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Uveitis Associated with Primary Angiitis of the Central Nervous System

Published online by Cambridge University Press:  02 December 2014

A.R. Woolfenden ,
N.K. Wade ,
P. Tang ,
A. Chalmers ,
G. Reid  and
P.A. Teal
A.R. Woolfenden
Affiliation:
Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
N.K. Wade
Affiliation:
Division of Ophthalmology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
P. Tang
Affiliation:
Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
A. Chalmers
Affiliation:
Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
G. Reid
Affiliation:
Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
P.A. Teal
Affiliation:
Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada
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Abstract

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Background:

In Primary Angiitis of the Central Nervous System (PACNS), disease is typically limited to the brain and spinal cord although other organs may be affected. Uveitis is occasionally seen in systemic vasculitides but is not a recognized manifestation of PACNS. We describe two patients who developed PACNS following the onset of uveitis.

Case Descriptions:

Case 1-A 47-year-old male suffered multiple TIAs and left pontine stroke shortly after two episodes of diffuse uveitis. A cerbral angiogram demonstrated multiple caliber changes within several intracranial vessels. Cyclophosphamide was added after his stroke occurred during pulse methylprednisolone therapy. Case 2- A 35-year-old male suffered a spinal cord TIA followed by hemispheric and brainstem infarctions two months after an episode of uveitis and Bell's palsy treated with oral prednisone. A cerebral angiogram demonstrated multiple caliber changes within several intracranial vessels. He was successfully treated with oral prednisone and cyclophosphamide.

Conclusions:

Uveitis should be considered a recognized feature of PACNS. Combination immunosuppressive therapy with prednisone and cyclophosphamide may be necessary for successful treatment.

Résumé:

RÉSUMÉ: Contexte:

Dans l'angéite primaire du système nerveux central (APSNC), il est typique d'observer une atteinte limitée au cerveau et à la moelle épinière, bien que d'autres organes puissent être touchés. On peut occasionnellement observer une uvéite dans les vasculites systémiques, mais l'uvéite n'est pas une manifestation reconnue de l'APSNC. Nous décrivons les cas de deux patients qui ont développé une APSNC suite à une uvéite.

Observations:

Observation 1 : Un homme de 47 ans a présenté de multiples épisodes d'accidents ischémiques transitoires (AIT) et un accident vasculaire cérébral peu après deux épisodes d'uvéite diffuse. Un angiogramme cérébral a mis en évidence de multiples changements de calibre au niveau de plusieurs vaisseaux intracrâniens. On a ajouté du cyclophosphamide à sa corticothérapie (méthylprednisolone) à doses en perfusions intraveineuses rapides. Cas 2 : Un homme de 35 ans a présenté une AIT à la moelle épinière suivie d'infarcissement hémisphérique et du tronc cérébral deux mois après un épisode d'uvéite et de paralysie de Bell traité par la prednisone par voie orale. Àl'angiogramme, on a observé de multiples changements de calibre de plusieurs vaisseaux intracrâniens. Il a été traité avec succès par la prednisone et la cyclophosphamide par voie orale.

Conclusions:

L'uvéite devrait être reconnue comme une manifestation possible de l'APSNC. Il peut être nécessaire d'avoir recours à un traitement immunosuppresseur combinant la prednisone et la cyclophosphamide pour assurer le succès du traitement.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 34 , Issue 1 , February 2007 , pp. 81 - 83
Copyright
Copyright © The Canadian Journal of Neurological 2007

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