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Moyamoya Disease: A Review of Current Literature
Moyamoya disease (MMD) is an infrequent disease of cerebral vasculature characterized by long-standing and progressive occlusion of large intracranial arteries. It is seen predominantly in the East Asian population. Most of the cases of MMD are sporadic, but there is a small percentage that is famil...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7526970/ https://www.ncbi.nlm.nih.gov/pubmed/33014640 http://dx.doi.org/10.7759/cureus.10141 |
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author | Gupta, Apurv Tyagi, Anshika Romo, Moises Amoroso, Krystal C Sonia, FNU |
author_facet | Gupta, Apurv Tyagi, Anshika Romo, Moises Amoroso, Krystal C Sonia, FNU |
author_sort | Gupta, Apurv |
collection | PubMed |
description | Moyamoya disease (MMD) is an infrequent disease of cerebral vasculature characterized by long-standing and progressive occlusion of large intracranial arteries. It is seen predominantly in the East Asian population. Most of the cases of MMD are sporadic, but there is a small percentage that is familial. The mode of inheritance is reported to be autosomal dominant with incomplete penetrance. Studies show that the susceptibility gene of MMD is located on chromosome 17. The clinical presentation is variable and is influenced by the age and geographic region of the patient. Children mainly present with ischemia-related neurologic episodes whereas MMD in adults can manifest as either an ischemic event or an intracranial hemorrhage (ICH). The gold standard investigation for diagnosis is cerebral angiography which reveals a smoky appearance of arteries at the base of the skull, thus granting the disease its name. The treatment is mostly surgical and includes direct and indirect revascularization procedures, which prevent the recurrence of both ischemic and hemorrhagic strokes. However, combination revascularization procedures are now on the rise due to studies showing better long-term outcomes. The aim of the article is to critically analyze the current literature and updates on various aspects of MMD including, but not limited to, etiology, diagnosis, and treatment. |
format | Online Article Text |
id | pubmed-7526970 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-75269702020-10-01 Moyamoya Disease: A Review of Current Literature Gupta, Apurv Tyagi, Anshika Romo, Moises Amoroso, Krystal C Sonia, FNU Cureus Neurology Moyamoya disease (MMD) is an infrequent disease of cerebral vasculature characterized by long-standing and progressive occlusion of large intracranial arteries. It is seen predominantly in the East Asian population. Most of the cases of MMD are sporadic, but there is a small percentage that is familial. The mode of inheritance is reported to be autosomal dominant with incomplete penetrance. Studies show that the susceptibility gene of MMD is located on chromosome 17. The clinical presentation is variable and is influenced by the age and geographic region of the patient. Children mainly present with ischemia-related neurologic episodes whereas MMD in adults can manifest as either an ischemic event or an intracranial hemorrhage (ICH). The gold standard investigation for diagnosis is cerebral angiography which reveals a smoky appearance of arteries at the base of the skull, thus granting the disease its name. The treatment is mostly surgical and includes direct and indirect revascularization procedures, which prevent the recurrence of both ischemic and hemorrhagic strokes. However, combination revascularization procedures are now on the rise due to studies showing better long-term outcomes. The aim of the article is to critically analyze the current literature and updates on various aspects of MMD including, but not limited to, etiology, diagnosis, and treatment. Cureus 2020-08-30 /pmc/articles/PMC7526970/ /pubmed/33014640 http://dx.doi.org/10.7759/cureus.10141 Text en Copyright © 2020, Gupta et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Neurology Gupta, Apurv Tyagi, Anshika Romo, Moises Amoroso, Krystal C Sonia, FNU Moyamoya Disease: A Review of Current Literature |
title | Moyamoya Disease: A Review of Current Literature |
title_full | Moyamoya Disease: A Review of Current Literature |
title_fullStr | Moyamoya Disease: A Review of Current Literature |
title_full_unstemmed | Moyamoya Disease: A Review of Current Literature |
title_short | Moyamoya Disease: A Review of Current Literature |
title_sort | moyamoya disease: a review of current literature |
topic | Neurology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7526970/ https://www.ncbi.nlm.nih.gov/pubmed/33014640 http://dx.doi.org/10.7759/cureus.10141 |
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