Ataxia, Gluten

What Is Gluten Ataxia?

[dropcap]G[/dropcap]luten ataxia is a degeneration of the cerebellum (lower brain) caused by an immune reaction to gluten that is characterized by positive anti-gliadin antibodies, motor abnormalities including upper or lower limb ataxia, gait ataxia, and dysarthria (impaired muscles for producing speech). Ataxia involves lack of coordination in purposeful movements of the body like lifting objects and walking.¹

In addition to anti-gliadin antibodies, patients with gluten ataxia have oligoclonal bands in their cerebrospinal fluid, inflammation at the cerebellum, and anti-Purkinje cell antibodies.¹

The duration of immune reactivity to gluten that results in Purkinje cell damage in the cerebellum correlates with the severity and the presence of cerebellar atrophy (loss of brain tissue).² That is, more gluten damages more brain cells.

Studies suggest that persons with gluten ataxia may have additional antibodies that react with Purkinje cells and are not present in patients with anything other than gluten ataxia alone. It seems likely that the Purkinje cells of the cerebellum share epitopes with gliadin proteins.¹

Q: What is Purkinje cell damage?

A: Purkinje cell damage is a finding commonly encountered in cerebellar degeneration. Purkinje cells are large flask-shaped neurons lying between the molecular and granular layer of the cerebellar cortex (outer layer) that have dendrites (branched processes of the cell body that conduct messages to the cell body) extending to the cortex of the cerebellum and into the white matter of the brain.

Purkinje cells are sensitive to vitamin E status in the body.

What Is Gluten Ataxia In Celiac Disease and/or Gluten Sensitivity?

• Gluten ataxia is associated with celiac disease. Most patients with gluten ataxia have negligible gastrointestinal symptoms.³
• Ataxia may be the sole presentation in latent celiac disease. Therefore, antigliadin antibody testing for celiac disease is essential at first presentation of patients with sporadic idiopathic ataxia.³
• A study by Pennisi et al. reveals that patients with celiac disease seem to exhibit a relatively distinct pattern of cortical excitability to TMS assessment (transcranial magnetic stimulation), suggesting that even asymptomatic patients might disclose a subclinical neurological involvement.⁴
• Several studies have shown that screening for celiac disease and gluten sensitivity is beneficial in patients with ataxias.¹

How Prevalent Is Gluten Ataxia In Celiac Disease and/or Gluten Sensitivity?

• Celiac disease was found in 24% of patients with gluten ataxia.⁵
• Gluten ataxia is the most common neurological complication of gluten sensitivity, affecting 100% of patients with gait ataxia, and as high as 68% of patients with idiopathic ataxia.²

What Are The Symptoms Of Gluten Ataxia?

• Gluten ataxia is marked by lack of coordination in movement and locomotion.

How Does Gluten Ataxia Develop In Celiac Disease and/or Gluten Sensitivity?

• Neurologic dysregulation. Gluten ataxia results from an unclear relation between gluten sensitivity, malabsorption, nutritional deficiencies, and ataxia. Immunologically mediated neural damage implies an autoimmune mechanism from exposure to gluten.⁶,⁷
• Nutritional deficiency. The level of markers for vitamin E, a powerful antioxidant, were significantly lower in celiac disease than in controls.⁸

Does Gluten Ataxia Respond To Gluten-Free Diet?

Yes. Patients improve on a gluten free diet.⁹Symptoms may persist, but gluten free diet prevents further deterioration. Prompt diagnosis of gluten ataxia affords complete resolution of symptoms after strict adherence to a gluten free diet.¹⁰

6 Steps To Improve Gluten Ataxia In Celiac Disease and/or Gluten Sensitivity:

• [dropcap]1[/dropcap]Remove the Trigger. Maintain a Strict, Nutritious Gluten Free Diet:

[box type=”shadow” ]Treatment. This condition responds to the complete elimination of gluten, which is the required treatment that improves both brain and gut health.

• Gut health is the foundation to restore ALL health. Restored health will enable you to maintain a strict gluten free diet, just as other life tasks will be easier.
• A strict gluten free diet means removing 100% of wheat, barley, rye and oats from the diet.
• Cutting out bread and other obvious sources of gluten is not good enough for recovery. Even 1/8th teaspoon of flour or bread crumb is enough to sustain the inflammation that is damaging your small intestine, causing increased permeability (leaky gut) and allowing undigested gluten to enter your body where it can damage structures and function, and instigate immune inflammatory responses.

Correct Your Individual Nutritional Needs.

• Eat foods that can replenish missing nutrients. Find them under NUTRIENT DEFICIENCIES.
• Take nutritional supplements as needed. Find them under NUTRIENT DEFICIENCIES.

Recovery. You should begin to feel better within a week and notice more energy as inflammation subsides and the  absorbing cells that make up the surface lining of your small intestine are better able to function.

• Intestinal lining cells are replaced every 5 days. The healing process is like sunburn where the damaged surface layer of skin sloughs off and is replaced with new normal cells.
• Leaky gut normally resolves in two month after starting a gluten free diet and brings about a big improvement in health. Improvement in intestinal permeability precedes morphometric recovery (cell appearance and structure) of the small intestine in celiac disease.¹¹
• The intestinal lining may take up to a year to heal.[/box]

• [dropcap]2[/dropcap] Reduce Inflammation. Foods to Eat and Foods Not to Eat:

Because gluten is inflammatory, eliminate OTHER inflammatory foods from your diet to reduce an additive effect to gluten. At the same time, try to eat foods that reduce inflammation (anti-inflammatory).

[box type=”shadow” ]Here Are Major Inflammatory Food Types That Reduce Healing:

• Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.¹²
• Allergenic Foods. Includes foods that trigger the immune sytem to produce IgE antibodies. Allergy testing is the usual way to discover these offending foods.
• Shelf Stable Processed Foods. Includes any that contain additives and preservatives. Look for them on the nutrition label of the box or package. Additives and preservatives also disrupt intestinal permeability causing leaky gut.¹²
• Fats. Limit deep fried foods, trans-fats, saturated fats (animal fat/butter), and EXCESSIVE omega-6 fatty acid oils like corn oil. Rancid fats, sodium caprate (a medium chain fat), and sucrose monester fatty acid (a food grade surfactant) induce significant disruption of the intestinal barrier that causes leaky gut.¹².
• Excessive Refined White Flours (bran layer removed). Includes products made from them such as cookies, bread, cakes, pies. Bran contains the vitamins and minerals that metabolize grains and slows the otherwise rapid entry of sugar from their digestion into the bloodstream. Also disrupt intestinal permeability causing leaky gut.¹²
• Refined Sugars.  Includes white sugar, corn fructose and high fructose corn syrup.
• Certain Spices. Includes paprika and cayenne pepper which disrupt intestinal permeability causing leaky gut.¹²
• Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.¹²[/box]

[box type=”shadow” ]Here Are Important Anti-Inflammatory Food Types to Promote Health:

• Fruits. Contain ample amounts of vitamins, minerals and phytochemicals which are naturally occuring components in plants that detoxify toxins, carcinogens (reducing the risk by 50%) and mutagens.
• Non-Starchy Vegetables. Support intestinal integrity and provide ample amounts of vitamins, minerals and phytochemicals. Includes lettuce, kale, onion, broccoli, garlic, and others.
• High Quality Complex Carbohydrates. Provide vitamins, minerals, and fiber while boosting serotonin levels to help you relax and feel calm. Includes whole grains, legumes, and root vegetables such as carrots, parsnips, sweet potatoes, turnips, red beets, and others.
• Antioxidants. Protect the body from inflammatory oxidant molecules that continually occur and help us handle stress and reduce irritability. Includes vitamin C-containing foods such as lemon, grapefruit, apricot, Brussels sprouts and strawberries, and others. Also, includes vitamin E-containing foods such as nuts, seeds, avocado, olive oil, and others. Cocoa is good, too.
• Omega-3 Fatty Acids. Balance opposing omega-6 fatty acids and bad fats. Fish sources includes tuna, salmon, cod, and others. Plants sources include flax, chia seeds, canola oil, and others.
• Probiotics. Supply normal microbes needed for colon health and health of the body such as these fermented foods: yogurt, kefir, and unpasteurized apple cider vinegar.
• Prebiotics/ High Fiber Foods.  Food with fiber keeps our population of colonic microbes healthy.
• Protective Herbs and Spices.  See below #6 below for examples.[/box]

• [dropcap]3[/dropcap] Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

 

• [dropcap]4[/dropcap] Manage Your Medications Safely:

[box type=”shadow” ]

Certain prescription drugs can cause vitamin E deficiency. Ask your doctor or pharmacist about this possible adverse effect if you are taking any of the drugs listed below. Do not stop prescribed medications without supervision.

This is not a complete listing.

WEIGHT LOSS DRUGS THAT BIND FAT also interfere with absorption of some nutrients.

• Zenicol (Orlistat®) depletes Vitamin E.

CHOLESTEROL DRUGS

• Colestid® and Questran® deplete Vitamin E.[/box]

• [dropcap]5[/dropcap]Nutritional Supplements To Help Correct Deficiencies:

[box type=”shadow” ]

The type and quantity of nutritional supplements that may be needed depend on which nutrients are deficient.

• Vitamin E supplements as prescribed to correct deficiency.
• Multivitamin/mineral combination once a day is useful to improve overall nutrient levels. This is a safe dose, but always check with your doctor to avoid interactions with medications.

Storage Note: Store container tightly sealed, away from heat, moisture and direct light to avoid loss of potency. That is, in a safe kitchen cabinet – not in the bathroom or on the kitchen table.[/box]

• [dropcap]6[/dropcap]Manage Natural Remedies: 

[box type=”shadow” ]Hydration:

• Eight glasses of water are recommended per day unless there is a contraindication such as kidney or heart disease. The Institute of Medicine recommends approximately 2.7 liters (91 ounces) of total water, from all beverages and foods, each day for women and 3.7 liters (125 ounces) daily of total water for men.
• If you are thirsty, drink water. Add fresh, squeezed lemon to water. Lemon is anti-inflammatory, alkalizing and provides vitamin C.
• Hydration Test: Urine should be pale yellow. Fingertips should be plump, without pruning but this may not be reliable when fingers are swollen with edema. Lips should be plump, without puckering. The feeling of thirst can be unreliable.
• What is wrong with soda, coffee, tea, and alcohol? These drinks are dehydrating, increase acid, and deplete nutrients.[/box]

[box type=”shadow” ]Carminatives. The following  anti-inflammatory plant sources called carminitives help heal the digestive tract. They also tone the digestive muscles which improves peristalsis, thus aiding in the expulsion of gas from the stomach and intestine to relieve digestive colic and gastric discomfort.

Carminative Food Remedies:

• Raspberry.
• Carrot is also a cleansing digestive tonic.
• Grape is also bile stimulating and a cleansing remedy for sluggish digestion and laxative.
• Redbeets also stimulate and improve digestion and are easily digested.
• Cabbage also stimulates and improves digestion and is also a liver decongestant.
• Lettuce also stimulates and improves digestion and is also an alterative, meaning it improves the function of organs involved with the digestion and excretion of waste products to bring about a gradual change.
• Potatoes are antispasmodic (due to atropine like properties) and a liver remedy.

Carminative Herb Remedies:

• Sage is also a digestive, astringent, bile stimulant and energy tonic that heals the mucosa.  Drink as tea or use in cooking.
• Chamomile, lemon balm, and fennel, (as a tea) also help relieve nervous tension.
• Parsley also relieves indigestion.
• Rosemary as a tea and in cooking also is a nervous system tonic for stress and fatigue, bile stimulant, and can relieve headaches and indigestion.
• Thyme is also soothing remedy useful for stimulating digestion of rich, fatty foods.

Carminative Spice Remedies:

• Cloves are also antispasmodic.
• Nutmeg is also useful for indigestion.
• Ginger.[/box]

[box type=”shadow” ]Exercise Helps:

Exercise improves circulation and rids the body of toxins.

• Walking is aerobic exercise that reconditions the whole body to improve stamina. Read more about Exercise and Fitness.
• Weight training builds muscle. Read more about Exercise and Fitness.
• Stretching improves flexibilty. Read more about Exercise and Fitness.

Note: Exercise is important, but the amount and type of exercise undertaken depends on your health. Your first priority is to heal. [/box]

What Do Medical Research Studies Tell About Gluten Ataxia In Celiac Disease and/or Gluten Sensitivity?

RESEARCH STUDY SUMMARIES

“Excitability of the motor cortex in de novo patients with celiac disease.” This study investigating the profile of cortical excitability to Transcranial Magnetic Stimulation (TMS) in a group of 20 newly diagnosed celiac disease patients found that a pattern of cortical excitability characterized by “disinhibition” and “hyperfacilitation” was found in patients with celiac disease. Immune system dysregulation might play a central role in triggering changes of the motor cortex excitability.

Patients underwent a screening for cognitive and neuropsychiatric symptoms by means of the Mini Mental State Examination and the Structured Clinical Interview for DSM-IV Axis I Disorders, respectively. Instrumental exams, including electroencephalography and brain computed tomography, were also performed. Cortico-spinal excitability was assessed by means of single and paired-pulse TMS using the first dorsal interosseus muscle of the dominant hand. TMS measures consisted of resting motor threshold, motor evoked potentials, cortical silent period (CSP), intracortical inhibition (ICI) and facilitation (ICF). None of the patients was on gluten-free diet. A group of 20 age-matched healthy controls was used for comparisons.

RESULTS: Patients with celiac disease showed a significantly shorter CSP (78.0 vs 125.0 ms, p<0.025), a reduced ICI (0.3 vs 0.2, p<0.045) and an enhanced ICF (1.1 vs 0.7, p<0.042) compared to controls. A dysthymic disorder was identified in five patients. The effect size between dysthymic and non-dysthymic patients indicated a low probability of interference with the CSP (Cohen’s d -0.414), ICI (-0.278) and ICF (-0.292) measurements.¹³

“Dietary treatment of gluten neuropathy.” This study investigating the effect of a gluten-free diet in patients with idiopathic sensorimotor axonal neuropathy and circulating antigliadin antibodies found that the gluten-free diet may be a useful therapeutic intervention for patients with gluten neuropathy.

There was a significant difference in the change of sural sensory action potentials (pre-defined primary endpoint), with evidence of improvement in the intention-to-treat group and deterioration in the control group. Subjective change in neuropathy symptoms also showed significant differences, with patients in the intention-to-treat group reporting improvement and those in the control group reporting deterioration.

Consecutive patients underwent baseline neurophysiological assessment and were offered a gluten-free diet. Those who went on the diet formed the intention-to-treat group and those who did not were the control group. Repeat neurophysiological assessment and subjective evaluation of neuropathy symptoms were performed at 1 year. A total of 35 patients participated in the study, with 25 patients going on the diet and 10 not doing so.⁹

“Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics.” This study investigating celiac disease in patients with various ataxia demonstrated the following clinical characteristics for gluten ataxia: ocular signs in 84%, dysarthria in 66%, upper limb in 75%, lower limb in 90%, and gait ataxia in 100%; and atrophy of cerebellum in 79%, white matter hyperintensities in 19%; and sensorimotor axonal neuropathy in 45%. GI symptoms were present in only 13%, Celiac Disease in 24%, and HLA DQ2 in 72% of patients.⁵

“The humoral response in the pathogenesis of gluten ataxia.” This study investigating gluten sensitivity in patients with sporadic ataxia demonstrated susceptibility of both the central and peripheral nervous system to the immune response triggered by sensitivity to gluten. Researchers demonstrated antibodies against Purkinje cells in patients with gluten ataxia.

Autopsy in two patients showed lymphocytic infiltration of the cerebellum, damage to the posterior columns of the spinal cord, and sparse infiltration of the peripheral nerves. Antibodies to endomysium may be useful in the identification of those patients with cryptic celiac disease, but may not be found in patients with gluten sensitivity and histologically normal mucosa.⁷

“Oxidative stress in subjects affected by celiac disease.” This study investigating the role of oxidative stress in celiac disease demonstrated the level of markers for vitamin E were significantly lower in celiac disease than in controls.¹⁴

Sources:

1. Jessica R. Jackson, William W. Eaton, Nicola G. Cascella, Alessio Fasano, and Deanna L. Kelly Neurologic and Psychiatric Manifestations of Celiac Disease and Gluten Sensitivity. Psychiatr Q. Mar 2012; 83(1): 91–102. doi:  10.1007/s11126-011-9186-y [↩] [↩] [↩] [↩]
2. Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, et al. Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet. Nov 14, 1998;352(9140):1582-5. [↩] [↩]
3. Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, et al. Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet. Nov 14, 1998;352(9140):1582-5. [↩] [↩]
4. Pennisi G, Lanza G, Giuffrida S, Vinciguerra L, Puglisi V, Cantone M, Pennisi M, D’Agate CC, Naso P, Aprile G, Malaguarnera G, Ferri R, Bella R. Excitability of the motor cortex in de novo patients with celiac disease. PLoS One. 2014 Jul 25;9(7):e102790. doi: 10.1371/journal.pone.0102790. eCollection 2014. [↩]
5. Hadjivassiliou M, Grunewald R, Sharrack B, et al. Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics. Brain. Mar 2003;126(Pt 3):685-91. [↩] [↩]
6. Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, et al. Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet. Nov 14, 1998;352(9140):1582-5. [↩]
7. Hadjivassiliou M, Boscolo S, Davies-Jones, et.al. The humoral response in the pathogenesis of gluten ataxia. Neurology. Apr 23, 2002;58(8):1221-6. [↩] [↩]
8. Odetti P, Valentini S, Aragno I, Garibaldi S, Pronzato MA, Rolandi E, Barreca T. Oxidative stress in subjects affected by celiac disease. Free Radical Research. Jul 1998;29(1):17-24. [↩]
9. Hadjivassiliou M, Kandler RH, Chattopadhyay AK, Davies-Jones AG, Jarratt JA, Sanders DS, Sharrack B, Grünewald RA. Dietary treatment of gluten neuropathy. Muscle Nerve. 2006 Dec;34(6):762-6. [↩] [↩]
10. Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, et al. Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet. Nov 14, 1998;352(9140):1582-5. [↩]
11. Cummins AG, Thompson FM, Butler RN, et al. Improvement in intestinal permeability precedes morphometric recovery of the small intestine in coeliac disease. Clinical Science. Apr 2001;100(4):379-86. [↩]
12. Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [↩] [↩] [↩] [↩] [↩] [↩]
13. Pennisi G, Lanza G, Giuffrida S, Vinciguerra L, Puglisi V, Cantone M, Pennisi M, D’Agate CC, Naso P, Aprile G, Malaguarnera G, Ferri R, Bella R. Excitability of the motor cortex in de novo patients with celiac disease. PLoS One. 2014 Jul 25;9(7):e102790. doi: 10.1371/journal.pone.0102790. eCollection 2014. [↩]
14. Odetti P, Valentini S, Aragno I, Garibaldi S, Pronzato MA, Rolandi E, Barreca T. Oxidative stress in subjects affected by celiac disease. Free Radical Research. Jul 1998;29(1):17-24. [↩]