Journal of Neurology

, 257:59 | Cite as

Antineuronal antibodies in sporadic late-onset cerebellar ataxia

  • K. BürkEmail author
  • M. Wick
  • G. Roth
  • P. Decker
  • R. Voltz
Original Communication


Sporadic late-onset cerebellar ataxia of unknown cause is considered a neurodegenerative disorder whose underlying mechanisms are still unknown. To identify antineuronal autoantibodies, immunohistochemical and immunoblotting techniques were performed in 67 patients with sporadic cerebellar degeneration of unknown cause. Elevated P/Q-type voltage-gated calcium channel (VGCC)-specific antibodies were found in eight patients (11.9%). There was no hint of a paraneoplastic disorder in any of the patients. The present findings suggest an autoimmune contribution to the pathophysiology of a subgroup of sporadic late-onset cerebellar ataxia.


Voltage-gated calcium channels VGCC Idiopathic late onset cerebellar ataxia Multiple system atrophy Paraneoplastic cerebellar degeneration 



This study was supported by the Deutsche Forschungsgemeinschaft DFG (project D7, SFB571 Autoimmunität, RV) and the Herrmann and Lilly Schilling Foundation. We thank Prof. Dr. Hans-Georg Rammensee for critical reading of the manuscript.

Conflict of interest statement



  1. 1.
    Harding AE (1981) “Idiopathic” late onset cerebellar ataxia. A clinical and genetic study of 36 cases. J Neurol Sci 51(2):259–271CrossRefPubMedGoogle Scholar
  2. 2.
    Dalmau JO, Rosenfeld MR (2009) Paraneoplastic syndromes of the CNS. Lancet Neurol 7(4):327–340CrossRefGoogle Scholar
  3. 3.
    Voltz R, Gultekin SH, Rosenfeld MR, Gerstner E, Eichen J, Posner JB et al (1999) A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med 340(23):1788–1795CrossRefPubMedGoogle Scholar
  4. 4.
    Lennon VA, Kryzer TJ, Griesmann GE, O’Suilleabhain PE, Windebank AJ, Woppmann A et al (1995) Calcium-channel antibodies in the Lambert–Eaton syndrome and other paraneoplastic syndromes. N Engl J Med 332(22):1467–1474CrossRefPubMedGoogle Scholar
  5. 5.
    Motomura M, Johnston I, Lang B, Vincent A, Newsom-Davis J (1995) An improved diagnostic assay for Lambert–Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry 58(1):85–87CrossRefPubMedGoogle Scholar
  6. 6.
    Gilman S, Low PA, Quinn N, Albanese A, Ben Shlomo Y, Fowler CJ et al (1999) Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 163(1):94–98CrossRefPubMedGoogle Scholar
  7. 7.
    Burk K, Melms A, Schulz JB, Dichgans J (2001) Effectiveness of intravenous immunoglobin therapy in cerebellar ataxia associated with gluten sensitivity. Ann Neurol 50(6):827–828CrossRefPubMedGoogle Scholar
  8. 8.
    Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, Davies-Jones GA, Gibson A, Jarratt JA et al (1998) Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet 352(9140):1582–1585CrossRefPubMedGoogle Scholar
  9. 9.
    Candler PM, Hart PE, Barnett M, Weil R, Rees JH (2004) A follow up study of patients with paraneoplastic neurological disease in the UK. J Neurol Neurosurg Psychiatry 75(10):1411–1415CrossRefPubMedGoogle Scholar
  10. 10.
    Wullner U, Klockgether T, Petersen D, Naegele T, Dichgans J (1993) Magnetic resonance imaging in hereditary and idiopathic ataxia. Neurology 43(2):318–325PubMedGoogle Scholar
  11. 11.
    Darnell RB, Posner JB (2003) Paraneoplastic syndromes involving the nervous system. N Engl J Med 349(16):1543–1554CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • K. Bürk
    • 1
    • 2
    • 3
    Email author
  • M. Wick
    • 4
  • G. Roth
    • 5
  • P. Decker
    • 1
  • R. Voltz
    • 6
    • 7
  1. 1.Institute for Cell Biology, Department of ImmunologyUniversity of TübingenTübingenGermany
  2. 2.Institute of Brain ResearchUniversity of TübingenTübingenGermany
  3. 3.Department of NeurologyUniversity of MarburgMarburgGermany
  4. 4.Department of Laboratory MedicineUniversity of MunichMunichGermany
  5. 5.Department of Microsystems EngineeringUniversity of FreiburgFreiburgGermany
  6. 6.Department of NeuroimmunologyUniversity of MunichMunichGermany
  7. 7.Department of Palliative MedicineUniversity of CologneCologneGermany

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