Neurovascular manifestations in connective tissue diseases: The case of Marfan Syndrome.
Mech Ageing Dev
; 191: 111346, 2020 10.
Article
en En
| MEDLINE
| ID: mdl-32920077
ABSTRACT
Patients with connective tissue diseases (CTDs) are suspected to be at higher risk for cerebrovascular involvement, such as intracranial aneurysms, dissections and strokes, than the general population. Particularly, Marfan Syndrome (MFS) has been reported as associated with an increased risk of cerebrovascular alterations. Literature data report different prevalence of intracranial aneurysms in MFS, ranging from 4 % to 29 %, suggesting a role of genetic cause that involves the regulation of the TGF-ß signaling. Ischemic and hemorrhagic strokes have been also reported in MFS, but with an estimated prevalence from 3 % to 4 %. However, the aetiology of both events appears to be reliable more to a cardiac source than to the primary connective tissue defect. Finally, the available literature suggests that MFS patients have a higher prevalence of arterial tortuosity of neck and head vessels and these findings may be related to an enhanced chance of dissection. Overall, despite of the lack of studies, we could affirm that it may exists an increased prevalence of some neurovascular findings in MFS patients. Nevertheless, further studies are required to determine the true prevalence of these features and investigate specific gene mutations involved in MFS.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Transducción de Señal
/
Aneurisma Intracraneal
/
Factor de Crecimiento Transformador beta
/
Accidente Cerebrovascular Isquémico
/
Accidente Cerebrovascular Hemorrágico
/
Síndrome de Marfan
Tipo de estudio:
Prevalence_studies
/
Risk_factors_studies
Límite:
Humans
Idioma:
En
Revista:
Mech Ageing Dev
Año:
2020
Tipo del documento:
Article
País de afiliación:
Italia