Magnetic Resonance Imaging Characteristics of Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic Manifestations.
Clin Neuroradiol
; 30(2): 229-236, 2020 Jun.
Article
em En
| MEDLINE
| ID: mdl-30627749
ABSTRACT
BACKGROUND:
Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a rare hereditary disease presenting with distinct imaging features in middle-aged adults. This article describes the typical imaging features focusing on the longitudinal course of RVCL-S lesions.METHODS:
In this study six subjects (five male, five related) with RVCL-S were retrospectively included from two university hospitals. The median age of symptom onset was 40⯱ 6 years. Magnetic resonance imaging (MRI) covering baseline and a median follow-up period of 33 months was reviewed in a structured way focusing on morphology, contrast enhancement and diffusion restriction of brain lesions.RESULTS:
All patients showed patchy, T2 hyperintense white matter lesions (mean number 7.7⯱ 1.8) with a periventricular predominance at the frontal lobes (59%). In all subjects, rim-enhancing white matter lesions with temporary diffusion restriction were present for a mean of 5.0⯱ 3.9 months. Median duration of blood brain barrier disruption was 20 months.CONCLUSION:
Periventricular patchy white matter lesions in the frontal lobes as well as rim-enhancing lesions with prolonged diffusion restriction and long-lasting contrast enhancement are characteristic imaging findings in RCVL-S and can be helpful in the differential diagnosis.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Artéria Retiniana
/
Veia Retiniana
/
Doenças Vasculares
/
Imageamento por Ressonância Magnética
/
Leucoencefalopatias
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article