Cerebellar ataxia and coeliac disease

Sander, Howard W.; Magda, Paul; Chin, Russell L.; Wu, Anita; Brannagan, Thomas H.; Green, Peter H. R.; Latov, Norman

doi:10.1016/s0140-6736(03)14743-5

Cited by 54 publications

(29 citation statements)

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“…Amongst the atypical presentation forms, mention must be made of the association with neurological pathology (20,21) such as peripheral neuropathies and ataxia. The series we present describes 3 cases, including one ataxia and another peripheral mononeuritis.…”

Section: Discussionmentioning

confidence: 99%

Coeliac disease: clinical features in adult populations

Fernández

González

Fuente

2010

Rev. esp. enferm. dig.

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Introduction: coeliac disease (CD) is a chronic disease of the small intestine, which is caused by gluten intolerance, producing malabsorption of nutrients and vitamins. Clinical manifestations of CD in adults are highly variable, including intestinal and extra-intestinal symptoms. The disease may also occur in individuals who are asymptomatic.Objective: our objective is to describe the incidence and clinical manifestations of CD in adults.Material and methods: a retrospective study was carried out in patients diagnosed of CD between January 1990 and December 2008. Diagnosis was based on serologic tests and duodenal biopsy, which were compatible with CD in all of them.Results: sixty eight adult patients were diagnosed of CD in this period. Mean age was 33 (18-65) years and 50 (74%) were women. The clinical manifestations were diarrhoea in 38 (55%), abdominal pain in 27 (40%), loss of weight in 15 (22%), dyspepsia in 13 (19%). Analytical results showed a slight increase of transaminases in 26 (38%), ferropenic anaemia in 33 (48.5%) cases, sub-clinical hypothyroidism in 3 (4.5%) patients, and folic acid deficiency in 16 (23.5%) cases. Almost all patients were diagnosed between 2000 and 2008: 60 (87%). Population-based incidence of CD in adults had increased from 0.7-2/100,000 per year in the nineties to 3.5-10.3/100,000 in the last years.Conclusions: CD can appear at any age and with a wide manifestation spectrum, which can be atypical in some cases. Patients with ferropenic anaemia and a negative response to treatment or those with an unexplained increase in transaminases should be screening for CD. Atypical manifestations and low suspect index can delay diagnosis even during years. There is a marked increase in the incidence-rates of CD in adults over time.Key words: Coeliac disease. Incidence. Anti-transglutaminase antibodies. Gluten-free diet. Autoimmune disorders. RESUMENIntroducción: la enfermedad celiaca (EC) es una enfermedad crónica que afecta al intestino delgado, causada por intolerancia al gluten cuyas manifestaciones clínicas son muy variables incluyendo síntomas extraintestinales y formas asintomáticas.Objetivo: nuestro objetivo es describir la incidencia y manifestaciones clínicas de la EC del adulto.Métodos: estudio retrospectivo de los pacientes mayores de 18 años diagnosticados de EC entre enero-1990 y diciembre-2008 mediante test serológicos y biopsia duodenal.Resultados: se incluyeron 68 pacientes con una mediana de edad de 33 años (18-65); 50 (74%) mujeres. Las manifestaciones clínicas fueron: diarrea en 38 (55%), dolor abdominal en 27 (40%), pérdida de peso en 15 (22%), dispepsia en 13 (19%) y 3 dermatitis herpetiforme. Los principales datos analíticos fueron: aumento de transaminasas en 26 (38%), anemia ferropénica en 33 (48,5%), hipotiroidismo subclínico en 3 (4,5%) y déficit de áci-do fólico en 16 (23,5%) casos. Casi todos los pacientes han sido diagnosticados entre los años 2000 y 2008: 60 (87%). La incidencia de EC en adultos ha aumentado desde 0,7-2/100.000 habitantes por año en la déca...

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Section: Discussionmentioning

confidence: 99%

Coeliac disease: clinical features in adult populations

Fernández

González

Fuente

2010

Rev. esp. enferm. dig.

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“…In addition, the associated cerebellar ataxia and peripheral neuropathy have been reported to respond in some cases to gluten-free diet or therapy with i.v. Igs (12)(13)(14). In other studies, patient serum and anti-gliadin Abs have been shown to bind neural tissue, implying that the Abs may be cross-reacting with autoantigens (15,16).…”

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confidence: 88%

Immune Cross-Reactivity in Celiac Disease: Anti-Gliadin Antibodies Bind to Neuronal Synapsin I

Alaedini

Okamoto

Briani

et al. 2007

The Journal of Immunology

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Celiac disease is an immune-mediated disorder triggered by ingestion of wheat gliadin and related proteins in genetically susceptible individuals. In addition to the characteristic enteropathy, celiac disease is associated with various extraintestinal manifestations, including neurologic complications such as neuropathy, ataxia, seizures, and neurobehavioral changes. The cause of the neurologic manifestations is unknown, but autoimmunity resulting from molecular mimicry between gliadin and nervous system proteins has been proposed to play a role. In this study, we sought to investigate the immune reactivity of the anti-gliadin Ab response toward neural proteins. We characterized the binding of affinity-purified anti-gliadin Abs from immunized animals to brain proteins by one- and two-dimensional gel electrophoresis, immunoblotting, and peptide mass mapping. The major immunoreactive protein was identified as synapsin I. Anti-gliadin Abs from patients with celiac disease also bound to the protein. Such cross-reactivity may provide clues into the pathogenic mechanism of the neurologic deficits that are associated with gluten sensitivity.

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“…Whilst the benefits of a gluten-free diet in the treatment of patients with CD and DH have long been established, there are very few studies, mainly case reports, of the effect of gluten-free diet on the neurological manifestations of gluten sensitivity. Most of these reports primarily concern patients with established CD who then develop neurological symptoms [19,20,21,22]. These studies suggest variable but overall favourable responsiveness to a gluten-free diet.…”

Section: Treatmentmentioning

confidence: 99%

Gluten-Related Disorders: Gluten Ataxia

Hadjivassiliou

Sanders

Aeschlimann

2015

Dig Dis

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The term gluten-related disorders (GRD) refers to a spectrum of diverse clinical manifestations triggered by the ingestion of gluten in genetically susceptible individuals. They include both intestinal and extraintestinal manifestations. Gluten ataxia (GA) is one of the commonest neurological manifestations of GRD. It was originally defined as otherwise idiopathic sporadic ataxia in the presence of circulating antigliadin antibodies of IgA and/or IgG type. Newer more specific serological markers have been identified but are not as yet readily available. GA has a prevalence of 15% amongst all ataxias and 40% of all idiopathic sporadic ataxias. It usually presents with gait and lower limb ataxia. It is of insidious onset with a mean age at onset of 53 years. Up to 40% of patients have evidence of enteropathy on duodenal biopsy. Gastrointestinal symptoms are seldom prominent and are not a reliable indicator for the presence of enteropathy. Furthermore, the presence of enteropathy does not influence the response to a gluten-free diet. Most patients will stabilise or improve with strict adherence to gluten-free diet depending on the duration of the ataxia prior to the treatment. Up to 60% of patients with GA have evidence of cerebellar atrophy on MR imaging, but all patients have spectroscopic abnormalities primarily affecting the vermis. Recent evidence suggests that patients with newly diagnosed coeliac disease presenting to the gastroenterologists have abnormal MR spectroscopy at presentation associated with clinical evidence of subtle cerebellar dysfunction. The advantage of early diagnosis and treatment (mean age 42 years in patients presenting with gastrointestinal symptoms vs. 53 years in patients presenting with ataxia) may protect the first group from the development and/or progression of neurological dysfunction.

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Cerebellar ataxia and coeliac disease

Cited by 54 publications

References 5 publications

Coeliac disease: clinical features in adult populations

Coeliac disease: clinical features in adult populations

Immune Cross-Reactivity in Celiac Disease: Anti-Gliadin Antibodies Bind to Neuronal Synapsin I

Gluten-Related Disorders: Gluten Ataxia

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