Abstract
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.
References
Harding AE (1981) “Idiopathic” late onset cerebellar ataxia. A clinical and genetic study of 36 cases. J Neurol Sci 51(2):259–271
Dalmau JO, Rosenfeld MR (2009) Paraneoplastic syndromes of the CNS. Lancet Neurol 7(4):327–340
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):1788–1795
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):1467–1474
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):85–87
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):94–98
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):827–828
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):1582–1585
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):1411–1415
Wullner U, Klockgether T, Petersen D, Naegele T, Dichgans J (1993) Magnetic resonance imaging in hereditary and idiopathic ataxia. Neurology 43(2):318–325
Darnell RB, Posner JB (2003) Paraneoplastic syndromes involving the nervous system. N Engl J Med 349(16):1543–1554
Acknowledgments
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.
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Bürk, K., Wick, M., Roth, G. et al. Antineuronal antibodies in sporadic late-onset cerebellar ataxia. J Neurol 257, 59–62 (2010). https://doi.org/10.1007/s00415-009-5262-8
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DOI: https://doi.org/10.1007/s00415-009-5262-8