Recurrent arterial ischemic stroke with good response to mycophenolate mofetil.


Journal

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
ISSN: 1532-2130
Titre abrégé: Eur J Paediatr Neurol
Pays: England
ID NLM: 9715169

Informations de publication

Date de publication:
Jan 2019
Historique:
received: 13 02 2018
revised: 24 10 2018
accepted: 06 11 2018
pubmed: 12 12 2018
medline: 6 4 2019
entrez: 12 12 2018
Statut: ppublish

Résumé

Arterial ischemic stroke is rare in childhood. Often, the diagnosis is made after considerable delay. A thorough workup to pinpoint the underlying etiology is necessary, as a correct diagnosis is the determining factor in treatment decision. In case of primary angiitis of the central nervous system, treatment with corticosteroids and immunosuppressive agents is indicated. We described an eleven-year-old boy who presented at the age of six years with left hemiparesis and hemianopia. Cerebral imaging showed acute ischemia in the right posterior cerebral artery territory. Extensive workup was negative. In the following eight months, he had recurrent strokes on three separate occasions due to progressive arteriopathy involving multiple large- and medium-sized vessels. A presumed diagnosis of primary angiitis of the central nervous system was made. Pulse intravenous methylprednisolone therapy was started followed by oral prednisolone. After the fourth stroke, a six-month treatment with cyclophosphamide was given which was followed by maintenance treatment with azathioprine. Shortly after cessation of corticosteroids and cyclophosphamide the subject relapsed. Cyclophosphamide was restarted in combination with corticosteroids and subsequently replaced by mycophenolate mofetil. Under mycophenolate mofetil maintenance treatment combined with low-dose corticosteroids, the patient achieved disease control with a relapse-free period of more than four years. A guideline for current treatment of relapsing central nervous system angiitis in childhood is missing in the literature. We describe a subject with multiple relapses despite treatment with corticosteroids and immunosuppressive agents, and stabilization of his clinical condition and of the radiological signs under mycophenolate mofetil treatment.

Sections du résumé

BACKGROUND BACKGROUND
Arterial ischemic stroke is rare in childhood. Often, the diagnosis is made after considerable delay. A thorough workup to pinpoint the underlying etiology is necessary, as a correct diagnosis is the determining factor in treatment decision. In case of primary angiitis of the central nervous system, treatment with corticosteroids and immunosuppressive agents is indicated.
CASE STUDY METHODS
We described an eleven-year-old boy who presented at the age of six years with left hemiparesis and hemianopia. Cerebral imaging showed acute ischemia in the right posterior cerebral artery territory. Extensive workup was negative. In the following eight months, he had recurrent strokes on three separate occasions due to progressive arteriopathy involving multiple large- and medium-sized vessels. A presumed diagnosis of primary angiitis of the central nervous system was made. Pulse intravenous methylprednisolone therapy was started followed by oral prednisolone. After the fourth stroke, a six-month treatment with cyclophosphamide was given which was followed by maintenance treatment with azathioprine. Shortly after cessation of corticosteroids and cyclophosphamide the subject relapsed. Cyclophosphamide was restarted in combination with corticosteroids and subsequently replaced by mycophenolate mofetil. Under mycophenolate mofetil maintenance treatment combined with low-dose corticosteroids, the patient achieved disease control with a relapse-free period of more than four years.
CONCLUSION CONCLUSIONS
A guideline for current treatment of relapsing central nervous system angiitis in childhood is missing in the literature. We describe a subject with multiple relapses despite treatment with corticosteroids and immunosuppressive agents, and stabilization of his clinical condition and of the radiological signs under mycophenolate mofetil treatment.

Identifiants

pubmed: 30528093
pii: S1090-3798(18)30080-1
doi: 10.1016/j.ejpn.2018.11.003
pii:
doi:

Substances chimiques

Immunosuppressive Agents 0
Cyclophosphamide 8N3DW7272P
Prednisolone 9PHQ9Y1OLM
Mycophenolic Acid HU9DX48N0T
Azathioprine MRK240IY2L

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

222-227

Informations de copyright

Copyright © 2018 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Auteurs

Benedikte Van Driessche (B)

Department of Paediatrics, Division of Paediatric Neurology, Ghent University Hospital, C. Heymanslaan 10, B-9000, Gent, Belgium. Electronic address: Benedikte.VanDriessche@ugent.be.

Patrick Verloo (P)

Department of Paediatrics, Division of Paediatric Neurology, Ghent University Hospital, C. Heymanslaan 10, B-9000, Gent, Belgium. Electronic address: Patrick.Verloo@ugent.be.

Nele Herregods (N)

Department of Radiology, Division of Paediatric Radiology, Ghent University Hospital, C. Heymanslaan 10, B-9000, Gent, Belgium. Electronic address: Nele.Herregods@uzgent.be.

Veerle Mondelaers (V)

Department of Paediatrics, Division of Paediatric Haemato-oncology and Stem Cell Transplantation, Ghent University Hospital, C. Heymanslaan 10, B-9000, Gent, Belgium. Electronic address: Veerle.Mondelaers@uzgent.be.

Joke Dehoorne (J)

Department of Paediatrics, Division Paediatric Rheumatology, Ghent University Hospital, C. Heymanslaan 10, B-9000, Gent, Belgium. Electronic address: Joke.Dehoorne@uzgent.be.

Rudy Van Coster (R)

Department of Paediatrics, Division of Paediatric Neurology, Ghent University Hospital, C. Heymanslaan 10, B-9000, Gent, Belgium. Electronic address: Rudy.VanCoster@ugent.be.

Helene Verhelst (H)

Department of Paediatrics, Division of Paediatric Neurology, Ghent University Hospital, C. Heymanslaan 10, B-9000, Gent, Belgium. Electronic address: Helene.Verhelst@ugent.be.

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Classifications MeSH