Cerebral Angiitis

Definition

Cerebral angiitis is an inflammation of the small arteries in the brain.

Description

Cerebral angiitis is a type of vasculitis in which an aberrant immune response results in inflammation and destruction of the small arteries that feed brain tissue. As a result of the inflammation, blood clots form within the arteries, compromising blood flow and resulting in decreased oxygen delivery to vulnerable brain tissue. Two types of cerebral angiitis have been recognized. The first type is considered to be an encephalopathic type, which results in wide-spread, slowly progressive damage to the brain. The second type causes abrupt, acute damage to a focal area of the brain, similar to a stroke.

Demographics

While cerebral angiitis can affect people of all ages, it is most common in the middle aged. Cerebral angiitis affects slightly more males than females. It may also be responsible for the unusual presentation of vasculitis in children, often following a simple chicken pox infection. Cerebral angiitis can also occur as a rare complication of allogeneic bone marrow transplant (bone marrow transplant received from a donor).

Causes and symptoms

Cerebral angiitis may occur spontaneously, with no known cause, or in conjunction with, or as a sequela to (an aftereffect of) a variety of viral infections, including herpes zoster (shingle), varicella zoster (chicken pox), and HIV/AIDS.

Symptoms can include slowly progressive headache, nausea, vomiting, stiff neck, confusion, irritability, loss of memory, seizures, and dementia. Cerebral angiitis may also cause the sudden onset of more acute and focal loss of function, such as sudden loss of the use of one side of the body or the inability to speak.

Diagnosis

Cerebral angiitis may be diagnosed by examining a sample of cerebrospinal fluid, which will likely reveal increased levels of protein and abnormal white cell activity. MRI scanning of the brain will usually show a diffuse pattern of lesions throughout the white matter of the brain, although the stroke-like type of cerebral angiitis may reveal a more focal area of damage. Biopsy of a sample of brain tissue is the most definitive diagnostic test; it will reveal inflammation and immune system activity affecting the damaged small arteries of the brain.

Treatment team

Individuals with cerebral angiitis may be treated by a neurologist or a rheumatologist.

Treatment

Treatment for cerebral angiitis addresses the inflammation and the immune response, both of which are responsible for the complications of the condition. Corticosteroids (to quell inflammation) and cyclophosphamide (to dampen the immune system) may be given in tandem, often at high doses for about six weeks, and then at lower doses for up to a year. Occasionally, symptoms rebound after the dose is dropped, requiring that the higher dose be reutilized; even after supposed cure, relapse may supervene, necessitating another course of corticosteroids and cyclophosphamide.

Some patients with cerebral angiitis will also benefit from the administration of anticoagulant agents to thin the blood and prevent arterial obstruction by blood clots.

Recovery and rehabilitation

The type of rehabilitation program required will depend on the types of deficits caused by cerebral angiitis, but may include physical therapy, occupational therapy, and speech and language therapy.

Prognosis

Untreated cerebral angiitis will inevitably progress to death, often within a year of the onset of the disease. More research is needed to define the prognosis of treated cerebral angiitis; current research suggests that slightly more than half of all treated patients have a good outcome.

BOOKS

Sergent, John S. "Polyarteritis and related disorders." In Kelley's Textbook of Rheumatology, 6th edition, edited by Shaun Ruddy, et al. St. Louis: W. B. Saunders Company, 2001.

PERIODICALS

Rollnik, J. D., A. Brandis, K. Dehghani, J. Bufler, M. Lorenz, F. Heidenreich, and F. Donnerstag. "Primary angiitis of CNS (PACNS)." Nervenarzt 72, no. 10 (October 2001): 798–801.

Singh, S., S. John, T. P. Joseph, and T. Soloman. "Primary angiitis of the central nervous system: MRI features and clinical presentation." Australasian Radiology 47, no. 2 (June 2003): 127–134.

Singh, S., S. John, T. P. Joseph, and T. Soloman. "Prognosis of patients with suspected primary CNS angiitis and negative brain biopsy." Neurology 61, no. 6 (September 2003) 831–833.

Rosalyn Carson-DeWitt, MD