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A. CT scan shows coarse calcification. B. MRI shows frontal atrophy. C: Intra-operative leptomeningeal angiomatosis. Courtesy intechopen.com "Epilepsy Surgery in Children" by V. Terra, A, Sakamoto, H. Machado
A. CT scan shows coarse calcification. B. MRI shows frontal atrophy (shrinkage). C: Intra-operative angiomatosis. Courtesy intechopen.com “Epilepsy Surgery in Children” by V. Terra, A Sakamoto, H. Machado

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What Is Cortical Calcifying Angiomatosis?

[dropcap]C ortical calcifying angiomatosis is a cortical vascular (brain blood vessel) abnormality that is characterized by calcification of blood vessels and is usually present in the parietal or occipital cortical and subcortical regions of the brain.1

The brain has 2 sides or hemispheres. Each side has 4 lobes, making 8 lobes altogether: 2 frontal lobes behind the forehead, 2 temporal lobes at ear level, 2 occipital lobes make up the rear of the brain, and 2 parietal lobes located behind the frontal lobes and above the temporal lobes.

Q: How do calcifications of blood vessels appear?

A: Calcium deposits are jagged owing to natural crystal formation by calcium, which is a mineral.

What Is Cortical Calcifying Angiomatosis In Celiac Disease and/or Gluten Sensitivity?

  • Cortical calcifying angiomatosis is an associated neurologic disorder of celiac disease that is resistant to epilepsy treatment.
  • A cortical vascular abnormality with patchy pial angiomatosis, fibrosed veins, and large jagged microcalcifications that was found on resection of the right occipital lobe is described below in a medical case report.
  • The second case report below describes migraine with severe hypertension and brain swelling that did not respond to steroid treatment.

How Prevalent Is Cortical Calcifying Angiomatosis In Celiac Disease and/or Gluten Sensitivity?

Cortical calcifying angiomatosis is associated with celiac disease but the prevalence is not established.2,3

What Are The Symptoms Of Cortical Calcifying Angiomatosis?

Cortical calcifying angiomatosis is marked by neurological abnormalities including:

  • Seizures.
  • Migraine.
  • Intracranial hypertension (high blood pressure due to problems within the brain rather than the heart or blood vessels).

How Does Cortical Calcifying Angiomatosis Develop In Celiac Disease and/or Gluten Sensitivity?

  • Cortical calcifying angiomatosis results from folate deficiency in celiac disease.4

Does Cortical Calcifying Angiomatosis Respond To Gluten-Free Diet?

Yes. Seizures may be stabilized by gluten restriction and folate supplementation.5

6 Steps To Improve Cortical Calcifying Angiomatosis In Celiac Disease and/or Gluten Sensitivity:

  • [dropcap]1 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.6
  • The intestinal lining may take up to a year to heal.[/box]
  • [dropcap]2  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.7
  • 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.7
  • 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.7.
  • 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.7
  • 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.7
  • Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.8[/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  Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

 

  • [dropcap]4  Manage Your Medications Safely:

[box type=”shadow” ]

Certain prescription drugs cause folate deficiency which is the cause of cortical calcifying angiomatosis. In particular, notice that the anti-convulsant drugs used for seizures are a cause of calcifications in the brain.

Ask your doctor or pharmacist about this possible adverse effect if you are taking any of the drugs listed below and how to supplement for the depleted nutrients. Do not stop prescribed medications without supervision.

This is not a complete listing.

FEMALE HORMONES disrupt intestinal permeability.

  • Oral Contraceptives (Norinyl®, Ortho-Novum®, Triphasil®, and others) deplete Folic Acid and others.
  • Oral Estrogen/Hormone Replacement (Evista®, Prempro®, Premarin®, Estratab® and others) deplete Folic Acid and others.

DIURETICS

  • Potassium Sparing Diuretics (Midamor®, Aldactone®, Dyrenium® and others) deplete Folic Acid and others.

DIABETIC DRUGS 

  • Metformin® depletes Folic acid and others.

ANTI-INFLAMMATORIES disrupt intestinal permeability.

  • Corticosteroids (Prednisone, Medrol®, Aristocort®, Decadron) deplete Folic Acid and others.
  • NSAIDS (Motrin®, Aleve®, Advil®, Anaprox®, Dolobid®, Feldene®, Naprosyn® and others) deplete Folic acid and others.
  • Aspirin and Salicylates deplete Folic acid and others.

ANTICONVULSANTS

  • Phenobarbital and Barbituates; and Dilantin®, Tegretol®, Mysoline®, Depakane/Depacon® deplete Folic Acid and others.

ANTIBIOTICS  disrupt intestinal permeability.

  • Gentomycin, Neomycin, Streptomycin, Cephalosporins, Penicillins deplete B Vitamins and others.

ANTACIDS / ULCER MEDICATIONS

  • Pepcid®, Tagamet®, Zantac® deplete Folic Acid and others.
  • Magnesium and Aluminum Antacid preparations (Gaviscon®, Maalox®, Mylanta®) deplete Folic Acid and others.
  • Alka Seltzer®, Baking Soda deplete Folic Acid  and others.

CHOLESTEROL DRUGS

  • Colestid® and Questran® Folic acid and others.[/box]
  • [dropcap]5 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.

  • 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.
  • Folic acid as prescribed following a blood test for serum level.

Storage NoteStore 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 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 but is not advised in this condition due to increased pressure in the brain that occurs.
  • 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 Cortical Calcifying Angiomatosis In Celiac Disease and/or Gluten Sensitivity?

CASE REPORT SUMMARIES

“Cortical vascular abnormalities in the syndrome of celiac disease, epilepsy, bilateral occipital calcifications, and folate deficiency.” This case report describes pathological changes in the syndrome of celiac disease, folate deficiency, bilateral occipital calcifications, and intractable epilepsy. A child with this disorder had a field defect (visual) correlating with active lateralized epileptic discharges and asymmetrical lesions. A cortical vascular abnormality with patchy pial angiomatosis, fibrosed veins, and large jagged microcalcifications was found on resection of the right occipital lobe.2

“Meningo-cortical calcifying angiomatosis and celiac disease.” This case report describes a patient with ophthalmic migraine found to have bilateral (both sides) cerebellar and cerebral calcifications. She progressively developed severe intracranial hypertension, with swelling of the brain and downward transtentorial and tonsillar herniation. Resection of the right occipital pole demonstrated meningo-cortical calcifying angiomatosis. Within 2 months, brain swelling and papilledema disappeared. Subtle signs of malabsorption led to diagnosis of celiac disease.3

Sources:
  1. Wills AJ. The neurology and neuropathology of celiac disease. Neuropathology and Applied Neurobiology. 2000:26:493-496. []
  2. Bye AM, Andermann F, Robitaille Y, Oliver M, Bohane T, Andermann E. Cortical vascular abnormalities in the syndrome of celiac disease, epilepsy, bilateral occipital calcifications, and folate deficiency. Annals of Neurology. Sep 1993;34(3):399-403. [] []
  3. La Mantia L, Pollo B, Svoiardo M, et al. Meningo-cortical calcifying angiomatosis and celiac disease. Clinical Neurology and Neurosurgery. Sep 1998;100(3):209-15. [] []
  4. Bye AM, Andermann F, Robitaille Y, Oliver M, Bohane T, Andermann E. Cortical vascular abnormalities in the syndrome of celiac disease, epilepsy, bilateral occipital calcifications, and folate deficiency. Annals of Neurology. Sep 1993;34(3):399-403. []
  5. Wills AJ. The neurology and neuropathology of celiac disease. Neuropathology and Applied Neurobiology. 2000:26:493-496. []
  6. 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. []
  7. 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. [] [] [] [] []
  8. 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. []
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