A 25-year-old female presented to a university neurology clinic with a 1-month history of progressive ataxia, downbeat nystagmus and spastic tetraparesis. Personal history revealed polyarthralgias and weight loss. Family history was negative. Following thorough history, laboratory, neurophysiological and MRI investigations, a diagnosis of cerebellar ataxia due to coeliac disease was done. The patient was treated with strict gluten-free diet and intravenous administration of immunoglobulins. Although there are many controversies about neurological manifestations of coeliac disease, this case pointed to strong association between these two disorders. The findings of elevated protein content in the cerebrospinal fluid with positive oligoclonal bands suggested an immune-mediated process, further supported by positive anti-endomysium antibodies and anti-transglutaminase antibodies in the cerebrospinal fluid.
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Stahl JS, Averbuch-Heller L, Leigh RJ (2000) Acquired nystagmus. Arch Ophthalmol 118:544–549
Dieterich M (2007) Central vestibular disorders. J Neurol 254:559–568
Hojsak I, Mišak Z, Kolaček S (2005) Coeliac disease today: the search for complications and associated disorders. Paediatr Croat 49:79–84
Grossman G (2008) Neurological complications of coeliac disease: what is the evidence? Pract Neurol 8:77–89
Hadjivassiliou M, Grünewald RA, Chattopadhyay AK, Davies-Jones GA, Gibson A, Jarratt JA, Kandler RH, Lobo A, Powell T, Smith CM (1998) Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet 352:1582–1585
Hadjivassiliou M, Boscolo S, Davies-Jones GA, Grünewald RA, Not T, Sanders DS, Simpson JE, Tongiorgi E, Williamson CA, Woodroofe NM (2002) The humoral response in the pathogenesis of gluten ataxia. Neurology 58:1221–1226
Abele M, Schöls L, Schwartz S, Klockgether T (2003) Prevalence of antigliadin antibodies in ataxia patients. Neurology 60:1674–1675
Ihara M, Makino F, Sawada H, Mezaki T, Mizutani K, Nakase H, Matsui M, Tomimoto H, Shimohama S (2006) Gluten sensitivity in Japanese patients with adult-onset cerebellar ataxia. Intern Med 45:135–140
Briani C, Zara G, Toffanin E, Ruggero S, Ferrarini A, De Lazzari F, Luca M, Faggian D, Grassivaro F, Ermani M, Pezzani R, Giometto B, D’Odorico A (2005) Neurological complications of celiac disease and autoimmune mechanisms: preliminary data of a prospective study in adult patients. Ann NY Acad Sci 1051:148–155
Cakir D, Tosun A, Polat M, Celebisoy N, Gokben S, Aydogdu S, Yagci RV, Tekgul H (2007) Subclinical neurological abnormalities in children with celiac disease receiving a gluten-free diet. J Pediatr Gastroenterol Nutr 45:366–369
Bürk K, Bösch S, Müller CA, Melms A, Zühlke C, Stern M, Besenthal I, Skalej M, Ruck P, Ferber S, Klockgether T, Dichgans J (2001) Sporadic cerebellar ataxia associated with gluten sensitivity. Brain 124:1013–1019
Pengiran Tengah DS, Wills AJ, Holmes GK (2002) Neurological complications of coeliac disease. Postgrad Med J 78:393–398
Schrödl D, Kahlenberg F, Peter-Zimmer K, Hermann W, Kühn HJ, Mothes T (2004) Intrathecal synthesis of autoantibodies against tissue transglutaminase. J Autoimmun 22:335–340
Schöls L, Bauer P, Schmidt T, Schulte T, Riess O (2004) Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol 3:291–304
Fogel BL, Perlman S (2007) Clinical features and molecular genetics of autosomal recessive cerebellar ataxias. Lancet Neurol 6:245–257
Bhidayasiri R, Perlman SL, Pulst SM, Geschwind DH (2005) Late-onset Friedreich ataxia: phenotypic analysis, magnetic resonance imaging findings, and review of the literature. Arch Neurol 62:1865–1869
Panegyres PK (2008) Diagnosis and management of Whipple’s disease of the brain. Pract Neurol 8:311–317
Gerard A, Sarrot-Reynauld F, Liozon E, Cathebras P, Besson G, Robin C, Vighetto A, Mosnier JF, Durieu I, Vital Durand D, Rousset H (2002) Neurologic presentation of Whipple disease: report of 12 cases and review of the literature. Medicine (Baltimore) 81:443–457
Sporis D, Habek M, Mubrin Z, Poljaković Z, Hajnsek S, Bence-Zigman Z (2007) Psychosis and EEG abnormalities as manifestations of Hashimoto encephalopathy. Cogn Behav Neurol 20:138–140
Ch’ng CL, Jones MK, Kingham JG (2007) Celiac disease and autoimmune thyroid disease. Clin Med Res 5:184–192
Sanz Y (2009) Novel perspectives in celiac disease therapy. Mini Rev Med Chem 9:359–367
Sander HW, Magda P, Chin RL, Wu A, Brannagan TH 3rd, Green PH, Latov N (2003) Cerebellar ataxia and coeliac disease. Lancet 362:1548
Souayah N, Chin RL, Brannagan TH, Latov N, Green PH, Kokoszka A, Sander HW (2008) Effect of intravenous immunoglobulin on cerebellar ataxia and neuropathic pain associated with celiac disease. Eur J Neurol 15:1300–1303
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Habek, M., Hojsak, I., Barun, B. et al. Downbeat nystagmus, ataxia and spastic tetraparesis due to coeliac disease. Neurol Sci 32, 911–914 (2011). https://doi.org/10.1007/s10072-011-0506-7
- Coeliac disease
- Immunoglobulin A anti-endomysium antibodies
- Immunoglobulin A anti-transglutaminase antibodies
- Cerebrospinal fluid