Related Experiment Video
Updated: May 22, 2026
10:50
Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
Published on: March 26, 2019
Adult primary central nervous system vasculitis
Carlo Salvarani1, Robert D Brown, Gene G Hunder
1Unit of Rheumatology, Department of Internal Medicine, Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy. salvarani.carlo@asmn.re.it
Lancet (London, England)
|May 12, 2012
Summary
Primary central nervous system (CNS) vasculitis affects the brain and spinal cord, presenting with diverse neurological symptoms. Early diagnosis and treatment are crucial for preventing severe outcomes.
Area of Science:
- Neurology
- Immunology
Background:
- Primary central nervous system (CNS) vasculitis is a rare, idiopathic inflammatory condition affecting the brain and spinal cord.
- It typically affects individuals around 50 years old, presenting with varied neurological symptoms like headache, cognitive changes, focal weakness, or stroke.
Purpose of the Study:
- To summarize the key aspects of primary CNS vasculitis, including its presentation, diagnostic challenges, and management.
- To highlight the importance of early recognition for effective treatment and improved patient outcomes.
Main Methods:
- Review of existing literature on primary CNS vasculitis.
- Analysis of diagnostic criteria, including neuroimaging (MRI), cerebrospinal fluid analysis, and biopsy.
- Discussion of treatment strategies and differential diagnoses.
Main Results:
- Serological inflammatory markers are typically normal, while cerebrospinal fluid abnormalities are common (80-90%).
- A normal brain MRI makes diagnosis unlikely; CNS tissue biopsy is the definitive diagnostic test.
- Angiography is frequently used but has moderate sensitivity and specificity.
Conclusions:
- Early recognition of primary CNS vasculitis is vital for timely intervention.
- Treatment with corticosteroids and potentially cytotoxic drugs can prevent serious neurological sequelae.
- Distinguishing primary CNS vasculitis from conditions like reversible cerebral vasoconstriction syndromes and secondary vasculitis is critical.
Related Concept Videos
01:26Encephalitis ll: Pathophysiology
Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
01:22Cerebral Edema ll: Pathophysiology
Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
01:19Multiple Sclerosis l: Introduction
Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
01:19Encephalitis l: Introduction
Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
01:18Disorders of the Autonomic Nervous System
The autonomic nervous system (ANS) is an intricate network of nerves that controls functions such as the regulation of heart rate, digestion, and blood pressure regulation. When this system malfunctions, it can lead to various disorders that affect multiple bodily functions. One common feature of many autonomic disorders is the involvement of smooth blood vessels, which play a crucial role in regulating blood flow throughout the body.
Raynaud's disease, also known as Raynaud's phenomenon, is a...
Raynaud's disease, also known as Raynaud's phenomenon, is a...
01:16Vascular Spasm
The vascular phase, also known as vasospasm, is the initial stage of hemostasis, crucial for preventing excessive bleeding when a blood vessel is injured. After a vessel is cut, nerves in the damaged area trigger pain and other sensory impulses. Simultaneously, the smooth muscles in the vessel wall contract, resulting in a vascular spasm. This contraction reduces the vessel's diameter at the injury site, slowing or stopping blood loss through the vessel wall. Vascular spasms typically last for...