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. 1999 Jan;66(1):32–35. doi: 10.1136/jnnp.66.1.32

Idiopathic cerebellar ataxia associated with celiac disease: lack of distinctive neurological features

M T Pellecchia 1, R Scala 1, A Filla 1, G De Michele 1, C Ciacci 1, P Barone 1
PMCID: PMC1736152  PMID: 9886447

Abstract

OBJECTIVES—To determine the occurrence of celiac disease in a population of ataxic patients without definite diagnosis and to characterise distinctive features which may help to differentiate cerebellar ataxia with and without celiac disease.
METHODS—Twenty four ataxic patients without definite diagnosis (group A) and 23 ataxic patients with definite diagnosis (group B) were screened for antigliadin (AGAs) and antiendomysium antibodies (EMAs). Patients with a positive AGA or EMA test underwent endoscopic biopsy of the duodenal mucosa.
RESULTS—There was an increased prevalence of celiac disease in group A (3/24) compared with group B (0/23). None of the celiac patients presented gastrointestinal symptoms or malabsorption signs. None of the ataxic patients with celiac disease had early onset ataxia.
CONCLUSIONS—Celiac disease is associated with ataxic syndromes without definite diagnosis, suggesting that it plays a part in the pathogenesis of some ataxic syndromes. The absence of distinctive neurological features in ataxic patients with celiac disease suggests that a search should be made for celiac disease markers in all ataxic patients without definite diagnosis.



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Selected References

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  1. Ackerman Z., Eliashiv S., Reches A., Zimmerman J. Neurological manifestations in celiac disease and vitamin E deficiency. J Clin Gastroenterol. 1989 Oct;11(5):603–605. [PubMed] [Google Scholar]
  2. Battisti C., Dotti M. T., Formichi P., Bonuccelli U., Malandrini A., Carrai M., Tripodi S. A., Federico A. Disappearance of skin lipofuscin storage and marked clinical improvement in adult onset coeliac disease and severe vitamin E deficiency after chronic vitamin E megatherapy. J Submicrosc Cytol Pathol. 1996 Jul;28(3):339–344. [PubMed] [Google Scholar]
  3. Behse F., Buchthal F. Normal sensory conduction in the nerves of the leg in man. J Neurol Neurosurg Psychiatry. 1971 Aug;34(4):404–414. doi: 10.1136/jnnp.34.4.404. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Bhatia K. P., Brown P., Gregory R., Lennox G. G., Manji H., Thompson P. D., Ellison D. W., Marsden C. D. Progressive myoclonic ataxia associated with coeliac disease. The myoclonus is of cortical origin, but the pathology is in the cerebellum. Brain. 1995 Oct;118(Pt 5):1087–1093. doi: 10.1093/brain/118.5.1087. [DOI] [PubMed] [Google Scholar]
  5. Catassi C., Fabiani E., Rätsch I. M., Coppa G. V., Giorgi P. L., Pierdomenico R., Alessandrini S., Iwanejko G., Domenici R., Mei E. The coeliac iceberg in Italy. A multicentre antigliadin antibodies screening for coeliac disease in school-age subjects. Acta Paediatr Suppl. 1996 May;412:29–35. doi: 10.1111/j.1651-2227.1996.tb14244.x. [DOI] [PubMed] [Google Scholar]
  6. Catassi C., Rätsch I. M., Fabiani E., Rossini M., Bordicchia F., Candela F., Coppa G. V., Giorgi P. L. Coeliac disease in the year 2000: exploring the iceberg. Lancet. 1994 Jan 22;343(8891):200–203. doi: 10.1016/s0140-6736(94)90989-x. [DOI] [PubMed] [Google Scholar]
  7. Chinnery P. F., Reading P. J., Milne D., Gardner-Medwin D., Turnbull D. M. CSF antigliadin antibodies and the Ramsay Hunt syndrome. Neurology. 1997 Oct;49(4):1131–1133. doi: 10.1212/wnl.49.4.1131. [DOI] [PubMed] [Google Scholar]
  8. Collin P., Pirttilä T., Nurmikko T., Somer H., Erilä T., Keyriläinen O. Celiac disease, brain atrophy, and dementia. Neurology. 1991 Mar;41(3):372–375. doi: 10.1212/wnl.41.3.372. [DOI] [PubMed] [Google Scholar]
  9. Cooke W. T., Smith W. T. Neurological disorders associated with adult coeliac disease. Brain. 1966 Dec;89(4):683–722. doi: 10.1093/brain/89.4.683. [DOI] [PubMed] [Google Scholar]
  10. Filla A., De Michele G., Cavalcanti F., Pianese L., Monticelli A., Campanella G., Cocozza S. The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia. Am J Hum Genet. 1996 Sep;59(3):554–560. [PMC free article] [PubMed] [Google Scholar]
  11. Finelli P. F., McEntee W. J., Ambler M., Kestenbaum D. Adult celiac disease presenting as cerebellar syndrome. Neurology. 1980 Mar;30(3):245–249. doi: 10.1212/wnl.30.3.245. [DOI] [PubMed] [Google Scholar]
  12. Ghezzi A., Filippi M., Falini A., Zaffaroni M. Cerebral involvement in celiac disease: a serial MRI study in a patient with brainstem and cerebellar symptoms. Neurology. 1997 Nov;49(5):1447–1450. doi: 10.1212/wnl.49.5.1447. [DOI] [PubMed] [Google Scholar]
  13. Gobbi G., Bouquet F., Greco L., Lambertini A., Tassinari C. A., Ventura A., Zaniboni M. G. Coeliac disease, epilepsy, and cerebral calcifications. The Italian Working Group on Coeliac Disease and Epilepsy. Lancet. 1992 Aug 22;340(8817):439–443. doi: 10.1016/0140-6736(92)91766-2. [DOI] [PubMed] [Google Scholar]
  14. Hadjivassiliou M., Chattopadhyay A. K., Davies-Jones G. A., Gibson A., Grünewald R. A., Lobo A. J. Neuromuscular disorder as a presenting feature of coeliac disease. J Neurol Neurosurg Psychiatry. 1997 Dec;63(6):770–775. doi: 10.1136/jnnp.63.6.770. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Hadjivassiliou M., Gibson A., Davies-Jones G. A., Lobo A. J., Stephenson T. J., Milford-Ward A. Does cryptic gluten sensitivity play a part in neurological illness? Lancet. 1996 Feb 10;347(8998):369–371. doi: 10.1016/s0140-6736(96)90540-1. [DOI] [PubMed] [Google Scholar]
  16. Hall W. H. Proximal muscle atrophy in adult celiac disease. Am J Dig Dis. 1968 Aug;13(8):697–704. doi: 10.1007/BF02232993. [DOI] [PubMed] [Google Scholar]
  17. Halsted C. The many faces of celiac disease. N Engl J Med. 1996 May 2;334(18):1190–1191. doi: 10.1056/NEJM199605023341811. [DOI] [PubMed] [Google Scholar]
  18. Hermaszewski R. A., Rigby S., Dalgleish A. G. Coeliac disease presenting with cerebellar degeneration. Postgrad Med J. 1991 Nov;67(793):1023–1024. doi: 10.1136/pgmj.67.793.1023. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Johnston S. D., Watson R. G., McMillan S. A., McMaster D., Evans A. Preliminary results from follow-up of a large-scale population survey of antibodies to gliadin, reticulin and endomysium. Acta Paediatr Suppl. 1996 May;412:61–64. doi: 10.1111/j.1651-2227.1996.tb14255.x. [DOI] [PubMed] [Google Scholar]
  20. Kaplan J. G., Pack D., Horoupian D., DeSouza T., Brin M., Schaumburg H. Distal axonopathy associated with chronic gluten enteropathy: a treatable disorder. Neurology. 1988 Apr;38(4):642–645. doi: 10.1212/wnl.38.4.642. [DOI] [PubMed] [Google Scholar]
  21. Kelkar P., Ross M. A., Murray J. Mononeuropathy multiplex associated with celiac sprue. Muscle Nerve. 1996 Feb;19(2):234–236. doi: 10.1002/(SICI)1097-4598(199602)19:2<234::AID-MUS19>3.0.CO;2-U. [DOI] [PubMed] [Google Scholar]
  22. Kepes J. J., Chou S. M., Price L. W., Jr Progressive multifocal leukoencephalopathy with 10-year survival in a patient with nontropical sprue. Report of a case with unusual light and electron microscopic features. Neurology. 1975 Nov;25(11):1006–1012. doi: 10.1212/wnl.25.11.1007. [DOI] [PubMed] [Google Scholar]
  23. Kristoferitsch W., Pointner H. Progressive cerebellar syndrome in adult coeliac disease. J Neurol. 1987 Feb;234(2):116–118. doi: 10.1007/BF00314115. [DOI] [PubMed] [Google Scholar]
  24. Lu C. S., Thompson P. D., Quinn N. P., Parkes J. D., Marsden C. D. Ramsay Hunt syndrome and coeliac disease: a new association? Mov Disord. 1986;1(3):209–219. doi: 10.1002/mds.870010306. [DOI] [PubMed] [Google Scholar]
  25. Marsh M. N. Gluten, major histocompatibility complex, and the small intestine. A molecular and immunobiologic approach to the spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology. 1992 Jan;102(1):330–354. [PubMed] [Google Scholar]
  26. Mauro A., Orsi L., Mortara P., Costa P., Schiffer D. Cerebellar syndrome in adult celiac disease with vitamin E deficiency. Acta Neurol Scand. 1991 Aug;84(2):167–170. doi: 10.1111/j.1600-0404.1991.tb04927.x. [DOI] [PubMed] [Google Scholar]
  27. Muller A. F., Donnelly M. T., Smith C. M., Grundman M. J., Holmes G. K., Toghill P. J. Neurological complications of celiac disease: a rare but continuing problem. Am J Gastroenterol. 1996 Jul;91(7):1430–1435. [PubMed] [Google Scholar]
  28. Pulst S. M., Nechiporuk A., Nechiporuk T., Gispert S., Chen X. N., Lopes-Cendes I., Pearlman S., Starkman S., Orozco-Diaz G., Lunkes A. Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nat Genet. 1996 Nov;14(3):269–276. doi: 10.1038/ng1196-269. [DOI] [PubMed] [Google Scholar]
  29. Tietge U. J., Schmidt H. H., Manns M. P. Neurological complications in celiac disease. Am J Gastroenterol. 1997 Mar;92(3):540–540. [PubMed] [Google Scholar]
  30. Trier J. S. Celiac sprue. N Engl J Med. 1991 Dec 12;325(24):1709–1719. doi: 10.1056/NEJM199112123252406. [DOI] [PubMed] [Google Scholar]
  31. Ward M. E., Murphy J. T., Greenberg G. R. Celiac disease and spinocerebellar degeneration with normal vitamin E status. Neurology. 1985 Aug;35(8):1199–1201. doi: 10.1212/wnl.35.8.1199. [DOI] [PubMed] [Google Scholar]

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