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- W2100060114 abstract "Celiac disease (CD) long has been associated with neurologic and psychiatric disorders including cerebellar ataxia, peripheral neuropathy, epilepsy, dementia, and depression. Earlier reports mainly have documented the involvement of the nervous system as a complication of prediagnosed CD. However, more recent studies have emphasized that a wider spectrum of neurologic syndromes may be the presenting extraintestinal manifestation of gluten sensitivity with or without intestinal pathology. These include migraine, encephalopathy, chorea, brain stem dysfunction, myelopathy, mononeuritis multiplex, Guillain-Barré-like syndrome, and neuropathy with positive antiganglioside antibodies. The association between most neurologic syndromes described and gluten sensitivity remains to be confirmed by larger epidemiologic studies. It further has been suggested that gluten sensitivity (as evidenced by high antigliadin antibodies) is a common cause of neurologic syndromes (notably cerebellar ataxia) of otherwise unknown cause. Additional studies showed high prevalence of gluten sensitivity in genetic neurodegenerative disorders such as hereditary spinocerebellar ataxia and Huntington’s disease. It remains unclear whether gluten sensitivity contributes to the pathogenesis of these disorders or whether it represents an epiphenomenon. Studies of gluten-free diet in patients with gluten sensitivity and neurologic syndromes have shown variable results. Diet trials also have been inconclusive in autism and schizophrenia, 2 diseases in which sensitivity to dietary gluten has been implicated. Further studies clearly are needed to assess the efficacy of gluten-free diet and to address the underlying mechanisms of nervous system pathology in gluten sensitivity. Celiac disease (CD) long has been associated with neurologic and psychiatric disorders including cerebellar ataxia, peripheral neuropathy, epilepsy, dementia, and depression. Earlier reports mainly have documented the involvement of the nervous system as a complication of prediagnosed CD. However, more recent studies have emphasized that a wider spectrum of neurologic syndromes may be the presenting extraintestinal manifestation of gluten sensitivity with or without intestinal pathology. These include migraine, encephalopathy, chorea, brain stem dysfunction, myelopathy, mononeuritis multiplex, Guillain-Barré-like syndrome, and neuropathy with positive antiganglioside antibodies. The association between most neurologic syndromes described and gluten sensitivity remains to be confirmed by larger epidemiologic studies. It further has been suggested that gluten sensitivity (as evidenced by high antigliadin antibodies) is a common cause of neurologic syndromes (notably cerebellar ataxia) of otherwise unknown cause. Additional studies showed high prevalence of gluten sensitivity in genetic neurodegenerative disorders such as hereditary spinocerebellar ataxia and Huntington’s disease. It remains unclear whether gluten sensitivity contributes to the pathogenesis of these disorders or whether it represents an epiphenomenon. Studies of gluten-free diet in patients with gluten sensitivity and neurologic syndromes have shown variable results. Diet trials also have been inconclusive in autism and schizophrenia, 2 diseases in which sensitivity to dietary gluten has been implicated. Further studies clearly are needed to assess the efficacy of gluten-free diet and to address the underlying mechanisms of nervous system pathology in gluten sensitivity. Celiac disease (CD) long has been associated with neurologic and psychiatric disorders, notably cerebellar ataxia, peripheral neuropathy, epilepsy, dementia, and depression. A wider spectrum of neurologic syndromes increasingly are recognized both as complications of prediagnosed CD and as initial manifestation of CD.1Luostarinen L. Pirttila T. Collin P. Coeliac disease presenting with neurological disorders.Eur Neurol. 1999; 42: 132-135Crossref PubMed Scopus (121) Google Scholar, 2Zelnik N. Pacht A. Obeid R. Lerner A. Range of neurologic disorders in patients with celiac disease.Pediatrics. 2004; 113: 1672-1676Crossref PubMed Scopus (211) Google Scholar Cooke and Smith3Cooke W. Smith W. Neurological disorders associated with adult coeliac disease.Brain. 1966; 89: 683-722Crossref PubMed Scopus (325) Google Scholar first reported a group of 16 patients with biopsy examination-proven CD who had gait ataxia and peripheral neuropathy. Cerebellar involvement was reported in some of these patients and in subsequent multiple case reports.4Missen G.A. Intestinal malignant lymphoma and cerebellar cortical degeneration complicating idiopathic steatorrhoea report of a case with a review of the literature.Guys Hosp Rep. 1966; 115: 359-385PubMed Google Scholar, 5Finelli P.F. McEntee W.J. Ambler M. Kestenbaum D. Adult celiac disease presenting as cerebellar syndrome.Neurology. 1980; 30: 245-249Crossref PubMed Google Scholar, 6Lu 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: 209-219Crossref PubMed Scopus (57) Google Scholar, 7Kristoferitsch W. Pointner H. Progressive cerebellar syndrome in adult coeliac disease.J Neurol. 1987; 234: 116-118Crossref PubMed Scopus (26) Google Scholar, 8Hermaszewski R.A. Rigby S. Dalgleish A.G. Coeliac disease presenting with cerebellar degeneration.Postgrad Med J. 1991; 67: 1023-1024Crossref PubMed Scopus (33) Google Scholar Although some patients had low vitamin E levels,9Harding A.E. Muller D.P. Thomas P.K. Willison H.J. Spinocerebellar degeneration secondary to chronic intestinal malabsorption a vitamin E deficiency syndrome.Ann Neurol. 1982; 12: 419-424Crossref PubMed Scopus (152) Google Scholar, 10Mauro A. Orsi L. Mortara P. Costa P. Schiffer D. Cerebellar syndrome in adult celiac disease with vitamin E deficiency.Acta Neurol Scand. 1991; 84: 167-170Crossref PubMed Scopus (61) Google Scholar, 11Battisti 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; 28: 339-344PubMed Google Scholar others had normal vitamin levels and the cerebellar syndrome was attributed to CD mechanisms, rather than malabsorption.12Ward M.E. Murphy J.T. Greenberg G.R. Celiac disease and spinocerebellar degeneration with normal vitamin E status.Neurology. 1985; 35: 1199-1201Crossref PubMed Google Scholar, 13Muller 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; 91: 1430-1435PubMed Google Scholar Studies also have shown that cerebellar and other neurologic syndromes may be the presenting manifestation of CD.1Luostarinen L. Pirttila T. Collin P. Coeliac disease presenting with neurological disorders.Eur Neurol. 1999; 42: 132-135Crossref PubMed Scopus (121) Google Scholar, 13Muller 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; 91: 1430-1435PubMed Google Scholar, 14Hadjivassiliou 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; 347: 369-371Abstract PubMed Scopus (300) Google Scholar In one study, 10 of 144 (7%) CD patients presented with neurologic symptoms.1Luostarinen L. Pirttila T. Collin P. Coeliac disease presenting with neurological disorders.Eur Neurol. 1999; 42: 132-135Crossref PubMed Scopus (121) Google Scholar In patients with cerebellar ataxia of unknown cause, biopsy examination-proven CD was found in 16% (4 of 25),14Hadjivassiliou 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; 347: 369-371Abstract PubMed Scopus (300) Google Scholar 16.7% (4 of 24),1Luostarinen L. Pirttila T. Collin P. Coeliac disease presenting with neurological disorders.Eur Neurol. 1999; 42: 132-135Crossref PubMed Scopus (121) Google Scholar 12.5% (3 of 24),15Pellecchia M.T. Scala R. Filla A. De Michele G. Ciacci C. Barone P. Idiopathic cerebellar ataxia associated with celiac disease lack of distinctive neurological features.J Neurol Neurosurg Psychiatry. 1999; 66: 32-35Crossref PubMed Scopus (109) Google Scholar and 1.9% (2 of 104).16Burk K. Bosch S. Muller C.A. Melms A. Zuhlke C. Stern M. Besenthal I. Skalej M. Ruck P. Ferber S. Klockgether T. Dichgans J. Sporadic cerebellar ataxia associated with gluten sensitivity.Brain. 2001; 124: 1013-1019Crossref PubMed Scopus (142) Google Scholar It is accepted widely that patients with ataxia of unknown cause should be screened for CD. However, it remains controversial whether gluten sensitivity (defined as “a state of heightened immunologic responsiveness to ingested gluten in genetically predisposed individuals”17Marsh M.N. The natural history of gluten sensitivity defining, refining and re-defining.QJM. 1995; 88: 9-13PubMed Google Scholar) without intestinal involvement should be considered the cause of cerebellar degeneration in ataxia of otherwise unknown cause.18Wills A.J. Unsworth D.J. The neurology of gluten sensitivity separating the wheat from the chaff.Curr Opin Neurol. 2002; 15: 519-523Crossref PubMed Scopus (33) Google Scholar The diagnosis of gluten sensitivity is made by showing high antigliadin antibodies even in the absence of intestinal pathology and the antibodies that correlate with mucosal damage (antiendomysium and anti-tissue transglutaminase antibodies) and that are considered highly specific for CD.19Volta U. Molinaro N. De Franchis R. Forzenigo L. Landoni M. Fratangelo D. Bianchi F.B. Correlation between IgA antiendomysial antibodies and subtotal villous atrophy in dermatitis herpetiformis.J Clin Gastroenterol. 1992; 14: 298-301Crossref PubMed Scopus (31) Google Scholar The prevalence of positive antigliadin antibodies in ataxia of unknown cause has varied between 0% and 41% in different studies.14Hadjivassiliou 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; 347: 369-371Abstract PubMed Scopus (300) Google Scholar, 15Pellecchia M.T. Scala R. Filla A. De Michele G. Ciacci C. Barone P. Idiopathic cerebellar ataxia associated with celiac disease lack of distinctive neurological features.J Neurol Neurosurg Psychiatry. 1999; 66: 32-35Crossref PubMed Scopus (109) Google Scholar, 16Burk K. Bosch S. Muller C.A. Melms A. Zuhlke C. Stern M. Besenthal I. Skalej M. Ruck P. Ferber S. Klockgether T. Dichgans J. Sporadic cerebellar ataxia associated with gluten sensitivity.Brain. 2001; 124: 1013-1019Crossref PubMed Scopus (142) Google Scholar, 20Combarros O. Infante J. Lopez-Hoyos M. Bartolome M.J. Berciano J. Corral J. Volpini V. Celiac disease and idiopathic cerebellar ataxia.Neurology. 2000; 54: 2346Crossref PubMed Scopus (35) Google Scholar, 21Bushara K.O. Goebel S.U. Shill H. Goldfarb L.G. Hallett M. Gluten sensitivity in sporadic and hereditary cerebellar ataxia.Ann Neurol. 2001; 49: 540-543Crossref PubMed Scopus (112) Google Scholar, 22Hadjivassiliou M. Grunewald R.A. Davies-Jones G.A. Causes of cerebellar degeneration gluten ataxia in perspective.J Neurol Sci. 2001; 187: S520Google Scholar, 23Abele M. Schols L. Schwartz S. Klockgether T. Prevalence of antigliadin antibodies in ataxia patients.Neurology. 2003; 60: 1674-1675Crossref PubMed Scopus (66) Google Scholar On average, antigliadin antibodies seem to be more prevalent in ataxia than in the general population (30%; 89 of 209 in ataxic patients compared with 8%–12% in controls), suggesting an association between cerebellar degeneration and gluten sensitivity.14Hadjivassiliou 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; 347: 369-371Abstract PubMed Scopus (300) Google Scholar, 15Pellecchia M.T. Scala R. Filla A. De Michele G. Ciacci C. Barone P. Idiopathic cerebellar ataxia associated with celiac disease lack of distinctive neurological features.J Neurol Neurosurg Psychiatry. 1999; 66: 32-35Crossref PubMed Scopus (109) Google Scholar, 16Burk K. Bosch S. Muller C.A. Melms A. Zuhlke C. Stern M. Besenthal I. Skalej M. Ruck P. Ferber S. Klockgether T. Dichgans J. Sporadic cerebellar ataxia associated with gluten sensitivity.Brain. 2001; 124: 1013-1019Crossref PubMed Scopus (142) Google Scholar, 20Combarros O. Infante J. Lopez-Hoyos M. Bartolome M.J. Berciano J. Corral J. Volpini V. Celiac disease and idiopathic cerebellar ataxia.Neurology. 2000; 54: 2346Crossref PubMed Scopus (35) Google Scholar, 21Bushara K.O. Goebel S.U. Shill H. Goldfarb L.G. Hallett M. Gluten sensitivity in sporadic and hereditary cerebellar ataxia.Ann Neurol. 2001; 49: 540-543Crossref PubMed Scopus (112) Google Scholar, 22Hadjivassiliou M. Grunewald R.A. Davies-Jones G.A. Causes of cerebellar degeneration gluten ataxia in perspective.J Neurol Sci. 2001; 187: S520Google Scholar However, there also is evidence of a high prevalence of antigliadin antibody positivity in genetic neurodegenerative disorders such as hereditary spinocerebellar ataxia and Huntington’s disease.21Bushara K.O. Goebel S.U. Shill H. Goldfarb L.G. Hallett M. Gluten sensitivity in sporadic and hereditary cerebellar ataxia.Ann Neurol. 2001; 49: 540-543Crossref PubMed Scopus (112) Google Scholar, 24Bushara K.O. Nance M. Gomez C.M. Antigliadin antibodies in Huntington’s disease.Neurology. 2004; 62: 132-133Crossref PubMed Scopus (39) Google Scholar It remains unclear whether gluten sensitivity contributes to the pathogenesis of these disorders or whether it represents an epiphenomenon. It also is controversial whether antigliadin antibodies directly interact with the nervous system.25Dick D.J. Abraham D. Falkous G. Hishon S. Cerebellar ataxia in coeliac disease—no evidence of a humoral aetiology.Postgrad Med J. 1995; 71: 186Crossref PubMed Scopus (11) Google Scholar, 26Hadjivassiliou M. Boscolo S. Davies-Jones G.A. Grunewald R.A. Not T. Sanders D.S. Simpson J.E. Tongiorgi E. Williamson C.A. Woodroofe N.M. The humoral response in the pathogenesis of gluten ataxia.Neurology. 2002; 58: 1221-1226Crossref PubMed Scopus (186) Google Scholar The effect of gluten-free diet on ataxia has been variable.27Chinnery P.F. Reading P.J. Milne D. Gardner-Medwin D. Turnbull D.M. CSF antigliadin antibodies and the Ramsay Hunt syndrome.Neurology. 1997; 49: 1131-1133Crossref PubMed Scopus (44) Google Scholar, 28Pellecchia M.T. Scala R. Perretti A. De Michele G. Santoro L. Filla A. Ciacci C. Barone P. Cerebellar ataxia associated with subclinical celiac disease responding to gluten-free diet.Neurology. 1999; 53: 1606-1608Crossref PubMed Google Scholar, 29Bushara K.O. Shill H. Hallett M. Open-label trial of gluten-free diet in sporadic and hereditary cerebellar ataxia with gluten sensitivity.Mov Disord. 2002; 17 (abstr): S325Google Scholar, 30Hadjivassiliou M. Davies-Jones G.A. Sanders D.S. Grunewald R.A. Dietary treatment of gluten ataxia.J Neurol Neurosurg Psychiatry. 2003; 74: 1221-1224Crossref PubMed Scopus (172) Google Scholar There are rare reports showing efficacy of immunomodulation with intravenous immunoglobulins.31Burk K. Melms A. Schulz J.B. Dichgans J. Effectiveness of intravenous immunoglobin therapy in cerebellar ataxia associated with gluten sensitivity.Ann Neurol. 2001; 50: 827-828Crossref PubMed Scopus (75) Google Scholar, 32Sander H.W. Magda P. Chin R.L. Wu A. Brannagan 3rd, T.H. Green P.H. Latov N. Cerebellar ataxia and coeliac disease.Lancet. 2003; 362: 1548Abstract Full Text Full Text PDF PubMed Scopus (60) Google Scholar Evidence for peripheral neuropathy has been found in up to 49% of CD patients.33Cicarelli G. Della Rocca G. Amboni M. Ciacci C. Mazzacca G. Filla A. Barone P. Clinical and neurological abnormalities in adult celiac disease.Neurol Sci. 2003; 24: 311-317Crossref PubMed Scopus (110) Google Scholar In a recent series of 20 patients with neuropathy and biopsy examination-confirmed CD, 9 patients presented with neuropathic symptoms before the diagnosis of CD was made.34Chin R.L. Sander H.W. Brannagan T.H. Green P.H. Hays A.P. Alaedini A. Latov N. Celiac neuropathy.Neurology. 2003; 60: 1581-1585Crossref PubMed Scopus (202) Google Scholar Chronic distal, symmetric, predominantly sensory neuropathy is described most commonly in patients with CD; however, pure motor neuropathy, mononeuritis multiplex, Guillain-Barré-like syndrome and autonomic neuropathy also have been reported.3Cooke W. Smith W. Neurological disorders associated with adult coeliac disease.Brain. 1966; 89: 683-722Crossref PubMed Scopus (325) Google Scholar, 34Chin R.L. Sander H.W. Brannagan T.H. Green P.H. Hays A.P. Alaedini A. Latov N. Celiac neuropathy.Neurology. 2003; 60: 1581-1585Crossref PubMed Scopus (202) Google Scholar, 35Banerji N.K. Hurwitz L.J. Neurological manifestations in adult steatorrhoea (probable Gluten enteropathy).J Neurol Sci. 1971; 14: 125-141Abstract Full Text PDF PubMed Scopus (50) Google Scholar, 36Kaplan J.G. Pack D. Horoupian D. DeSouza T. Brin M. Schaumburg H. Distal axonopathy associated with chronic gluten enteropathy a treatable disorder.Neurology. 1988; 38: 642-645Crossref PubMed Google Scholar, 37Hadjivassiliou M. Chattopadhyay A.K. Davies-Jones G.A. Gibson A. Grunewald R.A. Lobo A.J. Neuromuscular disorder as a presenting feature of coeliac disease.J Neurol Neurosurg Psychiatry. 1997; 63: 770-775Crossref PubMed Scopus (106) Google Scholar, 38Usai P. Usai Satta P. Lai M. Corda M.G. Piras E. Calcara C. Boy M.F. Morelli A. Balestrieri A. Bassotti G. Autonomic dysfunction and upper digestive functional disorders in untreated adult coeliac disease.Eur J Clin Invest. 1997; 27: 1009-1015Crossref PubMed Scopus (83) Google Scholar The most common electrophysiologic diagnosis is that of mixed sensorimotor axonal peripheral neuropathy.35Banerji N.K. Hurwitz L.J. Neurological manifestations in adult steatorrhoea (probable Gluten enteropathy).J Neurol Sci. 1971; 14: 125-141Abstract Full Text PDF PubMed Scopus (50) Google Scholar, 36Kaplan J.G. Pack D. Horoupian D. DeSouza T. Brin M. Schaumburg H. Distal axonopathy associated with chronic gluten enteropathy a treatable disorder.Neurology. 1988; 38: 642-645Crossref PubMed Google Scholar, 37Hadjivassiliou M. Chattopadhyay A.K. Davies-Jones G.A. Gibson A. Grunewald R.A. Lobo A.J. Neuromuscular disorder as a presenting feature of coeliac disease.J Neurol Neurosurg Psychiatry. 1997; 63: 770-775Crossref PubMed Scopus (106) Google Scholar However, electrophysiologic studies can be normal or only mildly abnormal in many CD patients with neuropathy.34Chin R.L. Sander H.W. Brannagan T.H. Green P.H. Hays A.P. Alaedini A. Latov N. Celiac neuropathy.Neurology. 2003; 60: 1581-1585Crossref PubMed Scopus (202) Google Scholar Indeed, CD was found in 8% of 400 patients with neuropathy and normal electrophysiologic studies.34Chin R.L. Sander H.W. Brannagan T.H. Green P.H. Hays A.P. Alaedini A. Latov N. Celiac neuropathy.Neurology. 2003; 60: 1581-1585Crossref PubMed Scopus (202) Google Scholar In one study, antiganglioside antibodies were found (by using rapid ganglioside agglutination immunoassay39Alaedini A. Wirguin I. Latov N. Ganglioside agglutination immunoassay for rapid detection of autoantibodies in immune-mediated neuropathy.J Clin Lab Anal. 2001; 15: 96-99Crossref PubMed Scopus (18) Google Scholar) in 13 (65%) of 20 patients with CD and neuropathy.34Chin R.L. Sander H.W. Brannagan T.H. Green P.H. Hays A.P. Alaedini A. Latov N. Celiac neuropathy.Neurology. 2003; 60: 1581-1585Crossref PubMed Scopus (202) Google Scholar These autoantibodies bind to the Schwann cell surface, nodes of Ranvier, and axons in peripheral nerves. Antiganglioside antibodies commonly are associated with autoimmune peripheral neuropathies with some specificity (eg, immunoglobulin [Ig]G antibodies to GM1 and GD1b in Guillain-Barré syndrome variants with axonal degeneration, anti-GQ1b and GT1b in Miller-Fisher’s syndrome, and IgM antibodies to GM1 in multifocal motor neuropathy). Alaedini et al40Alaedini A. Green P.H. Sander H.W. Hays A.P. Gamboa E.T. Fasano A. Sonnenberg M. Lewis L.D. Latov N. Ganglioside reactive antibodies in the neuropathy associated with celiac disease.J Neuroimmunol. 2002; 127: 145-148Abstract Full Text Full Text PDF PubMed Scopus (73) Google Scholar reported 6 patients with CD who were positive for 1 or more IgG antibodies to GM1, GM2, GD1a, and GD1b (4 patients had anti-GM1 antibodies and none had GQ1b or GT1b). There are few case reports documenting the co-occurrence of CD with less common neuromuscular syndromes such as polymyositis, dermatomyositis, inclusion body myositis, and neuromyotonia.37Hadjivassiliou M. Chattopadhyay A.K. Davies-Jones G.A. Gibson A. Grunewald R.A. Lobo A.J. Neuromuscular disorder as a presenting feature of coeliac disease.J Neurol Neurosurg Psychiatry. 1997; 63: 770-775Crossref PubMed Scopus (106) Google Scholar, 41Henriksson K.G. Hallert C. Norrby K. Walan A. Polymyositis and adult coeliac disease.Acta Neurol Scand. 1982; 65: 301-319Crossref PubMed Scopus (64) Google Scholar, 42Marie I. Lecomte F. Hachulla E. 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Non-malignant complications of coeliac disease.Acta Paediatr Suppl. 1996; 412: 68-75Crossref PubMed Google Scholar On the other hand, a higher CD prevalence (.8%–2.5%) was found in epilepsy patients compared with the general population.49Fois A. Vascotto M. Di Bartolo R.M. Di Marco V. Celiac disease and epilepsy in pediatric patients.Childs Nerv Syst. 1994; 10: 450-454Crossref PubMed Scopus (60) Google Scholar, 50Cronin C.C. Jackson L.M. Feighery C. Shanahan F. Abuzakouk M. Ryder D.Q. Whelton M. Callaghan N. Coeliac disease and epilepsy.QJM. 1998; 91: 303-308Crossref PubMed Scopus (95) Google Scholar, 51Luostarinen L. Dastidar P. Collin P. Peraaho M. Maki M. Erila T. Pirttila T. Association between coeliac disease, epilepsy and brain atrophy.Eur Neurol. 2001; 46: 187-191Crossref PubMed Scopus (40) Google Scholar, 52Pratesi R. Gandolfi L. Martins R.C. Tauil P.L. Nobrega Y.K. Teixeira W.A. Is the prevalence of celiac disease increased among epileptic patients?.Arq Neuropsiquiatr. 2003; 61: 330-334Crossref PubMed Scopus (36) Google Scholar A more specific syndrome of bilateral occipital cerebral calcification and seizures has been associated with CD. This association first was described by Visakorpi et al53Visakorpi J. Kuitunen P. Pelkonen P. Intestinal malabsorption a clinical study of 22 children over 2 years of age.Acta Paediatr Scand. 1970; 59: 273-280Crossref PubMed Scopus (30) Google Scholar in 1970 and subsequently was confirmed by several studies.54Garwicz S. Mortensson W. Intracranial calcification mimicking the Sturge-Weber syndrome a consequence of cerebral folic acid deficiency?.Pediatr Radiol. 1976; 5: 5-9Crossref PubMed Scopus (46) Google Scholar, 55Molteni N. Bardella M.T. Baldassarri A.R. Bianchi P.A. 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Celiac disease and epilepsy in pediatric patients.Childs Nerv Syst. 1994; 10: 450-454Crossref PubMed Scopus (60) Google Scholar screened 783 children presenting with seizures and found CD in 9 patients with partial and/or grand mal seizures. Of these only 3 (.3%) had cerebral calcification (2 had occipital calcification and occipital seizures with episodes of transient blindness). Luostarinen et al51Luostarinen L. Dastidar P. Collin P. Peraaho M. Maki M. Erila T. Pirttila T. Association between coeliac disease, epilepsy and brain atrophy.Eur Neurol. 2001; 46: 187-191Crossref PubMed Scopus (40) Google Scholar examined brain scans of 130 patients with epilepsy and found 11 patients with intracerebral calcifications (3 had posterior calcifications) but none had CD. Magaudda et al59Magaudda A. Dalla Bernardina B. De Marco P. Sfaello Z. Longo M. Colamaria V. Daniele O. Tortorella G. Tata M.A. Di Perri R. et al.Bilateral occipital calcification, epilepsy and coeliac disease clinical and neuroimaging features of a new syndrome.J Neurol Neurosurg Psychiatry. 1993; 56: 885-889Crossref PubMed Scopus (78) Google Scholar found CD in only about half of epileptic patients with bilateral occipital calcification who underwent duodenal biopsy examination. The majority of the patients reported had complex partial seizures referable to the occipital or temporal lobes; however, secondarily generalized seizures, other seizure types, and episodic headaches also were described.60Crosato F. Senter S. Cerebral occipital calcifications in celiac disease.Neuropediatrics. 1992; 23: 214-217Crossref PubMed Scopus (19) Google Scholar, 61Arroyo H.A. De Rosa S. Ruggieri V. de Davila M.T. Fejerman N. Epilepsy, occipital calcifications, and oligosymptomatic celiac disease in childhood.J Child Neurol. 2002; 17: 800-806Crossref PubMed Scopus (54) Google Scholar The syndrome can be distinguished clinically from Sturge-Weber syndrome by the absence of facial angioma (port wine stain or nevus flammeus), bilaterality of the occipital calcification, and their extension into the subcortical region on computed tomography scan. The calcified lesions described in CD also lack contrast enhancement on magnetic resonance imaging and, unlike Sturge-Weber syndrome, are not associated with cerebral atrophy or vascular anomalies.59Magaudda A. Dalla Bernardina B. De Marco P. Sfaello Z. Longo M. Colamaria V. Daniele O. Tortorella G. Tata M.A. Di Perri R. et al.Bilateral occipital calcification, epilepsy and coeliac disease clinical and neuroimaging features of a new syndrome.J Neurol Neurosurg Psychiatry. 1993; 56: 885-889Crossref PubMed Scopus (78) Google Scholar The histology of the calcified lesions in CD is similar but not identical to Sturge-Weber syndrome and consists of patchy pial angiomas, fibrosed veins, and large jagged microcalcifications.62Bye A.M. Andermann F. Robitaille Y" @default.
- W2100060114 created "2016-06-24" @default.
- W2100060114 creator A5070256248 @default.
- W2100060114 date "2005-04-01" @default.
- W2100060114 modified "2023-09-27" @default.
- W2100060114 title "Neurologic presentation of celiac disease" @default.
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- W2100060114 doi "https://doi.org/10.1053/j.gastro.2005.02.018" @default.
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