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Celiac.com 10/27/2014 - There have been a few reports tying cortical myoclonus with ataxia to celiac disease. Such reports also suggest that the former is unresponsive to a gluten-free diet. A team of researchers recently set out to determine if there is any significant connection between the two conditions. The research team included Ptolemaios G. Sarrigiannis, Nigel Hoggard, Daniel Aeschlimann, David S. Sanders, Richard A. Grünewald, Zoe C. Unwin, and Marios Hadjivassiliou. They are variously associated with the Departments of Gastroenterology, Neurology, Neurophysiology and Neuroradiology at Royal Hallamshire Hospital, in Sheffield, UK, and with the College of Biomedical and Life Sciences at Cardiff University in Cardiff, UK. The team presented detailed electro-clinical characteristics of a new syndrome of progressive cortical hyperexcitability with ataxia and refractory celiac disease. Regular follow ups of over 600 patients with neurological manifestations due to gluten sensitivity revealed 9 patients with this syndrome. They found that all nine patients, six men and three women, experienced asymmetrical irregular myoclonus involving one or more limbs and sometimes face. This was often stimulus sensitive and became more widespread over time. Three patients had a history of Jacksonian march, and five had at least one secondarily generalized seizure. Electrophysiology showed evidence of cortical myoclonus. Three showed a phenotype of epilepsia partialis continua at onset. All patients showed clinical, imaging and/or pathological evidence of cerebellar involvement. All patients followed a strict gluten-free diet, and most successfully eliminated gluten-related antibodies. However, all patients still showed evidence of enteropathy, suggests that refractory celiac disease is to blame. During the study, two patients died from enteropathy-associated lymphoma and one from status epilepticus. Five patients were treated with mycophenolate and one in addition with rituximab and IV immunoglobulins. These patients showed improvement of ataxia and enteropathy, but continued to suffer the effects of myoclonus. These results indicate that myoclonus ataxia might be the most common neurological manifestation of refractory celiac disease. The clinical involvement, apart from ataxia, covers the whole clinical spectrum of cortical myoclonus. Source: Open Original Shared LinkOpen Original Shared Link

Celiac.com 10/08/2022 - Celiac disease is now recognized as a spectrum of gluten-sensitive illness in which the gut is no longer seen as the sole target. For example, dermatitis herpetiformis is a skin manifestation of gluten sensitivity. Celiac disease is no longer considered just in individuals with classic intestinal damage but in individuals with other signs of immune activation and/or degrees of gut involvement, triggered by the ingestion of gluten. Substantial evidence demonstrates that the nervous system also can be a target organ with or without the presence of gut involvement(1). Neurological Complications in Celiac Disease Approximately ten percent of celiac disease patients develop neurological complications(2) . Based on case studies, the most common neurological disorders associated with celiac disease are cerebellar dysfunction, epilepsy and peripheral neuropathy. From 1964 to 2000, data compiled from case reports of 83 celiac patients with neurological complications revealed that 70% of them were diagnosed with either ataxia or peripheral neuropathy(3) . A retrospective data survey of 620 patients attending the Derby Coeliac Clinic found the following neurological and psychiatric complications: Depression (12%), epilepsy (4%), migraine (3%), carpal tunnel syndrome (2%), stroke (2%), anxiety (2%), self poisoning (2%), myopathy (1%), learning difficulty (1%), sciatica (1%), meningitis (1%), Parkinson’s disease (1%), tension headache (1%), multiple sclerosis (1%) and peripheral neuropathy (1%)2 . However, further study needs to clarify the relationship between celiac disease and these complications. A recent study found 13 (8%) among 160 celiac patients had neurological disorders(4) . Ten of the thirteen patients had central nervous system disorders such as epilepsy, attention/memory impairment, and cerebellar ataxia. The remaining patients had peripheral nervous system disorders. In eleven out of thirteen cases, the celiac disease diagnosis came after the onset of the neurological disorder. Seven celiac patients were diagnosed and treated within six months of the neurological onset. All seven had either substantial or complete resolution of their neurological symptoms. In contrast, four out of five patients who were diagnosed with celiac disease from 10 to 264 months after the appearance of their neurological disorder showed no improvement in their neurological symptoms on the gluten-free diet. This study demonstrated that the crucial timing of treatment with a gluten-free diet in celiac patients who have neurological disorders might affect whether the neurological symptoms are reversible. While the incidence of neurological complications in celiac disease is estimated to be ten percent, the incidence of celiac disease in neurological patients is still unknown. Celiac disease can escape detection in blood antibody screenings(5). Not all gluten-sensitive individuals demonstrate classic biopsy evidence of celiac disease, but exhibit milder intestinal features. Also, not all celiac patients present with gastrointestinal symptoms. Therefore, neurological patients with gluten-sensitivity may be missed if they are evaluated for neurological symptoms alone. To identify gluten-sensitivity in neurological patients, antigliadin antibodies were determined in two groups of neurological patients. In a group with idiopathic neurological illness versus another group with identifiable neurological illness, a marked difference of 57% versus 5%, respectively, were antigliadin antibody positive(2) . Twenty-six (86%) of those in the idiopathic group consented to small bowel biopsy and nine (34%) of them had intestinal features characteristic of celiac disease. However, 12% of the healthy blood donors were also antigliadin antibody positive and no explanation was given. Therefore, it was unclear whether the rate of gluten-sensitive neurological illness could be overstated by 12 percent or that 12 percent of the normal population could have gluten-sensitivity. Gluten Ataxia Gluten ataxia is the most common form of neurological dysfunction to be attributed to gluten sensitivity1 . Up to 41% of sporadic idiopathic ataxia is caused by gluten ataxia, as evidenced by the presence of antigliadin antibodies. Patients with gluten ataxia have difficulty controlling their upper and/or lower limb movements. Hadjivassiliou et al found 79% (54 of 68) of gluten ataxia patients had damage to the part of the brain called the cerebellum which is involved in coordination and steadiness. Not all gluten-sensitive, neurological patients will also have classic intestinal biopsy evidence of celiac disease. Of 51 gluten ataxia patients who underwent duodenal biopsy, 24% of them had biopsy proof of celiac disease and only 13% had gastrointestinal symptoms1 . Yet, treatment with a gluten-free diet can be helpful, irrespective of gut involvement. For example, 26 patients with gluten ataxia were offered a gluten-free diet and were confirmed to be adhering to the gluten-free diet by evidence of negative serology within six months to one year of treatment(6). When compared to the control group of patients with gluten ataxia who did not receive treatment of a gluten-free diet, all 26 patients in the treatment group improved significantly in their ataxia based on a battery of tests. The response observed in the treatment group was irrespective of gut involvement or the duration of the ataxia (mean duration of nine years). These results contrasted with the expectation that the ataxia would remain despite evidence of the loss of cerebellar Purkinje cells which are the target cells in gluten ataxia(3, 6). Malabsorption or Autoimmunity? Two potential mechanisms to explain the neurological dysfunctions of celiac disease are nutrient deficiencies due to malabsorption, and autoimmune disease. Currently, it is unknown which of these, or both, is the underlying cause of neurological disorder in celiac disease. A reference to these potential mechanisms came in 1966 when Cooke and Smith reported a landmark study of 16 adult celiac patients with neurological complications3 . For most of them, symptoms of classic celiac disease pre-existed their neurological symptoms. All 16 were found with extreme weight loss and vitamin deficiencies along with anemia due to severe malabsorption. Subsequently, over half of them died, despite gluten restriction, due to the progression of their neurological complications involving sensory ataxia and/or other features. Post-mortem findings in four patients revealed cerebellar Purkinje cell loss and T-cells (type of white blood cell) infiltrating parts of the brain, brainstem, and spinal cord(7). Deficiencies of folic acid, vitamin B-12, and vitamin E have been implicated as a potential cause of neurological complications(4). However, vitamin deficiencies alone do not explain the absence of neurological deficits in some patients(2). In addition, vitamin deficiencies are rarely found or can be attributed to neurological dysfunction in association with gluten ataxia patients of which the majority don’t have histological evidence of celiac disease(3). Furthermore, in a current study of 13 neurological celiac patients, only 2 had been diagnosed with malabsorption(4). In support of the hypothesis of an autoimmune mechanism of celiac disease neurological complications, Hadjivassiliou et al found that gluten ataxia patients with inflammation located in the white matter of the cerebellum part of the brain was marked by the loss of Purkinje cells. The inflammation in celiac disease, which is thought to be mediated by T-cells, is not confined to the small bowel as gliadin-specific T cells and antigliadin antibodies are found in the blood(8). Antigliadin antibodies also have been found in the cerebrospinal fluid(3) . In gluten ataxia patients, antigliadin antibodies were found to bind to Purkinje cells in the cerebellum that might result in damage to this part of the brain(9). This finding suggests that common binding sites are shared between cerebellar Purkinje cells and gliadin proteins. Patients with gluten ataxia also have anti-Purkinje cell antibodies. How antigliadin antibodies gain access to the cerebellum might be due to possible alterations of the blood-brain barrier. In further support of an autoimmune basis of celiac disease neurological complications, 8 of 13 celiac patients with neurological dysfunction had anti-neuronal antibodies to the central nervous system(4). This was significantly higher when compared with only 1 in 20 celiac patients who had anti-neuronal antibodies but no neurological involvement. Furthermore, 30 non-celiac control patients, who had other autoimmune gastrointestinal diseases or had donated blood, had no detectable anti-neuronal antibodies. After one year of treatment on a gluten-free diet, anti-neuronal antibodies disappeared in 6 of the 8 celiac patients with neurological dysfunction. In 5 of these 6 patients, the neurological symptoms partially or completely resolved. However, anti-neuronal antibodies are not specific for neurological disorders associated with celiac disease since they are also found in other patients with nervous system disorders. Identification of neurological patients with gluten-sensitivity with or without histological evidence of celiac disease is necessary in order to provide them the opportunity for treatment with a gluten-free diet. Identification should be further aided when the exact mechanisms of neurological complications in gluten-sensitive patients are understood. Finally, immediate treatment with a gluten-free diet early in the progression of the disease may be crucial in the prognosis of whether the neurological disorder is reversible. Glossary of Terms: ataxia: impaired muscle coordination Central Nervous System (CNS): the portion of the nervous system involving the brain and spinal cord cerebellum: portion of the brain involved in equilibrium and coordination; cerebellar (adj.) dementia: impaired intellectual function epilepsy: neurologic disease resulting in convulsions or loss of consciousness idiopathic: the disease has an unknown cause neurological: having to do with the nervous system neuron: nerve cell neuropathy: any disease of the nervous system paroxysm: convulsion Peripheral Nervous System (PNS): the portion of the nervous system outside of the brain and spinal cord; peripheral (adj.) Purkinje cell: a type of neuron that is highly branched, mostly found in the cerebellum References: Hadjivassiliou M et al 2003. Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics. Brain 126: 685-91. Tengah D et al 2002. Neurological complications of coeliac disease. Postgrad Med J 78: 393-98. Hadjivassiliou, et al. 2002. Gluten sensitivity as a neurological illness. J Neurol Neurosurg Psychiatry 72: 560-3 Volta U, et al. 2002. Clinical findings and anti-neuronal antibodies in coeliac disease with neurological disorders. Scand J Gastroenterol 37: 1276-81. Tursi A et al 2001. Low prevalence of antigliadin and anti-endomysium antibodies in subclinical/silent celiac disease. Am J Gastroenterol 96: 1507-1510. Hadjivassiliou M, et al. 2003. Dietary treatment of gluten ataxia. J Neurol Neurosurg Psychiatry 74: 1221-24. Will AJ. 2000. The neurology and neuropathy of coeliac disease. Neuropathy and Applied Neurobio 226: 493-96. Cross A, and Golumbek, P. 2003. Neurologic manifestations of celiac disease. Neurology 60: 1566-1568. Hadjivassiliou M, et al. 2002. The humoral response in the pathogenesis of gluten ataxia. Neurology 58: 1221-26.

Celiac.com 02/18/2021 - There has been some evidence to support the idea that patients with anti-GAD ataxia and no gluten sensitivity may benefit form immunosuppressive drugs. A team of researchers recently set out to report the clinical characteristics and treatment of patients with progressive cerebellar ataxia associated with anti-GAD antibodies. The research team included M. Hadjivassiliou, P. G. Sarrigiannis, P. D. Shanmugarajah, D. S. Sanders, R. A. Grünewald, P. Zis & N. Hoggard. They are variously affiliated with the Academic Department of Neurosciences, and the Academic Department of Neuroradiology at the Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Trust, in Sheffield, UK. The team conducted a retrospective review of all 50 patients with anti-GAD ataxia treated at the Sheffield Ataxia Centre over the last 25 years. The rate of anti-GAD ataxia was 2.5% amongst 2,000 patients with progressive ataxia of various causes. Average onset age was 55, with an average duration of 8 years. The researchers found gaze-evoked nystagmus in 26% of subjects, cerebellar dysarthria in 26%, limb ataxia in 44% and gait ataxia in all of them. Nine patients suffered from severe ataxia, 12 from moderate ataxia, and 29 from mild ataxia. Ninety percent of patients had a history other autoimmune diseases. Just over half had a family history of autoimmune diseases. In nearly three out of four patients, baseline MR spectroscopy of the vermis was abnormal at presentation. Thirty-five patients had positive serological evidence of gluten sensitivity. All 35 patients began a gluten-free diet (GFD). Eighteen patients, more than half, improved, while 13 patients stabilized, three patients began the GFD too recently to draw conclusions, and one patient got worse. Sixteen patients received mycophenolate. Seven patients, nearly 45%, improved, while two patients stabilized, six patients began the medication too recently to draw conclusions, and one did not tolerate the drug. There is considerable overlap between anti-GAD ataxia and gluten ataxia. For patients with both conditions, a strict GFD alone is sufficient treatment. However, patients with anti-GAD ataxia and no gluten sensitivity respond well to immunosuppression. Read more in The Cerebellum (2020)

Celiac.com 04/30/2019 - Doctors recognize that patients with gluten sensitivity, whether or not they have enteropathy, or even gastrointestinal symptoms, can suffer from a range of debilitating neurological manifestations. Researchers Panagiotis Zis and Marios Hadjivassiliou of the Academic Department of Neurosciences, Sheffield Teaching hospitals NHS Trust and University of Sheffield, Royal Hallamshire Hospital, Sheffield, UK, recently published an overview of current literature in an effort to assess the available treatment options for the neurological manifestations of gluten-related disorders, specifically, serologically confirmed gluten sensitivity and celiac disease. For patients with gluten sensitivity, ataxia is the most common neurological manifestation, followed by peripheral neuropathy. Other conditions linked to gluten sensitivity and celiac disease and discussed in the review include: epilepsy headache encephalopathy movement disorders cognitive impairment muscle disorders The team found that a strict gluten-free diet is effective in treating neurological manifestations of gluten-related disorders. Rarely, patients need additional immunosuppressive treatment, usually mycophenolate. There have been cases where gluten ingestion triggered schizophrenic episodes in an undiagnosed patient. Meanwhile, a number of studies have shown that a gluten-free diet seems to help improve symptoms of psychological, neurological and inflammatory conditions, including schizophrenia, neuropathy, and diabetes. Stay tuned for more news about gluten and related health issues. Read more in Current Treatment Options in Neurology

Celiac.com 06/12/2020 - What happens in Gluten Ataxia? Well, first, we want every celiac person to know what Gluten Ataxia is to ensure we are on the same "wave length". Gluten Ataxia is an autoimmune disorder in which the antibodies that are released in sensitive individuals when digesting gluten attack part of the brain by mistake. Since Gluten is a protein found in wheat, rye and barley, one would think that gluten exposure would have nothing to do with the brain, but since most people have no trouble with digesting this protein, others have a gluten sensitivity or celiac disease. In some cases the body's reaction to gluten can become quite severe. In these cases, the body starts to attack the central nervous system which may cause gluten ataxia. People who have issues digesting gluten may also develop digestive problems that cause damage to the small intestine. Gluten Ataxia usually starts off with mild symptoms, and gradually become worse over time. When left untreated the condition could lead to permanent damage. There is also evidence that people who suffer from gluten ataxia will show signs of cerebellar atrophy. Cerebellum atrophy is the shrinkage of the cerebellum. The cerebellum if the part of the brain located in the back of the head above the neck. The cerebellum is responsible for movement and has a direct impact on activities such as balance, speech, posture, walking and running. Gluten Ataxia is a relatively new discovery and thus not yet widely known to doctors and other medical professionals. This can make a diagnosis and proper treatment difficult to obtain. However, there are groups of researchers dedicated to spreading information abut this rare condition. As mentioned, it is a progressive condition, which means that symptoms may start off mild and almost unnoticed, and gradually progress to being debilitating. The symptoms of gluten ataxia are similar to symptoms of other ataxia conditions, which can make it tricky to get an accurate diagnosis. The symptoms appear in basic movements, such as walking or arm control, unsteady gait, difficulty walking, and loss of precise movement skills such as the ability to write or button a shirt. Parents should be on the lookout for ataxia symptoms in their kids. Children with celiac disease, specifically those in their early teens, would likely benefit from mental health evaluation. Strict adherence to the gluten-free diet does not mean you will never get gluten ataxia, especially for those who are not strict enough with their gluten-free diets. Some researchers have estimated that potentially up to 41 percent of all people with ataxia of unknown origin may have gluten ataxia. Other studies have indicated much lower numbers. A review of mental health studies indicated a prevalence of roughly 23 percent in patients with unexplained ataxia. In the last eight years or so the celiac community has finally been made aware of "gluten sensitivity" as a legitimate diagnosis. Twenty-five years ago you would not have heard of it, but now it has been given a rightful place along side of celiac disease and dermatitis herpetiformis. The same is true for gluten ataxia, its recent discovery will allow those who have it to say: "Finally, finally, someone is finally listening to me!" Read more at medicalnewstoday.com

Celiac.com 05/09/2013 - Previous studies have shown an immunologic response primarily directed against transglutaminase (TG)6 in patients with gluten ataxia (GA). A team of researchers set out to see if Transglutaminase 6 antibodies could be helpful in the diagnosis of gluten ataxia. The team included M. Hadjivassiliou, P. Aeschlimann, D.S. Sanders, M. Mäki, K. Kaukinen, R.A. Grünewald, O. Bandmann, N. Woodroofe, G. Haddock, and D.P. Aeschlimann. They are variously affiliated with the Departments of Neurology (M.H., R.A.G., O.B.) and Gastroenterology (D.S.S.) at Royal Hallamshire Hospital in Sheffield, UK, the Matrix Biology & Tissue Repair Research Unit (P.A., D.P.A.) of the School of Dentistry at Cardiff University in Cardiff, UK, the Department of Paediatrics (M.M., K.K.) of the School of Medicine at University of Tampere in Finland, and the Department of Biological Sciences (N.W., G.H.) at Sheffield Hallam University in Sheffield, UK. For their prospective cohort study, the team looked at patients from the ataxia, gluten/neurology, celiac disease (celiac disease), and movement disorder clinics based at Royal Hallamshire Hospital (Sheffield, UK) and from the celiac disease clinic at Tampere University Hospital in Tampere, Finland. Patients were broken into groups that included idiopathic sporadic ataxia, gluten ataxia, celiac disease, and neurology, along with healthy control subjects. The team screened all subjects for TG6 antibodies, and conducted duodenal biopsies on all patients with positive blood screens. In addition, they analyzed biopsies from 15 consecutive patients with idiopathic sporadic ataxia and negative serology for gluten-related disorders for immunoglobulin A deposits against TG. They found TG6 antibodies in 21 of 65 (32%) patients with idiopathic sporadic ataxia, in 35 of 48 (73%) patients with GA, in 16 of 50 (32%) patients with celiac disease, in 4 of 82 (5%) neurological control subjects, and in just 2 of 57 (4%) healthy control subjects. The results showed that forty-two percent of patients with GA had enteropathy, as did 51% of patients with ataxia and TG6 antibodies. Five of 15 consecutive patients with idiopathic sporadic ataxia had immunoglobulin A deposits against TG2, 4 of which subsequently tested positive for TG6 antibodies. Follow-up screens showed that one year of gluten-free diet left TG6 antibody levels greatly reduced or undetectable. The study shows that antibodies against TG6 are gluten-dependent and that they seem to be a sensitive and specific indicator of gluten ataxia. Source: Neurology. 2013 Apr 10.

Celiac.com 12/29/2018 - Imbalance and clumsiness may not be the most common symptom of the nervous system related to gluten intolerance but one of the most researched areas. Physicians use the term “ataxia” to describe poor coordination and balance. It can affect your walking and your ability to stand. While many systems contribute to your balance your cerebellum in the brain is the location that organizes all of the information and navigates your movements precisely. Some doctors claim that ataxia is one of the most common disorders produced by gluten in relationship to our nervous system. Poor coordination and clumsiness does occur with gluten intolerance and affects children as well as adults. Evidence suggests that this is all due to the immune system’s reaction to gluten itself. In people who are genetically at-risk for gluten sensitivity, gluten induces an immune attack against the protein gliadin and this antibody not only attacks gliadin in the gut but also attacks tissues far away from the intestines. In this case, through the bloodstream, these antibodies travel to the cerebellum and attack the Purkinje cells. As these cells become inflamed from the immune attack, the ability to integrate all the “balance information” is impaired, and coordination suffers. Symptoms like poor balance and coordination can result. A study in Britain examined 224 people with ataxia disorders. Some had an inherited disorder of ataxia, some had ataxia combined with other neurologic symptoms, and some simply had ataxia without known cause. Of those that were without known cause, 41 percent were found to have anti-gliadin antibodies supporting gluten sensitivity as a cause. In another study, ten patients with headaches and/or clumsiness were placed on a gluten-free diet. Over time, nine of the ten showed a beneficial response in all symptoms. The evidence is overwhelming. The presence of gluten antibodies, shrinkage of the cerebellum and the dramatic response to dietary change all support gluten as the cause.

Celiac.com 10/03/2018 - Gluten-related disorders include the full spectrum of adverse clinical symptoms and conditions triggered by eating gluten. A team of researchers recently set out to review the available medical literature concerning MDs and gluten sensitivity with and without enteropathy. The research team included A Vinagre-Aragón, P Zis, RA Grunewald, and M Hadjivassiliou, with the Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, South Yorkshire, UK. Celiac disease or gluten sensitive enteropathy is the most common manifestation, but clinicians have reported a number of extra-intestinal manifestations, which may occur without enteropathy. Gluten sensitivity is another term that has been used to include all gluten-related disorders, including those where blood tests show antibodies to gluten in the absence of any enteropathy. Gluten ataxia is the most common extra-intestinal neurological manifestation, and has been well documented. Clinicians have reported movement disorders related to gluten sensitivity. To assess the current medical literature on movement disorders and gluten sensitivity, both with and without enteropathy, the team conducted a systematic search on the PubMed database, and included 48 articles that met the inclusion criteria into the present review. This review demonstrates that the range of gluten related movement disorders goes beyond gluten ataxia, and shows that the majority of patients with gluten-related disorders benefit from a gluten-free diet. Read the full review at: Nutrients. 2018 Aug 8;10(8). pii: E1034. doi: 10.3390/nu10081034.

Celiac.com 08/29/2018 - Up to one in twelve patients with gluten sensitivity develops neurological symptoms such as ataxia, dementia, seizures or peripheral neuropathy, though the reasons for this are still poorly understood. As a means of better understanding the immunological mechanisms behind this reality, a team of researchers recently reported the case of a 68‐year‐old male patient suffering from progressive ataxia and dementia associated with chronic diarrhea, and both elevated IgG and IgA antigliadin‐antibodies. The research team included Michel Mittelbronn, Jens Schittenhelm, Gellert Bakos, Rob A. De Vos, Manfred Wehrmann, Richard Meyermann, and Katrin Bürk. They are variously affiliated with the Institute of Brain Research at the University of Tübingen, and the Institute for Cell Biology, Department of Immunology at the University of Tübingen, Tübingen, Germany, the Neurological Institute/Edinger Institute, Goethe University Medical School, Frankfurt, the Department of Pathology, St. Georg Hospital, Leipzig, Germany, and with the Laboratory for Pathology, Enschede, the Netherlands. Autopsy indicated that frequent argyrophilic glial and neuronal inclusions within the basal nucleus of Meynert were the structural markers of the cognitive decline. The patient showed substantial neuronal loss in the cerebellar cortex and the inferior olives, along with infiltrating CD8+/perforin+/granzyme B+ cells, and reactive astrogliosis and microglial activation. In patients with gluten sensitivity and neurological disease, it is likely that CD8+ cytotoxic T and NK cells function as effector cells that trigger neuronal cell death, and thus might play some role in triggering cerebellar symptoms in gluten ataxia cases. The team concludes by noting that an absence of B‐ or plasma cells, along with multiple CD8+, granzyme B and perforin expressing cells in ataxia‐associated brain areas, indicates pronounced cytotoxic effects in neuro-pathogenesis of gluten sensitivity. This is one of the first reports to indicate that CD8+, perforin+, and granzyme B+ effector cells infiltrate the cerebellum and inferior olives in cases of gluten ataxia. Read more in: Neuropathology

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. Source: Cerebellum & Ataxias 20174:20

Celiac.com 11/25/2017 - We have long known that gluten intolerance, both celiac disease and gluten sensitivity, are highly associated with neurological symptoms. Migraines, ataxia (unstable gait), seizures, schizophrenia – the list is long. But a recent research study just published last month sheds some new light on exactly what the mechanism may be. Understanding why these debilitating symptoms occur as a result of a gluten intolerance will, hopefully, go a long way toward increased awareness among the lay public and clinicians alike. It is certainly true that too many millions of Americans suffer the effects of a gluten intolerance unknowingly. They only know that they feel unhealthy but have no idea that gluten is the culprit. The digestive tract is sometimes called the second brain. Some say that is because it is second in importance to the brain. After all, if the food that is consumed doesn't turn into fuel that can effectively feed the 10 trillion cells in the body, those cells will be unable to perform their job and keep the body healthy. In fact, poor digestion is absolutely linked to poor health and increased onset of degenerative disease. This article in Current Pain and Headache Reports looks at another possibility for naming the digestive tract the second brain, and it simply stems from anatomy. The digestive tract actually has a ‘mind of its own'; more correctly, it has a nervous system of its own, called the enteric nervous system. ‘Enteric' simply means having to do with the intestine. This nervous system, according to research, is very similar to the brain housed in the head in that it is bathed in similar chemicals (called neurotransmitters – which, interestingly enough, are mostly produced in the gut!). It sends and receives impulses and records experiences and is influenced by emotions. Some proof of the latter: Have you ever been nervous and had diarrhea? This particular study stated that experiencing ‘adverse events' created a state of hypervigilance (a state of being overly responsive - not a good thing) in the nervous system which was associated with migraines and IBS. Such ‘hypervigilance' was previously only associated with the central nervous system – the one attached to the brain in the head. This group of researchers suggests that the initiation of hypervigilance may very likely lie in the enteric nervous system also. What this means is that if the small intestine is genetically sensitive to gluten and gluten is ingested, it could set off a nervous system response that could create disabling diseases, such as migraines and IBS, but likely others as well. The take-away message is that it is truly critical to diagnose gluten intolerance as soon as possible. Once that hurdle is surmounted it then needs to be followed with a program of nutrition, lifestyle and diet that will ensure healing of the small intestine and a ‘calming' of the hypervigilant nervous system. You may sometimes hear this referred to as healing a leaky gut. Here at HealthNOW we often see this clinically in patients who seem intolerant to many different foods and can't seem to enjoy stable improvement of their symptoms, even after they eliminate gluten from their diet. The reason for this insufficient improvement is that a comprehensive follow-up program is missing – a program that addresses what we call the Secondary Effects of Gluten. This entails evaluating for any other food sensitivities, cross reactive foods, a tendency towards autoimmune disease, the presence of any infectious organisms, healing the leaky gut, balancing the probiotic population, and more. While increasing awareness of the presence of gluten intolerance is absolutely critical, neglecting the secondary effects, as mentioned above, can result in long-term ill health that is truly preventable. Have you experienced such symptoms? Have you removed gluten but are only partially healthier? I'd love to hear from you. To your good health.

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.

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