Progressive Myoclonic Ataxia 

What Is Progressive Myoclonic Ataxia?

[dropcap]P[/dropcap]rogressive myoclonic ataxia is a movement disorder characterized by defective muscular coordination of a muscle group with its origin in the brain.

The pathology is in the cerebellum.¹

Myoclonus in a context of progressive ataxia suggests one clinical form of the Ramsay-Hunt syndrome (progressive myoclonic ataxia, PMA), whose most frequent causes are: celiac disease, mitochondriopathies, some spino-cerebellar degenerations, and some late metabolic disorders.²

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

• Relationship between progressive myoclonic ataxia and celiac disease. Progressive myoclonic ataxia is an associated immune disorder in celiac disease. The association between celiac disease and other immune disorders may be due to the sharing of a common genetic background, such as HLA antigens. However, in a very large study, involving 909 patients with celiac disease, it was found that the development of immune disorders in celiac disease was clearly related to the duration of exposure to gluten.³

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

Progressive myoclonic ataxia has increased frequency in celiac disease.⁴,⁵

What Are The Symptoms Of Progressive Myoclonic Ataxia?

• Progressive myoclonic ataxia is marked by brief, rapid contraction of a muscle or a group of muscles.

How Does Progressive Myoclonic Ataxia Develop?

• Progressive myoclonic ataxia is thought to result from vitamin E deficiency and gluten exposure with immune mechanism.⁶
• The mechanism is unresolved and requires a more rigorous approach to examine the role of disordered metabolism secondary to trace vitamin deficiency as well as immunological dysfunction.⁷

Does Progressive Myoclonic Ataxia Respond To Gluten-Free Diet?

Response of progressive myoclonic ataxia to gluten free diet varies.

6 Steps To Improve Progressive Myoclonic 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 progressive myoclonic ataxia 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.⁸
• 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.⁹
• 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.⁹
• 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.⁹.
• 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.⁹
• 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.⁹
• Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.⁹[/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 green leafy vegetables such as lettuce and kale, also 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 medications deplete vitamin E which predisposes to progressive myoclonic ataxia. 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

• Zenicol (Orlistat®) depletes Vitamin E.

CHOLESTEROL DRUGS

• Colestid® and Questran® deplete Vitamin E.

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

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

• 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.
• Vitamin E as prescribed following blood test for status.

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 Progressive Myoclonic Ataxia In Celiac Disease and/or Gluten Sensitivity?

RESEARCH STUDY SUMMARIES

“Vitamin E levels in patients with celiac disease.” This study investigating vitamin E status in patients with active celiac disease and patients on a GFD demonstrated that plasma concentrations of vitamin E were significantly lower in untreated celiac disease patients. Vitamin E correction may offer benefit to newly diagnosed and those who fail to adhere to strict GF diet.¹⁰

“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 patients with celiac disease than in controls.¹¹

CASE REPORT SUMMARIES

“Atypical neurological involvement associated with celiac disease.” This case report describes asymptomatic celiac disease associated with stimulus induced myoclonus, stiff gait, and abnormalities of eye movement. A 31 year old female experienced jerks triggered by sudden sound or touch since her childhood, and recurrent episodes of nausea and vomiting. She had spasticity in all four limbs, brisk deep tendon reflexes with bilateral Achilles clonus, and flexor plantar reflexes. CT showed mild cortical cerebral atrophy. Serum anti-endomysium antibodies were negative, serum antigliadin antibodies IgA and IgG were positive. Small bowel biopsy revealed subtotal villus atrophy.

Celiac disease has to be considered in the differential diagnosis of patients presenting with neurologic symptoms of unknown origin, even in the abscence of gastrointestinal symptoms. Nausea and vomiting and stimulus-sensitive myoclonus responded well to a Gluten Free Diet but not the general neurologic condition.¹²

“Progressive myoclonic ataxia associated with coeliac disease.” This case report describes onset of a neurological syndrome dominated by action and stimulus sensitive myoclonus of cortical origin with mild ataxia and infrequent seizures. Condition progressed in 2 older patients without overt features of Celiac Disease or nutritional deficiency on a strict Gluten Free Diet. Post-mortum examination of the brain in one case showed selective symmetrical atrophy of the cerebellar hemispheres with Purkinje cell loss and Bergmann astrocytosis, and with preservation of the cerebral hemispheres and brainstem. Celiac Disease should be considered in the differential diagnosis of all patients presenting with a progressive myoclonic ataxic syndrome.¹³

Sources:

1. Bhatia KP, Brown P, Gregory R, et al. Progressive myoclonic ataxia associated with coeliac disease. The myoclonus is of cortical origin, but the pathology is in the cerebellum. Brain. Oct 1995;118(Pt 5):1087-93. [↩]
2. Borg M. Symptomatic myoclonus. Neurophysiol Clin. 2006 Sep-Dec;36(5-6):309-18. [↩]
3. La Villa G, Pantaleo P, Tarquini R, Cirami L, Perfetto F, Mancuso F, Laffi G. Multiple immune disorders in unrecognized celiac disease: a case report. World J Gastroenterol. 2003;9(6):1377-1380. [↩]
4. Hanagasi HA, Gurol E, Sahin HA, Emre M. Atypical neurological involvement associated with celiac disease. European Journal of Neurology. 2001;8:67-69 [↩]
5. Bhatia KP, Brown P, Gregory R, et al. Progressive myoclonic ataxia associated with coeliac disease. The myoclonus is of cortical origin, but the pathology is in the cerebellum. Brain. Oct 1995;118(Pt 5):1087-93. [↩]
6. Bhatia KP, Brown P, Gregory R, et al. Progressive myoclonic ataxia associated with coeliac disease. The myoclonus is of cortical origin, but the pathology is in the cerebellum. Brain. Oct 1995;118(Pt 5):1087-93. [↩]
7. Wills AJ. The neurology and neuropathology of celiac disease. Neuropathology and Applied Neurobiology. 2000:26:493-496. [↩]
8. 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. [↩]
9. 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. [↩] [↩] [↩] [↩] [↩] [↩]
10. Hozyasz KK, Chelchowska M, Laskowska-Klita T. Vitamin E levels in patients with celiac disease. Medycryna Wieku Rozwojowego. Oct-Dec 2003; 7(4 Pt 2):593-604. [↩]
11. 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. [↩]
12. Hanagasi HA, Gurol E, Sahin HA, Emre M. Atypical neurological involvement associated with celiac disease. European Journal of Neurology. 2001;8:67-69 [↩]
13. Bhatia KP, Brown P, Gregory R, et al. Progressive myoclonic ataxia associated with coeliac disease. The myoclonus is of cortical origin, but the pathology is in the cerebellum. Brain. Oct 1995;118(Pt 5):1087-93. [↩]