Biopsy-proven primary angiitis of the central nervous system mimicking leukodystrophy: A case report and review of the literature.
Angiitis
Biopsy
CNS
Leukodystrophy
Leukoencephalopathy
MRI
Journal
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
ISSN: 1532-2653
Titre abrégé: J Clin Neurosci
Pays: Scotland
ID NLM: 9433352
Informations de publication
Date de publication:
Jun 2019
Jun 2019
Historique:
received:
12
01
2019
accepted:
11
03
2019
pubmed:
27
3
2019
medline:
2
7
2019
entrez:
27
3
2019
Statut:
ppublish
Résumé
Primary Angiitis of the Central Nervous System (PACNS) is a rare form of idiopathic CNS vasculitis. Neuroimaging is often abnormal and characterized by multifocal brain lesions, but brain biopsy definitely confirms the diagnosis. We report the rare case of a 45-year-old female presenting with symptoms of intracranial hypertension and leukodystrophy-like neuroimaging findings. A comprehensive diagnostic work-up led to the unexpected diagnosis of a definite PACNS which was successfully treated by immunosuppressive treatment. Although rarely, PACNS can present as diffuse leukoencephalopathy on neuroimaging, and mimic even an inherited leukodystrophy. Therefore, in adults with leukodystrophy-like neuroimaging findings, careful examination of clinical and non-clinical features is mandatory to avoid missing the diagnosis of a treatable acquired disease.
Identifiants
pubmed: 30910547
pii: S0967-5868(19)30070-0
doi: 10.1016/j.jocn.2019.03.021
pii:
doi:
Substances chimiques
Immunosuppressive Agents
0
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
42-44Informations de copyright
Copyright © 2019 Elsevier Ltd. All rights reserved.