Recurrent arterial ischemic stroke with good response to mycophenolate mofetil.
Azathioprine
/ therapeutic use
Child
Cyclophosphamide
/ therapeutic use
Drug Therapy, Combination
Humans
Immunosuppressive Agents
/ therapeutic use
Male
Mycophenolic Acid
/ therapeutic use
Prednisolone
/ therapeutic use
Recurrence
Stroke
/ drug therapy
Vasculitis, Central Nervous System
/ complications
Arterial ischemic stroke
Childhood
Mycophenolate mofetil
Primary angiitis of the central nervous system
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
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
222-227Informations de copyright
Copyright © 2018 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.