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  • Jefferson Adams
    Jefferson Adams
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    Does Autoimmunity Play a Role in Cerebellar Ataxia with Sensory Ganglionopathy?

      Nineteen patients had gluten sensitivity, of whom 3 patients had biopsy proven celiac disease

    Caption: Photo: CC--www.twin-loc.fr

    Celiac.com 01/15/2018 - Cerebellar ataxia with sensory ganglionopathy is a disabling combination of neurological dysfunction that usually occurs as part of certain hereditary ataxias. However, some patients present this combination with no apparent genetic cause.

    A team of researchers recently set out to if autoimmunity might have a role to play in SG. The research team included Panagiotis Zis, Ptolemaios Georgios Sarrigiannis, Dasappaiah Ganesh Rao, Nigel Hoggard, David Surendran Sanders, and Marios Hadjivassiliou. They are variously affiliated with the Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK; the University of Sheffield, Sheffield, UK; the Department of Neuroradiology, Sheffield Teaching Hospitals NHS Foundaiton Trust, Sheffield, UK; the University of Sheffield, Sheffield, UK; and the Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.

    The team reviewed records of all patients that have been referred to the Sheffield Ataxia Centre who had neurophysiological and imaging data suggestive of SG and cerebellar ataxia respectively. We excluded patients with Friedreich's ataxia, a common cause of this combination. All patients were screened for genetic causes and underwent extensive investigations.

    They found 40 patients with combined cerebellar ataxia and sensory ganglionopathy. The majority of patients were initially diagnosed with cerebellar dysfunction, and about one-third were initially diagnosed with sensory ganglionopathy.

    For that one-third, the two diagnoses were made together. The average time between the two diagnoses was 6.5 ± 8.9 years, ranging from 0 up to 44 years. The most common initial symptom was unsteadiness, in 77.5% of patients, followed by patchy sensory loss in 17.5%, and peripheral neuropathic pain in 5%.

    Nineteen patients had gluten sensitivity, of whom 3 patients had biopsy proven celiac disease. Other abnormal immunological tests were present in another 15 patients. Six patients had malignancy, which was diagnosed within 5 years of the neurological symptoms. Only 3 patients were classified as having a truly idiopathic combination of cerebellar ataxia with sensory ganglionopathy.

    This study shows that immune pathogenesis plays a significant role in patients with the unusual combination of cerebellar ataxia and sensory ganglionopathy.

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  • About Me

    Jefferson Adams earned his B.A. and M.F.A. at Arizona State University, and has authored more than 2,000 articles on celiac disease. His coursework includes studies in biology, anatomy, medicine, and science. He previously served as Health News Examiner for Examiner.com, and provided health and medical content for Sharecare.com. Jefferson has spoken about celiac disease to the media, including an appearance on the KQED radio show Forum, and is the editor of the book "Cereal Killers" by Scott Adams and Ron Hoggan, Ed.D.

  • Related Articles

    Jefferson Adams
    Celiac.com 06/01/2015 - Earlier research on celiac disease and neuropathy has been hampered by the use of inpatient data, low study power, and lack of information on neuropathic characteristics.
    A team of researchers recently set out to accurately assess both relative and absolute risk of developing neuropathy in a nationwide population-based sample of patients with biopsy-verified celiac disease. The research team included Sujata P. Thawani, MD, MPH; Thomas H. Brannagan III, MD; Benjamin Lebwohl, MD, MS; Peter H. R. Green, MD; and Jonas F. Ludvigsson, MD, PhD.
    They are variously affiliated with the Peripheral Neuropathy Center at the Neurological Institute of Columbia University College of Physicians and Surgeons, the Celiac Disease Center in the Department of Medicine at Columbia University College of Physicians and Surgeons in New York, New York, with the Department of Medical Epidemiology and Biostatistics at the Karolinska Institutet in Stockholm, Sweden, and with the Department of Pediatrics, Örebro University Hospital, Örebro University, Örebro, Sweden.
    For their study, the team collected data on small-intestinal biopsies conducted at Sweden’s 28 pathology departments from 1969 to 2008. They compared the risk of neuropathy in a total of 28 ,232 celiac disease patients, all with villous atrophy, Marsh 3, against results from 139, 473 age- and sex-matched non-celiac control subjects.
    They used Cox proportional hazards regression to estimate hazard ratios (HRs), and 95% confidence intervals (CIs), for neuropathy as defined by relevant International Classification of Diseases codes in the Swedish National Patient Register; including both inpatient and outpatient data.
    They found that patients with biopsy-verified celiac disease faced a 2.5 times higher risk of developing neuropathy (95% CI, 2.1-3.0; P < .001). Celiac patients also had an increased risk of developing chronic inflammatory demyelinating neuropathy (2.8; 1.6-5.1; P = .001), autonomic neuropathy (4.2; 1.4-12.3; P = .009), and mononeuritis multiplex (7.6; 1.8-32.4; P = .006).
    However, the team found no association between celiac disease and acute inflammatory demyelinating polyneuropathy (0.8; 0.3-2.1; P = .68).
    The team found a significantly increased risk of neuropathy in patients with celiac disease, and they are recommending that doctors screen patients with neuropathy for celiac disease.
    Source:
    JAMA Neurol. Published online May 11, 2015. doi:10.1001/jamaneurol.2015.0475

    Jefferson Adams
    Gluten Triggers 1 in 4 Cases of Sporadic Ataxia
    Celiac.com 01/16/2017 - Cerebellar ataxias can be caused by a wide range of disease processes, either genetic or acquired. Establishing a clear diagnosis requires a methodical approach with expert clinical evaluation and investigation.
    A team of researchers recently published a description of the causes of ataxia in 1500 patients with cerebellar ataxia.  The research team included M Hadjivassiliou, J Martindale, P Shanmugarajah, R A Grünewald, P G Sarrigiannis, N Beauchamp, K Garrard, R Warburton, D S Sanders, D Friend, S Duty, J Taylor, and N Hoggard.
    They are variously affiliated with the Academic Department of Neurosciences, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK; Sheffield Diagnostic Genetics Service, Sheffield Children's NHS Foundation Trust, Sheffield, UK; the Department of Gastroenterology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK; and the Department of Neuroradiology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK.
    All patients in the study were referred to the Sheffield Ataxia Centre, UK, and underwent extensive examination, including, where appropriate genetic testing using next-generation sequencing (NGS).
    The team followed-up patients on a 6-month basis for reassessment and further investigations, as needed.
    The team assessed a total of 1500 patients over 20 years. Twenty per cent of those patients had a family history of ataxia, with the remaining having sporadic ataxia.
    The most common cause of sporadic ataxia was gluten ataxia at 25%. They found a genetic cause in 156, or 13% of sporadic cases, with alcohol excess causing 12% and a cerebellar variant of multiple system atrophy causing 11% of sporadic cases.
    Using NGS, they obtained positive results in 32% of 146 patients tested. The most common ataxia they found was EA2. A total of 57% of all familial ataxias were supported by genetic diagnosis. The most common genetic ataxias were Friedreich's ataxia (22%), SCA6 (14%), EA2 (13%), SPG7 (10%) and mitochondrial disease (10%).
    The diagnostic yield following attendance at the Sheffield Ataxia Centre was 63%. Immune-mediated ataxias are common. Advances in genetic testing have significantly improved the diagnostic yield of patients suspected of having a genetic ataxia.
    Making a diagnosis of the cause of ataxia is essential due to potential therapeutic interventions for immune and some genetic ataxias.
    Gluten is a culprit is 25% of sporadic ataxia cases, and clinicians should keep this in mind when diagnosing patients, as many of these cases can be reversed with a gluten-free diet.
    Source:
    J Neurol Neurosurg Psychiatry. doi:10.1136/jnnp-2016-314863

    Jefferson Adams
    Can a Gluten-Free Diet Help Ataxia Patients?
    Celiac.com 08/10/2017 - Gluten ataxia is defined as sporadic ataxia with positive antigliadin antibodies without an alternative cause. Gluten ataxia patients often receive MRS at baseline and again after a period on a gluten-free diet.
    A research team recently set out to evaluate the effect of gluten free diet on magnetic resonance spectroscopy (MRS) of the cerebellum in patients with gluten ataxia.
    The research team included M Hadjivassiliou, RA Grünewald, DS Sanders, P Shanmugarajah, N Hoggard. They are with the Academic Departments of Neurosciences (M.H., R.A.G., P.S.), Gastroenterology (D.S.S.), and Neuroradiology (N.H.), Sheffield Teaching Hospitals NHS Trust, UK.
    The team included 117 consecutive patients with gluten ataxia in their report. Sixty-three followed a strict a gluten-free diet with elimination of antigliadin antibodies, 35 ate a gluten-free diet, but still tested positive for antigliadin antibodies, while 19 patients were not following a gluten-free diet.
    The N-acetylaspartate (NAA)/creatine (Cr) area ratio from the cerebellar vermis increased in 62 out of 63 (98%) patients on strict a gluten-free diet, in 9 of 35 (26%) patients on a gluten-free diet, but positive antibodies, and in only 1 of 19 (5%) patients not on a gluten-free diet. The NAA/Cr ratio decreased in all 14 ataxia control patients (cerebellar variant of multisystem atrophy), while the researchers saw no differences in the MRS results between patients with celiac disease and those without.
    Better NAA/Cr ratios seen on follow-up scans supports previous findings that gluten ataxia patients see clinical improvement a gluten-free diet
    Such improvements can occur regardless of existing enteropathy, so patients with positive serology and negative duodenal biopsy should still maintain a strict a gluten-free diet.
    Source:
    Neurology. 2017 Jul 19. pii: 10.1212/WNL.0000000000004237.doi: 10.1212/WNL.0000000000004237.

    Jefferson Adams
    Could a New Cannabis Patch Bring Relief from Celiac-Related Nerve Pain?
    Celiac.com 11/27/2017 - For centuries, physicians have used cannabis to treat numerous disorders. Modern research shows that various cannabis compounds can alleviate symptoms from numerous conditions, including neurological disorders, cancer, rheumatism, epilepsy, sexual disorders, pain, among others.
    Many people with celiac disease suffer from neuropathy, which causes nerve pain, among other symptoms. Neuropathy can be difficult to treat. Nerve pain is a debilitating symptom that can significantly impair a patient's quality of life. Could a new cannabis patch change that?
    Some researchers think so. One California-based company, Cannabis Science, is developing an innovative new medicinal patch specifically designed to treat nerve pain. In addition to celiac related neuropathy, the patch could be helpful in treating nerve pain from many illnesses including fibromyalgia, diabetes, and multiple sclerosis.
    The National Institute of Health estimates that over 5 million Americans suffer from fibromyalgia, which has no known cure, and is difficult to treat. With diabetes on the rise in the U.S., diabetic nerve pain is also on the rise.
    When placed on the patient's skin, the patch developed by Cannabis Science delivers a measured dose of high potency cannabidiol (CBD) extract. CBD is the second major cannabinoid in marijuana after THC, but CBD has no psychoactive effects, so it won't get people high.
    When the patch is applied, the CBD is first absorbed into the blood, then moves to the central nervous system, where it delivers pain relief.
    Numerous studies have documented CBD's “anti-inflammatory and pain-relieving properties. More recent studies have shown that CBD provides relief from many kinds of pain.
    In addition to nerve pain, CBD has been shown to relieve inflammatory pain. Some studies have shown CBD to be more effective than current medication in treating inflammatory pain, such as pain from arthritis.
    As researchers home in on the pain-relieving properties of cannabis, look for more treatments to be developed, including treatments that may helpful for peopl with celiac disease.
    Read more: cannatech.news

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