Cerebral vasculitis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Cerebral vasculitis or central nervous system vasculitis (sometimes the word angiitis is used instead of "vasculitis") is vasculitis (inflammation of the blood vessel wall) involving the brain and occasionally the spinal cord. It affects all of the vessels: very small blood vessels (capillaries), medium-size blood vessels (arterioles and venules), or large blood vessels (arteries 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. Some of its signs and symptoms may resemble multiple sclerosis.[1] 10% have associated bleeding in the brain.[2]
Causes
"Primary" Central Nervous System (CNS) vasculitis is said to be present if there is no underlying cause. However, the immune system is known to play a part in the cause of the disease. This is because sometimes the immune system becomes over reactive and attacks the host's body. There are often antigens (medicines or illness that cause an allergic reaction) that are factors that help lead to this disease. Some of these possible antigens are infections, other forms of systemic vasculitis such as granulomatosis with polyangiitis or polyarteritis nodosa, connective tissue diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis, particular medications and drugs (amphetamine, cocaine and heroin), certain forms of cancer (particularly lymphomas, leukemia and lung cancer) and Behçet's disease. 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 and thrombotic thrombocytopenic purpura.[2]
Diagnosis
Cerebral angiography and magnetic resonance imaging, family medical history, symptoms, a complete physical examination, and ultimately biopsy of the brain, are often required for the diagnosis. Also, many lab tests must be done for the diagnosis: tests may reveal anemia (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 glucocorticoids. Then, if symptoms do not improve additional immunosuppression such as cyclophosphamide are added to decrease the immune system's attack on the body's own tissues.[2] 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.[3]
Specific Diseases
Giant cell arteritis (GCA)/ temporal arteritis
o 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
- In rare occasions stroke.
o Diagnostic criteria: Three of the five 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
o Treatment therapy
- High-dose corticosteroids
Takayasu’s arteritis
o 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.
o Diagnostic criteria: Three of the six 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.
o Treatment therapy
- 50% of patients respond to corticosteroid therapy alone in early phases
- Methotrexate or Azacytidine 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)
o 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
o Diagnostic Criteria: may be diagnosed with three of the ten
- More than 4 kg (8.8 lb) weight loss
- Lace-like purplish discoloration of the skin
- Testicular pain
- Pain in a muscle or group of muscles
- Damage to the peripheral nerves
- Elevation of blood pressure by more than 90 mmHg
- Creatinine levels in the urine greater than 1,5 mg/dl
- Hepatitis B or C virus antibodies
- An aneurysm or occlusion as shown in a pathologic arteriopraphy
- Histology findings typical of PAN
o Treatment therapy
- Prednisone and corticosteroid therapy
- In case of emergency, plasmapheresis may be tried
- In HV associated PAN a combo therapy is taken with virustatics such as lamivudine (Hepatitis B) or interferone-alpha and ribavirine (Hepatitis C)
Wegner’s granulomatosis (WG)
o Symptoms
- Men are affected twice as often as women
- Compression of structures surrounding the nose and paranasal sinuses
- Diabetes insipidus
- 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 seizures
o Diagnostic Criteria: two of the four
- Necrotizing ulcerating inflammation of nose, sinuses, mouth, or pharynx
- Irregular lung infiltrates
- Nephritis
- Granulomatous vascular and perivascular inflammation
o Treatment Therapy
- Pred and CYC
- AZA
- MMF
References
- ↑ Scolding NJ, Jayne DR, Zajicek JP, Meyer PA, Wraight EP, Lockwood CM (January 1997). "Cerebral vasculitis--recognition, diagnosis and management". QJM. 90 (1): 61–73. doi:10.1093/qjmed/90.1.61. PMID 9093590.
- ↑ 2.0 2.1 2.2 Rehman HU (November 2000). "Primary angiitis of the central nervous system" (PDF). J R Soc Med. 93 (11): 586–8. PMC 1298150. PMID 11198690.
- ↑ Scolding NJ, Wilson H, Hohlfeld R, Polman C, Leite I, Gilhus N (July 2002). "The recognition, diagnosis and management of cerebral vasculitis: a European survey". Eur. J. Neurol. 9 (4): 343–7. doi:10.1046/j.1468-1331.2002.00422.x. PMID 12099915.
4. Berlit, Peter. (January 2010). Us National Library of Medicine National Institute of Health. "Diagnosis and treatment of cerebral vasculitus." 29-42 doi: 10.1177/1756285609327123. 5. "Cerebral Vasculitis." Prime Health Channel. Deepamala Bhattacharya, 18 Dec. 2012. Web. 1 May 2015. <http://www.primehealthchannel.com/cerebral-vasculitis.html>.
6. "Central Nervous System Vasculitis." Cleveland Clinic. 6 Jan. 2012. Web. 1 May 2015. <http://my.clevelandclinic.org/health/diseases_conditions/hic_central_nervous_system_vasculitis>.
External links
- Central Nervous System Vasculitis at the Johns Hopkins Hospital vasculitis centre
- Primary angiitis of the central nervous system from the Cleveland Clinic