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Primary Central Nervous System Vasculitis in Children

Purpose: To identify a child with primary angiitis of the central nervous system (cPACNS) who is likely to progress using the profile of Benseler et al.

Description

Benseler et al identified factors associated with disease progression in a child with primary angiitis of the central nervous system (childhood PACNS, or cPACNS). These can help distinguish this high risk group from those with nonprogressive disease. The authors are from the Hospital for Sick Children in Toronto and University Children's Hospital in Bonn.


 

The nonprogressive group has a monophasic vasculitis with vasospasm that is rapidly reversible.

 

Progression was defined on angiography performed > 3 months after the initial diagnosis.

 

Risk factors associated with disease progression:

(1) neurocognitive dysfunction (likelihood ratio 40)

(2) multifocal parenchymal lesions on MRI (odds ratio 3)

(3) distal stenoses on angiography (odds ratio 15)

 

The authors combined these risk factors into a profile. This profile is only partially described in the paper. Based on the available material, the following profile table is proposed.

Factors Present

Risk Score

Risk of Disease Progression

none

1

very low

multifocal lesions

2

low

distal stenoses

3

low to moderate

neurocognitive defects

4

moderate

multifocal lesions and distal stenoses

5

moderate

multifocal lesions and neurocognitive defects

6

moderate to high

distal stenoses and neurocognitive defect

7

moderate to high

all 3

8

high

 


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Specialty

Objective

ICD-10