Cerebral vasculitis

Cerebral vasculitis
Synonyms	Central nervous system vasculitis, CNS vasculitis
Pronounce	N/A
Specialty	N/A
Symptoms	Headache, confusion, seizures, stroke, focal neurological deficits
Complications	Stroke, intracerebral hemorrhage, cognitive impairment
Onset	Variable
Duration	Variable
Types	N/A
Causes	Autoimmune disease, infection, drug-induced
Risks	Autoimmune disorders, infections, drug use
Diagnosis	MRI, cerebral angiography, lumbar puncture, biopsy
Differential diagnosis	Multiple sclerosis, migraine, infectious meningitis, primary angiitis of the central nervous system
Prevention	N/A
Treatment	Corticosteroids, immunosuppressive drugs, plasmapheresis
Medication	N/A
Prognosis	Variable, depends on cause and treatment
Frequency	Rare
Deaths	N/A

Cerebral vasculitis
Synonyms	Central nervous system vasculitis, Primary angiitis of the central nervous system (PACNS)
Pronounce
Specialty	N/A
Symptoms	Headache, Seizures, Cognitive dysfunction, Stroke-like symptoms, Neurological deficit
Complications	Stroke, Intracerebral hemorrhage, Cognitive decline, Seizure disorder, Disability
Onset	Typically adult, but can occur at any age
Duration	Chronic or relapsing-remitting
Types	Primary angiitis of the central nervous system (PACNS), Secondary cerebral vasculitis
Causes	Autoimmune disorders, Infections (e.g. HIV, hepatitis), Medications, Systemic vasculitis
Risks	Systemic lupus erythematosus, Rheumatoid arthritis, Behçet's disease, Polyarteritis nodosa
Diagnosis	MRI, Cerebral angiography, Brain biopsy
Differential diagnosis	Multiple sclerosis, Ischemic stroke, CNS lymphoma, Neurosarcoidosis
Prevention	Managing autoimmune disease, treating infections early
Treatment	Immunosuppressive therapy, Corticosteroids, Cyclophosphamide
Medication	Prednisone, Azathioprine, Methotrexate, Mycophenolate mofetil
Prognosis	Variable; better with early treatment, poorer with delayed diagnosis
Frequency	Rare; <3 cases per million annually
Deaths	Mortality varies; ~10–30% without treatment

Cerebral vasculitis is a rare condition characterized by inflammation of the blood vessels in the brain. This inflammation can lead to a variety of neurological symptoms due to the disruption of normal blood flow and potential damage to brain tissue.

Pathophysiology

Cerebral vasculitis involves the inflammation of the blood vessels within the central nervous system. This inflammation can cause the vessel walls to thicken, leading to a reduction in the diameter of the vessels and potentially resulting in ischemia or hemorrhage. The exact mechanism of inflammation can vary depending on the underlying cause, which may include autoimmune processes, infections, or other systemic diseases.

Causes

Cerebral vasculitis can be primary or secondary.

Primary Cerebral Vasculitis

Primary cerebral vasculitis, also known as primary angiitis of the central nervous system (PACNS), is a rare form of vasculitis that affects only the brain and spinal cord. The cause of PACNS is not well understood, but it is thought to involve an autoimmune response.

Secondary Cerebral Vasculitis

Secondary cerebral vasculitis occurs as a result of another condition. Possible causes include:

• Systemic lupus erythematosus
• Rheumatoid arthritis
• Infections such as HIV or hepatitis
• Certain medications and drugs

Symptoms

The symptoms of cerebral vasculitis can vary widely depending on the areas of the brain affected. Common symptoms include:

• Headache
• Seizures
• Stroke-like symptoms
• Cognitive dysfunction
• Focal neurological deficits

Diagnosis

Diagnosing cerebral vasculitis can be challenging due to its rarity and the nonspecific nature of its symptoms. A combination of clinical evaluation, imaging studies, and sometimes a biopsy of brain tissue is used to confirm the diagnosis.

Imaging

Magnetic resonance imaging (MRI) and angiography are commonly used to visualize the blood vessels and detect any abnormalities suggestive of vasculitis.

Biopsy

In some cases, a brain biopsy may be necessary to obtain a definitive diagnosis. This involves taking a small sample of brain tissue to be examined under a microscope for signs of inflammation.

Treatment

The treatment of cerebral vasculitis typically involves the use of immunosuppressive drugs to reduce inflammation. Commonly used medications include:

• Corticosteroids such as prednisone
• Cyclophosphamide
• Azathioprine

The choice of treatment depends on the severity of the condition and the underlying cause.

Prognosis

The prognosis for cerebral vasculitis varies depending on the cause and the promptness of treatment. Early diagnosis and treatment are crucial for improving outcomes and preventing permanent neurological damage.

Related pages

• Vasculitis
• Autoimmune disease
• Central nervous system