cerebellar ataxia were screened for autoimmune cerebellar ataxia (ACA)-related antibodies, including glutamic acid decarboxylase 65 (GAD65), delta/notch-like epidermal growth factor-related receptor (Tr/DNER), zinc finger protein 4 (ZIC4), inositol 1,4,5-triphosphate receptor 1 (ITPR1), Homer protein homologue 3 (Homer-3), neurochondrin (NCDN), Purkinje cell antibody 2 (PCA-2) and carbonic anhydrase-related protein VII (CARPVII). The antibodies were assessed by indirect immunofluorescence using transfected cells (cell-based assay, CBA) and monkey cerebellum (tissue-based assay, TBA) with the multi-antigen co-plate biochip mosaic technique. Patients with positive antibodies received immunotherapy and were followed up in the clinic. Clinical characteristics, laboratory data, and outcomes of antibody-positive patients were described, analysed and compared with previously reported cases. The NCDN antibody was positive in three male patients in whom the onset ages were four years and 11 months, two years and seven months and 67 years old. Serum antibody titres were 1:32, 1:100 and 1:320. Cerebral ataxia was the most prominent presentation. Cerebellar atrophy was found in one of the patients. Immunotherapy was effective in all three patients. The NCDN antibody is associated with autoimmune ataxia, and it has been suggested that the NCDN antibody should be tested in patients with cerebellar ataxia who are negative for routine ACA antibodies. Early immunotherapy may have a beneficial impact on prognosis.

Keywords

Neurochondrin NCDN Autoimmune Cerebellar Ataxia 
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Notes

Acknowledgments

The authors would like to thank Dr. Ren Xiaotun, Ding Changhong, Hang Tongli and Feng Weixing for the helpful discussions.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

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

Authors and Affiliations

  1. 1.Department of NeurologyBeijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthBeijingChina
  2. 2.Department of NeurologyPeking Union Medical College HospitalBeijingChina
  3. 3.Institute of PsychologyCAS Key Laboratory of Mental HealthBeijingChina
  4. 4.Department of PsychologyUniversity of Chinese Academy of SciencesBeijingChina

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