Cerebral vasculitis (sometimes the word angiitis is used instead of "vasculitis") is

vasculitis Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. Vasculitis is primarily caused ...

(inflammation of the

blood vessel The blood vessels are the components of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away ...

wall) involving the

brain A brain is an organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It is located in the head, usually close to the sensory organs for senses such as vision. It is the most complex organ in a ve ...

and occasionally the

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the sp ...

. It affects all of the vessels: very small blood vessels (

capillaries A capillary is a small blood vessel from 5 to 10 micrometres (μm) in diameter. Capillaries are composed of only the tunica intima, consisting of a thin wall of simple squamous endothelial cells. They are the smallest blood vessels in the body: ...

), medium-size blood vessels (

arteriole An arteriole is a small-diameter blood vessel in the microcirculation that extends and branches out from an artery and leads to capillaries. Arterioles have muscular walls (usually only one to two layers of smooth muscle cells) and are the primar ...

s and

venule A venule is a very small blood vessel in the microcirculation that allows blood to return from the capillary beds to drain into the larger blood vessels, the veins. Venules range from 7μm to 1mm in diameter. Veins contain approximately 70% of t ...

s), or large blood vessels (

arteries An artery (plural arteries) () is a blood vessel in humans and most animals that takes blood away from the heart to one or more parts of the body (tissues, lungs, brain etc.). Most arteries carry oxygenated blood; the two exceptions are the pu ...

and veins). If blood flow in a vessel with vasculitis is reduced or stopped, the parts of the body that receive blood from that vessel begins to die. It may produce a wide range of neurological symptoms, such as headache, skin rashes, feeling very tired, joint pains, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought or behavior, as well as the phenomena of a mass lesion in the brain leading to coma and

herniation A hernia is the abnormal exit of tissue or an organ, such as the bowel, through the wall of the cavity in which it normally resides. Various types of hernias can occur, most commonly involving the abdomen, and specifically the groin. Groin hernia ...

. Some of its signs and symptoms may resemble multiple sclerosis. 10% have associated bleeding in the brain.

Causes

"Primary" angiitis/vasculitis of the central nervous system (PACNS) is said to be present if there is no underlying cause. The exact mechanism of the primary disease is unknown, but the fundamental mechanism of all vasculitides is

autoimmunity In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". ...

. Other possible secondary causes of cerebral vasculitis are

infection An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dis ...

s, systemic auto-immune diseases such as systemic lupus erythematosus (SLE) and

rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are invol ...

medication A medication (also called medicament, medicine, pharmaceutical drug, medicinal drug or simply drug) is a drug used to diagnose, cure, treat, or prevent disease. Drug therapy ( pharmacotherapy) is an important part of the medical field and ...

s and drugs ( amphetamine,

cocaine Cocaine (from , from , ultimately from Quechua: ''kúka'') is a central nervous system (CNS) stimulant mainly used recreationally for its euphoric effects. It is primarily obtained from the leaves of two Coca species native to South Ameri ...

and heroin), some forms of

cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...

(

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enla ...

leukemia Leukemia ( also spelled leukaemia and pronounced ) is a group of blood cancers that usually begin in the bone marrow and result in high numbers of abnormal blood cells. These blood cells are not fully developed and are called ''blasts'' or ...

and

lung cancer Lung cancer, also known as lung carcinoma (since about 98–99% of all lung cancers are carcinomas), is a malignant lung tumor characterized by uncontrolled cell growth in tissues of the lung. Lung carcinomas derive from transformed, malign ...

) and other forms of systemic vasculitis such as

granulomatosis with polyangiitis Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis ...

, polyarteritis nodosa or

Behçet's disease Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the ey ...

. It may imitate, and is in turn imitated by, a number of other diseases that affect the blood vessels of the brain diffusely such as

fibromuscular dysplasia Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory disease of the blood vessels that causes abnormal growth within the wall of an artery. FMD has been found in nearly every arterial bed in the body although the most common ...

and

thrombotic thrombocytopenic purpura Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body. This results in a low platelet count, low red blood cells due to their breakdown, and often kidney, h ...

Diagnosis

Cerebral angiography Cerebral angiography is a form of angiography which provides images of blood vessels in and around the brain, thereby allowing detection of abnormalities such as arteriovenous malformations and aneurysms. It was pioneered in 1927 by the Portugue ...

and magnetic resonance imaging, family medical history, symptoms, a complete physical examination, and ultimately

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a dise ...

of the brain, are often required for the diagnosis. Also, many lab tests must be done for the diagnosis; tests may reveal

anemia Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, t ...

(a shortage of red blood cells), a high white blood cell count, a high platelet count, allergic reactions, immune complexes, antibodies (tools the body uses to fight off threats) and elevation of inflammatory markers. Another crucial part in the diagnosis of cerebral vasculitis is the use of imaging techniques. Techniques such as conventional digital subtraction angiography (DSA) and magnetic resonance imaging (MRI) are used to find and monitor cerebral involvement.

Treatment

Treatment is first with many different high-dose steroids, namely

glucocorticoid Glucocorticoids (or, less commonly, glucocorticosteroids) are a class of corticosteroids, which are a class of steroid hormones. Glucocorticoids are corticosteroids that bind to the glucocorticoid receptor that is present in almost every verteb ...

s. Then, if symptoms do not improve additional

immunosuppression Immunosuppression is a reduction of the activation or efficacy of the immune system. Some portions of the immune system itself have immunosuppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse reacti ...

such as cyclophosphamide are added to decrease the immune system's attack on the body's own tissues. Cerebral vasculitis is a very rare condition that is difficult to diagnose, and as a result there are significant variations in the way it is diagnosed and treated.

Specific Diseases

Giant cell arteritis Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. ...
(GCA)

(Also known as temporal arteritis)

Symptoms

* Fever * general uneasiness * weight loss * inflammation of the muscles causing stiffness in the shoulders; neck; and/or upper arms * persisting headache * pain in the jaw or ear while eating * double vision * partial loss of vision or blind spots * (on rare occasions) stroke.

Diagnostic criteria

Three or more of the following five criteria must be met: * Age 50 years or more * New developed headache * Tenderness of the superficial temporal artery * Elevated sedimentation rate, at least 50 mm/hour (blood test that reveals inflammatory activity) * Giant cell arteritis in a biopsy specimen from the temporal artery

Takayasu's arteritis Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of large vessel granulomatous vasculitisAmerican College of Physicians (ACP). Medical Knowledge Self-Assessment Program (MK ...

Symptoms

Starts with nonspecific symptoms such as: * Localized joint pain * Fever * Fatigue * Headaches * Rashes * Weight loss * Diagnosis usually does not happen until the blockage causes deficient blood flow to the extremities or to a stroke.

Classification criteria

Three or more of the following six criteria must be met: * Age when disease starts is under 50 * Decreased brachial artery pulse * Systolic blood pressure differs by more than 10mmHg between arms * Cramping caused by exercise in the extremities * Abnormal sounds (through stethoscope) over subclavian arteries or abdominal aorta * A narrowing or blockage in the aorta, its primary branches, or large arteries as seen through a radiograph of the arteries.

Treatment therapy

* 50% of patients respond to corticosteroid therapy alone in early phases * Methotrexate or Azathioprine are an alternative to corticosteroid immunosuppressants * There have been studies on Mycophenolate mofetil and anti-TNF therapies * In Takayasu’s arteritis it is vital to combine drug treatments often with low-dose aspirin or statin

Polyarteritis nodosa (PAN)

Symptoms

* Systemic illness with fever * General feeling of discomfort or uneasiness with cause difficult to identify * Weight loss * Arthritis * Black discoloration of skin primarily on the extremities * Severe inadequate blood supply to the extremities * Ischemic stroke, hemorrhages and a progressive encephalopathy with or without seizures may occur

Diagnostic Criteria

Three or more of the following ten criteria are required: * More than 4 kg (8.8 lb) weight loss * Lace-like purplish discoloration of the skin (livedo reticularis) * Testicular pain * Pain in a muscle or group of muscles ( myalgias) * Damage to peripheral nerves * Elevation of blood pressure by more than 90 mmHg *

Creatinine Creatinine (; ) is a breakdown product of creatine phosphate from muscle and protein metabolism. It is released at a constant rate by the body (depending on muscle mass). Biological relevance Serum creatinine (a blood measurement) is an importa ...

serum levels greater than 1,5 mg/dl * Hepatitis B or C virus antibodies * An

aneurysm An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall. Aneurysms may be a result of a hereditary condition or an acquired disease. Aneurysms can also be a nidus ( ...

or occlusion as shown in a pathologic

arteriography Angiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins, and the heart chambers. Modern angiography is perform ...

* Histology findings typical of PAN

Treatment therapy

* In PAN ''not'' associated with a hepatitis virus:

prednisone Prednisone is a glucocorticoid medication mostly used to suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium due to cancer and ad ...

and cyclophosphamide therapy. In case of emergency, plasmapheresis may be tried * In PAN associated with a hepatitis virus: combination therapy of

along with a virustatic, such as

lamivudine Lamivudine, commonly called 3TC, is an antiretroviral medication used to prevent and treat HIV/AIDS. It is also used to treat chronic hepatitis B when other options are not possible. It is effective against both HIV-1 and HIV-2. It is typicall ...

(

Hepatitis B Hepatitis B is an infectious disease caused by the '' Hepatitis B virus'' (HBV) that affects the liver; it is a type of viral hepatitis. It can cause both acute and chronic infection. Many people have no symptoms during an initial infection. ...

) or

interferon-alpha The type-I interferons (IFN) are cytokines which play essential roles in inflammation, immunoregulation, tumor cells recognition, and T-cell responses. In the human genome, a cluster of thirteen functional IFN genes is located at the 9p21.3 cyt ...

and

ribavirin Ribavirin, also known as tribavirin, is an antiviral medication used to treat RSV infection, hepatitis C and some viral hemorrhagic fevers. For hepatitis C, it is used in combination with other medications such as simeprevir, sofosbuvir, pe ...

( Hepatitis C)

Granulomatosis with polyangiitis Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis ...
(GPA)

Symptoms

* Men are affected twice as often as women * Compression of structures surrounding the nose and paranasal sinuses *

Diabetes insipidus Diabetes insipidus (DI), recently renamed to Arginine Vasopressin Deficiency (AVP-D) and Arginine Vasopressin Resistance (AVP-R), is a condition characterized by large amounts of dilute urine and increased thirst. The amount of urine produced ...

* Abnormal protrusion of the eyeball(s) * Nonseptic meningitis * Affection of the lung and kidney due to destruction of the arteries and veins * Ischemic stroke, hemorrhages, or encephalopathy with possible

seizure An epileptic seizure, informally known as a seizure, is a period of symptoms due to abnormally excessive or synchronous neuronal activity in the brain. Outward effects vary from uncontrolled shaking movements involving much of the body with l ...

Diagnostic Criteria

Two or more of the following four criteria are required: * Necrotizing ulcerating inflammation of nose, sinuses, mouth or pharynx * Irregular lung infiltrates * Nephritis * Granulomatous vascular and perivascular inflammation

Treatment Therapy

* Corticosteroids (e.g., Prednisolone) * Cyclophosphamide *

Azathioprine Azathioprine (AZA), sold under the brand name Imuran, among others, is an immunosuppressive medication. It is used in rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, and systemic lupus erythematosus, ...

Mycophenolate mofetil Mycophenolic acid (MPA) is an immunosuppressant medication used to prevent rejection following organ transplantation and to treat autoimmune conditions such as Crohn's disease and lupus. Specifically it is used following kidney, heart, and liv ...

References

External links

{{Cerebrovascular diseases Cerebrovascular diseases Inflammations Rheumatology Steroid-responsive inflammatory conditions

Causes

Diagnosis

Treatment

Specific Diseases

Giant cell arteritis Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. ... (GCA)

Symptoms

Diagnostic criteria

Takayasu's arteritis Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of large vessel granulomatous vasculitisAmerican College of Physicians (ACP). Medical Knowledge Self-Assessment Program (MK ...

Symptoms

Classification criteria

Treatment therapy

Polyarteritis nodosa (PAN)

Symptoms

Diagnostic Criteria

Treatment therapy

Granulomatosis with polyangiitis Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis ... (GPA)

Symptoms

Diagnostic Criteria

Treatment Therapy

References

External links

Giant cell arteritis Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. ...
(GCA)

Granulomatosis with polyangiitis Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis ...
(GPA)