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What Is Cerebral Vasculitis?
Cerebral vasculitis, also called vasculitis of the central nervous system (CNS), is an autoimune attack against elastin fibers in the walls of arteries that bring blood to the head. Early recogniton may reduce poor outcomes.1
Cerebral vasculitis is characterized by inflammation of large, medium, or small blood vessels which is often segmental with scattered foci (sites) of intense inflammation, and results in necrosis (death) of affected tissues with scarring that occludes, or blocks, blood flow.
Q: What happens when an artery is occluded by scarring?
A: When an artery is occluded by scarring, blood cannot flow through it thus preventing the body tissues it supplies with oxygen and nutrition. Depending on vessels that are affected, blindness, TIA (transient ischemic attack) or stroke may result from blockage or rupture (hemorrhage).
Blood flow through arteries can be seen by angiography procedure. The diagnosis is made by biopsy. Additionally, contrast-enhanced MRI, proven to be sensitive to extradural arteritis, for the identification of intracranial vessel wall inflammation shows that wall thickening and intramural contrast uptake are frequent findings in patients with active cerebral vasculitis affecting large brain arteries.2
Vasculitis may develop with autoimmune diseases, such as celiac disease, lupus eythematosis and rheumatoid arthritis due to immune complexing, and possibly severe infection and drug sensitivity.
What Is Cerebral Vasculitis In Celiac Disease and/or Gluten Sensitivity?
- Relationship between cerebral vasculitis and celiac disease. Cerebral vasculitis is a severe associated immune disorder in celiac disease.
- Relationship between cerebral vasculitis and gluten. 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, Ventura and his associates found that the development of immune disorders in celiac disease was clearly related to the duration of exposure to gluten.3
What Are The Symptoms Of Cerebral Vasculitis?
Cerebral vasculitis is marked by these symptoms:
- Fever.
- Headache.
- Malaise.
- Scalp tenderness.
- Jaw tenderness and pain on opening and closing mouth is possible.
- Neurologic deficits depending in part on the size of the affected vessel(s) which may include seizure, TIA (transient ischemic attack) and/or stroke.
How Does Cerebral Vasculitis Develop In Celiac Disease and/or Gluten Sensitivity?
- Cerebral vasculitis results from an immune mediated process.
Does Cerebral Vasculitis Respond To Gluten-Free Diet?
Studies are inadequate to determine prognosis of celiac disease-related cerebral vasculitis on a gluten free diet.
A case report describes a patient diagnosed with celiac disease subsequent to diagnosis of cerebral vasculitis as having a clinical course marked by persistent visual after effects, but no new vascular event on gluten free diet.4
6 Steps To Improve Cerebral Vasculitis In Celiac Disease and/or Gluten Sensitivity:
- 1Remove the Trigger. Maintain a Strict, Nutritious Gluten Free Diet:
Treatment. This condition responds to the complete elimination of gluten, which is the required treatment that improves both cerebral vasculitis 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.5
- The intestinal lining may take up to a year to heal.
- 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).
- Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.6
- 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.6
- 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.6.
- 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.6
- 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.6
- Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.6
- Cocoa and Black Tea increase blood sugar.
- Rosemary. Increases blood sugar levels and should not be used by persons with insulin resistance or diabetes.
- 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.
- 3 Information Sheet You Can Take to Your Doctor or Other Health Professional:
- 4 Manage Your Medications Safely:
Certain medications may cause cerebral vasculitis which would aggravate gluten-related cerebral vasculitis. 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.
Phenylpropanolamine (PPA) causes cerebral vasculitis. It is the major ingredient in more than 70 over-the-counter preparations including diet pills, nasal decongestants, and the legal “look-alike” stimulants.7
- 5Nutritional Supplements To Help Correct Deficiencies:
The type and quantity of nutritional supplements that may be needed depend on which nutrients are deficient.
- Multivitamin/mineral combination that provides 100% 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.
- 6Manage Natural Remedies:
- 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.
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. However, because it increases blood sugar levels, it should not be used by persons with insulin resistance or diabete.
- 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.
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.
What Do Medical Research Studies Tell About Cerebral Vasculitis In Celiac Disease and/or Gluten Sensitivity?
RESEARCH STUDY SUMMARIES
Risk of stroke in 28,000 patients with celiac disease: a nationwide cohort study in Sweden.” This nationwide Swedish study collected biopsy data from all 28 pathology departments in Sweden and identified 28,676 individuals with celiac disease diagnosed between 1969 and 2007 (Marsh 3: villous atrophy). Results show that patients with celiac disease are at only a small increased risk of stroke, which persists only for a brief period after diagnosis.
The absolute risk of stroke in patients with celiac disease was 267 per 100,000 person-years. The highest risk estimates occurred in the first year, with virtually no increased risk after more than 5 years of follow-up after celiac disease diagnosis. The hazard ratio (HR) for stroke in childhood celiac disease was 1.10.8
“Celiac disease associated with recurrent stroke: a coincidence or cerebral vasculitis?” This case report describes a patient with celiac disease who developed vasculitis of the central nervous system. She presented with recurrent transient ischemic attacks with right hemihypoesthesia and right hemiparesia, severe pre-tibial edema, decreased muscle tonus of extremities and decreased deep tendon reflexes. While hospitalized she suffered a reversible stroke. CT scan revealed multiple lacunar infarcts predominantly in frontotemporal regions. MR imaging revealed cerebellar, corticosubcortical chronic atrophy, left frontoparietal corticosubcortical chronic atrophy, a variety of small edematous lesions in cortical areas on T2 weighted images. Digital subtraction angiography revealed indefinite segmental narrowing of the left middle cerebral artery. The histological examination of temporal superficial arteries revealed patchy inflammatory cell infiltrates of both lymphocytes and macrophages. Serum C3-C4 and albumin levels were decreased.9
“Celiac disease and ischemic stroke.” These two case reports describe an association of celiac disease with ischemic stroke. The first patient had experienced several transient ischemic strokes in the past 2 years and then had an acute ischemic stroke involving the territory of the right posterior cerebral artery. Investigations revealed celiac disease with no other recognizable etiology. The clinical course was marked by persistent visual after effects, but no new vascular event.
The second patient had been followed since 1998 for celiac disease confirmed by pathology and serology tests. She was on a gluten-free diet. The patient had an ischemic stroke involving the territory of the left middle cerebral artery. Apart from a positive serology for celiac disease and iron deficiency anemia, the etiological work-up for stroke was negative.
The mechanisms of vascular involvement in celiac disease are controversial. The most widely incriminated factor is autoimmune central nervous system vasculitis, in which tissue transglutaminase, the main auto-antigen contributing to maintaining the integrity of endothelium tissue, plays a major role. Other mechanisms are still debated, mainly vitamin deficiency.
Being a potentially treatable cause of ischemic stroke, celiac disease must be considered as a potential etiology of stroke of unknown cause, particularly in young patients, and even without gastrointestinal manifestations.10
- Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, Giannini C, Meschia JF, Huston J 3rd, Hunder GG. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. 2007 Nov;62(5):442-51. [↩]
- Küker W, Gaertner S, Nagele T, Dopfer C, Schoning M, Fiehler J, Rothwell PM, Herrlinger U. Vessel wall contrast enhancement: a diagnostic sign of cerebral vasculitis. Cerebrovasc Dis. 2008;26(1):23-9. doi: 10.1159/000135649. Epub 2008 May 30. [↩]
- 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, Available at: http://www.wjgnet.com/1007-9327/9/1377.asp.)
- El Moutawakil B Chourkani N, Sibai M, Moutaouakil F, Rafai M, Bourezgui M, Slassi I. Celiac disease and ischemic stroke. Rev Neurol (Paris). 2009 Nov;165(11):962-6. doi: 10.1016/j.neurol.2008.09.002. [↩]
- 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. [↩]
- 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. [↩] [↩] [↩] [↩] [↩] [↩]
- Glick R, Hoying J, Cerullo L, Perlman S. Phenylpropanolamine: an over-the-counter drug causing central nervous system vasculitis and intracerebral hemorrhage. Case report and review. Neurosurgery. 1987 Jun;20(6):969-74. [↩]
- Ludvigsson JF1, West J, Card T, Appelros P. Risk of stroke in 28,000 patients with celiac disease: a nationwide cohort study in Sweden. J Stroke Cerebrovasc Dis. 2012 Nov;21(8):860-7. doi: 10.1016/j.jstrokecerebrovasdis.2011.05.008. [↩]
- Ozge A, Karakelle and H Kaleagasi; Celiac disease associated with recurrent stroke: a coincidence or cerebral vasculitis? European Journal of Neurology. 2001;8:373-374. [↩]
- El Moutawakil B Chourkani N, Sibai M, Moutaouakil F, Rafai M, Bourezgui M, Slassi I. Celiac disease and ischemic stroke. Rev Neurol (Paris). 2009 Nov;165(11):962-6. doi: 10.1016/j.neurol.2008.09.002. [↩]
How Prevalent Is Cerebral Vasculitis In Celiac Disease and/or Gluten Sensitivity?
Cerebral vasculitis is shown by case report as an association with celiac disease. ((Ozge A, Karakelle and H Kaleagasi; Celiac disease associated with recurrent stroke: a coincidence or cerebral vasculitis? European Journal of Neurology. 2001;8:373-374. [↩]