Related Experiment Video

Updated: Aug 7, 2026

Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
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

Primary Angiitis of the Central Nervous System: A Medical Mystery

Seby Robert1, Reddy Deepica2, Ketty Arpitha3

  • 1Department of Internal Medicine, Mayo Clinic Florida, Jacksonville, Florida, United States of America.

Romanian Journal of Internal Medicine = Revue Roumaine De Medecine Interne
|August 6, 2026
PubMed

Insights

Primary angiitis of the central nervous system (PACNS) is rare and difficult to diagnose. Early awareness and treatment with steroids and cyclophosphamide can lead to positive outcomes in patients with this neurological condition.

Area of Science:

  • Neurology
  • Immunology
  • Vascular Medicine

Background:

  • Primary angiitis of the central nervous system (PACNS) is an exceptionally rare condition.
  • The etiology of PACNS is unknown, often presenting with a prolonged prodromal phase.
  • PACNS is a diagnosis of exclusion, requiring the elimination of infectious, autoimmune, and malignant causes.

Purpose of the Study:

  • To highlight the diagnostic challenges and rarity of PACNS.
  • To emphasize the importance of clinical awareness for timely diagnosis.
  • To present a case demonstrating successful treatment of PACNS.

Main Methods:

  • Literature review on PACNS incidence and diagnostic criteria.
  • Case report detailing clinical presentation and diagnostic workup.
  • Analysis of treatment response in a patient with PACNS.

Main Results:

  • PACNS has a low incidence (2.4 per 1,000,000 person-years).
  • Diagnostic delays for PACNS can range from 6 to 23 months.
  • The presented case showed significant improvement with high-dose steroids and cyclophosphamide.

Conclusions:

  • Increased clinical suspicion is crucial for early PACNS diagnosis.
  • Prompt treatment with immunosuppressants like steroids and cyclophosphamide is effective.
  • Awareness of PACNS is vital for managing non-specific neurological symptoms.
Keywords:
Primary angiitiscentral nervous systemcyclophosphamideprodromesteroids

Related Concept Videos

Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
Bacterial Meningitis II: Pathophysiology01:26

Bacterial Meningitis II: Pathophysiology

Bacterial meningitis typically begins when pathogens such as Neisseria meningitidis and Streptococcus pneumoniae colonize the nasopharynx and invade the bloodstream. This process is facilitated by bacterial virulence factors, such as polysaccharide capsules, which resist phagocytosis and complement-mediated killing. Less commonly, bacteria reach the central nervous system via contiguous spread from infections like otitis media or sinusitis, through congenital or acquired dural defects, or...
Cerebral Edema ll: Pathophysiology01:22

Cerebral 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...
Arteries of the Head and Neck01:26

Arteries of the Head and Neck

The human body's intricate network of arteries ensures that every organ system receives the necessary oxygen and nutrients for optimal function. The arterial network in the head and neck region is particularly complex, providing vital blood flow to the brain, eyes, and other critical structures. Prominent arteries in this region include the internal carotid arteries and the vertebral arteries.
The internal carotid arteries supply blood to the anterior portion of the cerebrum. They enter the...
Mechanism of Angiogenesis01:10

Mechanism of Angiogenesis

Blood vessel formation starts early during embryonic development, around day 7. In the extraembryonic yolk sac, mesodermal precursor cells called hemangioblast proliferate and differentiate into angioblast. Angioblasts express vascular endothelial growth factor receptor 2 or VEGFR2, which binds VEGF-A, a proangiogenic factor, guiding blood vessel formation. VEGF signaling promotes angioblasts to form a blood island in the developing embryo. Angioblasts further differentiate, giving rise to...
Transient Ischemic Attack l: Introduction01:26

Transient Ischemic Attack l: Introduction

A transient ischemic attack (TIA) is a brief episode of neurological dysfunction caused by a temporary, focal reduction in cerebral blood flow. Although symptoms resemble those of an ischemic stroke, the interruption in perfusion is short-lived and does not cause permanent infarction. TIAs are clinically important because they often serve as early warning events for future stroke.Mechanisms of Transient Cerebral IschemiaTransient cerebral ischemia may arise through several mechanisms. One...