Volume 9 Supplement 1

Proceedings of 18th Pediatric Rheumatology European Society (PReS) Congress

Open Access

Primary angiitis of the central nervous system in children - case series

  • V Krakovská1Email author,
  • R Stibitzová2,
  • T Pískovský3,
  • Z Vrábelová4,
  • M Macků5,
  • D Němcová1 and
  • P Doležalová1
Pediatric Rheumatology20119(Suppl 1):P94

DOI: 10.1186/1546-0096-9-S1-P94

Published: 14 September 2011

Background

Primary angiitis of the central nervous system (PACNS) is a rare inflammatory brain disease with variable clinical manifestations. Only limited data exist with regard to efficient diagnostic and therapeutic algorithms and disease outcomes in children.

Aim

To characterize clinical features, neuroimaging findings, treatment and disease outcome in a small cohort of Czech children with PACNS.

Methods

A retrospective chart review of patients referred for suspected PACNS was performed. MRI lesions detected at disease onset were classified as unilateral (UL), bilateral (BL), unifocal (F), multifocal (MF), angiography (MRA and/or conventional) positive (medium/large vessel disease) or negative (small vessel disease). According to the evolution of clinical manifestations and neuroimaging PACNS was classified as progressive or non-progressive. Data on treatment and follow-up were collected.

Results

Seven female patients (all Caucasian, mean age 14,8 years, median F/U 2 years) were identified (Table). Three had favourable outcome. Four (1,2,5,7) had progressive disease resulting in significant impairment. Their mean interval from onset to diagnosis was 7 months. All 4 had multifocal lesions, 2 had negative angiography, 3 did not receive any anti-inflammatory therapy early in the disease course.

Table

Patient

1

2

3

4

5

6

7

Age at onset (years)

13

1,5

12

16

5,5

6

15

Time to diagnosis (months)

30

5

1

1,5

12

1

1,5

F/U (years)

4

13

5

0,5

2

1,5

1

Manifestation at onset

generalised seizures

focal seizure, stroke

seizure sensory deficit

sensory deficit, stroke

stroke

stroke with ataxia

seizures stroke

MRI at onset

UL, MF

UL, MF

BL, MF

BL,MF

UL,MF

UL, F

UL,MF

Angiography at onset

N/D

posit.

posit.

posit.

posit.

posit.

neg.

Initial therapy

AED

AED

ACA

ACA, CS

0

ACA

IVMP, AED

Manifestation at F/U

seizures cognitive dysfunction

hemiparesis developmental delay

normal

normal

cognitive dysfunction, hemiparesis

normal

organic psychosis

MRI at F/U

BL, MF

N/D

N/D

N/D

BL, MF

regression

BL, MF

Angiography F/U

neg.

N/D

N/D

N/D

regression

regression

neg.

Therapy at F/U

AED, CS

AED, ACA

ACA

CS

ACA

ACA

CYC, CS

ACA - acetylsalicylic acid, AED - anti-epileptic drugs, CS - oral corticosteroid (prednisone or methylprednisolone), CYC - cyclophosphamide, IVMP- intravenous methylprednisolone, N/D- not done

Conclusion

Unfavourable outcome of this patient cohort reflects potential severity as well as low physicians awareness of PACNS resulting in diagnostic delay and insufficient therapy. A working group of paediatric specialists is being formed in the Czech Republic in order to establish diagnostic and therapeutic algorithms in close interdisciplinary and multicenter collaboration.

Authors’ Affiliations

(1)
Paediatric Rheumatology Unit, General University Hospital in Prague
(2)
Department of Paediatric Neurology, Thomayer University Hospital in Prague
(3)
Department of Paediatrics, Teaching Hospital in Ostrava
(4)
Department of Paediatric Neurology, University Hospital Motol
(5)
Department of Paediatrics, University Hospital in Brno

Copyright

© Krakovská et al; licensee BioMed Central Ltd. 2011

This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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