Related Experiment Video

Updated: Aug 9, 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 central nervous system vasculitis in children

Susanne M Benseler1, Earl Silverman, Richard I Aviv

  • 1Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada, and University Children's Hospital, Bonn, Germany.

Arthritis and Rheumatism
|April 1, 2006
PubMed

Insights

Primary angiitis of the central nervous system (PACNS) in children presents in progressive and nonprogressive forms. Neurocognitive dysfunction, multifocal lesions, and distal stenoses predict progressive disease in pediatric PACNS patients.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Vascular Neurology

Background:

  • Primary angiitis of the central nervous system (PACNS) is a rare and poorly understood neurological condition.
  • Understanding its presentation and progression in children is crucial for effective management.

Purpose of the Study:

  • To characterize the clinical features, treatment, and outcomes of pediatric PACNS (cPACNS).
  • To identify predictors of disease progression in cPACNS to define high-risk patient groups.

Main Methods:

  • Retrospective analysis of 62 consecutive pediatric patients diagnosed with cPACNS.
  • Evaluation of clinical presentation, neuroimaging (conventional angiography, MR angiography), and cerebrospinal fluid findings.
  • Multivariate regression models used to identify predictors of angiographic disease progression at >3 months.

Main Results:

  • Two distinct subgroups emerged: progressive and nonprogressive cPACNS.
  • Progressive disease occurred in 20 of 62 children.
  • Predictors of progression included neurocognitive dysfunction, multifocal MR imaging lesions, and distal stenoses on angiography.

Conclusions:

  • Pediatric PACNS encompasses both progressive and nonprogressive disease forms.
  • Specific diagnostic features can predict disease progression in children.
  • Identification of high-risk cPACNS patients can guide immunosuppressive therapy selection.
Abstract

More Related Videos

Reliable Isolation of Central Nervous System Microvessels Across Five Vertebrate Groups10:35

Reliable Isolation of Central Nervous System Microvessels Across Five Vertebrate Groups

Published on: January 12, 2020

Related Concept Videos

Encephalitis ll: Pathophysiology01:26

Encephalitis 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...
Viral Meningitis01:18

Viral Meningitis

Viral meningitis is the most common form of meningitis and is often referred to as aseptic meningitis to indicate the absence of bacterial involvement. It is generally milder than bacterial meningitis, with symptoms including fever, headache, stiff neck, drowsiness, nausea, photophobia, and vomiting. Rarely, more severe manifestations or death may occur. Common causative agents include enteroviruses, particularly coxsackie A and B viruses and echoviruses, all members of the Enterovirus genus...
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...
Encephalitis l: Introduction01:19

Encephalitis 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...
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...
Vascular Spasm01:16

Vascular 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...