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Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients

Background and purpose: The aim of our study is to present, for the first time, the clinical, radiological, and neurocognitive characteristics of Greek adult patients with Moyamoya disease (MMD). Methods: We analyzed prospectively collected data of 12 patients referred to our department from 2004 to...

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Autores principales: Vassilopoulou, Sofia, Tountopoulou, Argyro, Korompoki, Eleni, Papageorgiou, Georgios, Kasselimis, Dimitrios, Velonakis, Georgios, Chatziioannou, Achilles, Potagas, Constantin, Spengos, Konstantinos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10531977/
https://www.ncbi.nlm.nih.gov/pubmed/37762892
http://dx.doi.org/10.3390/jcm12185951
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author Vassilopoulou, Sofia
Tountopoulou, Argyro
Korompoki, Eleni
Papageorgiou, Georgios
Kasselimis, Dimitrios
Velonakis, Georgios
Chatziioannou, Achilles
Potagas, Constantin
Spengos, Konstantinos
author_facet Vassilopoulou, Sofia
Tountopoulou, Argyro
Korompoki, Eleni
Papageorgiou, Georgios
Kasselimis, Dimitrios
Velonakis, Georgios
Chatziioannou, Achilles
Potagas, Constantin
Spengos, Konstantinos
author_sort Vassilopoulou, Sofia
collection PubMed
description Background and purpose: The aim of our study is to present, for the first time, the clinical, radiological, and neurocognitive characteristics of Greek adult patients with Moyamoya disease (MMD). Methods: We analyzed prospectively collected data of 12 patients referred to our department from 2004 to 2019. All patients underwent a thorough diagnostic work up, including extensive clinical, neuroradiological, and neurocognitive assessment. Results: Our study population consisted of 7 females and the median age at the time of the diagnosis was 43.5 years. No patient had a positive family history of the disease and roughly 50% were hypertensives. Ten patients presented with transient or permanent cerebrovascular ischemia and two patients suffered from hemorrhagic complications. The median NIHSS was 7.5 (0–23) and clinical status remained stable during follow-up with conservative treatment in most of the patients. The majority (83.3%) had bilateral disease confirmed by DSA. All lesions exclusively affected the anterior circulation, with 50% of patients presenting with stenoocclusive changes. No aneurysm or AVM were revealed. The most common neurocognitive deficits were in the executive and language domains. Conclusions: Our MMD patients had a later onset of the disease and an absence of familial occurrence. The most common manifestation was ischemia, transient or permanent, and all lesions affected the anterior circulation, whereas no vascular malformations (AVM, aneurysms) were demonstrated in brain imaging. These findings in Greek patients imply a probable different, Mediterranean phenotype.
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spelling pubmed-105319772023-09-28 Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients Vassilopoulou, Sofia Tountopoulou, Argyro Korompoki, Eleni Papageorgiou, Georgios Kasselimis, Dimitrios Velonakis, Georgios Chatziioannou, Achilles Potagas, Constantin Spengos, Konstantinos J Clin Med Article Background and purpose: The aim of our study is to present, for the first time, the clinical, radiological, and neurocognitive characteristics of Greek adult patients with Moyamoya disease (MMD). Methods: We analyzed prospectively collected data of 12 patients referred to our department from 2004 to 2019. All patients underwent a thorough diagnostic work up, including extensive clinical, neuroradiological, and neurocognitive assessment. Results: Our study population consisted of 7 females and the median age at the time of the diagnosis was 43.5 years. No patient had a positive family history of the disease and roughly 50% were hypertensives. Ten patients presented with transient or permanent cerebrovascular ischemia and two patients suffered from hemorrhagic complications. The median NIHSS was 7.5 (0–23) and clinical status remained stable during follow-up with conservative treatment in most of the patients. The majority (83.3%) had bilateral disease confirmed by DSA. All lesions exclusively affected the anterior circulation, with 50% of patients presenting with stenoocclusive changes. No aneurysm or AVM were revealed. The most common neurocognitive deficits were in the executive and language domains. Conclusions: Our MMD patients had a later onset of the disease and an absence of familial occurrence. The most common manifestation was ischemia, transient or permanent, and all lesions affected the anterior circulation, whereas no vascular malformations (AVM, aneurysms) were demonstrated in brain imaging. These findings in Greek patients imply a probable different, Mediterranean phenotype. MDPI 2023-09-13 /pmc/articles/PMC10531977/ /pubmed/37762892 http://dx.doi.org/10.3390/jcm12185951 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Vassilopoulou, Sofia
Tountopoulou, Argyro
Korompoki, Eleni
Papageorgiou, Georgios
Kasselimis, Dimitrios
Velonakis, Georgios
Chatziioannou, Achilles
Potagas, Constantin
Spengos, Konstantinos
Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients
title Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients
title_full Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients
title_fullStr Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients
title_full_unstemmed Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients
title_short Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients
title_sort moyamoya disease: clinical and radiological characteristics in adult greek patients
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10531977/
https://www.ncbi.nlm.nih.gov/pubmed/37762892
http://dx.doi.org/10.3390/jcm12185951
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