Moyamoya Syndrome after Radiation Therapy: a Clinical Report

Moyamoya syndrome (MMS) is a rare, chronic and progressive vasculopathy with a characteristic angiographic pattern and well-recognized predisposing conditions, such as cranial therapeutic radiation. We report the case of a 36-year-old Caucasian female with a history of craniopharyngioma treated with...

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Main Authors: Paulo Almeida, Ana Rocha, Gonçalo Alves, Tiago Parreira, Maria Silva, António Cerejo, Pedro Abreu, Ana Monteiro
Format: Article
Language:English
Published: SMC MEDIA SRL 2019-12-01
Series:European Journal of Case Reports in Internal Medicine
Subjects:
Online Access:https://www.ejcrim.com/index.php/EJCRIM/article/view/1337
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author Paulo Almeida
Ana Rocha
Gonçalo Alves
Tiago Parreira
Maria Silva
António Cerejo
Pedro Abreu
Ana Monteiro
author_facet Paulo Almeida
Ana Rocha
Gonçalo Alves
Tiago Parreira
Maria Silva
António Cerejo
Pedro Abreu
Ana Monteiro
author_sort Paulo Almeida
collection DOAJ
description Moyamoya syndrome (MMS) is a rare, chronic and progressive vasculopathy with a characteristic angiographic pattern and well-recognized predisposing conditions, such as cranial therapeutic radiation. We report the case of a 36-year-old Caucasian female with a history of craniopharyngioma treated with whole-brain radiotherapy 20 years previously. She was admitted to the emergency department with disorientation and imperceptible speech lasting for 1 hour. Upon examination, she had slight motor aphasia, without sensory or motor deficits. However, the neurological deficits worsened on standing position. The computed tomography (CT) angiogram and transcranial Doppler ultrasonography revealed occlusion of the distal portion of the left internal carotid artery (ICA). Mechanical thrombectomy was attempted without success. Head CT was repeated, revealing left periventricular acute ischaemic stroke. The cerebral angiography showed total occlusion of the left ICA with an exuberant network of transdural collateral vessels, confirming MMS. The patient completed a functional rehabilitation program with progressive improvement of deficits and maintained a multidisciplinary follow-up. MMS is a serious late complication from cranial radiation therapy and a well-described risk factor for ischaemic stroke in younger patients. Therefore, early detection and prompt treatment are mandatory, as the consequences can be disastrous, such as cognitive and neurologic decline due to repeated strokes.
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spelling doaj.art-7dfb885fdb7547c69e6a6a26c8b5d0d52022-12-22T01:32:23ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942019-12-0110.12890/2019_0013371337Moyamoya Syndrome after Radiation Therapy: a Clinical ReportPaulo Almeida0Ana Rocha1Gonçalo Alves2Tiago ParreiraMaria Silva3António Cerejo4Pedro Abreu5Ana Monteiro6Department of Internal Medicine, Centro Hospitalar Baixo Vouga, Aveiro, Portugal>Department of Neurology, Centro Hospitalar Universitário São João, Porto, PortugalDepartment of Neuroradiology, Centro Hospitalar Universitário São João, Porto, PortugalDepartment of Neuroradiology, Centro Hospitalar Universitário São João, Porto, PortugalClinical Neurosciences and Mental Health Department, Faculty of Medicine of the University of Porto, PortugalDepartment of Neurology, Centro Hospitalar Universitário São João, Porto, PortugalDepartment of Neurology, Centro Hospitalar Universitário São João, Porto, PortugalMoyamoya syndrome (MMS) is a rare, chronic and progressive vasculopathy with a characteristic angiographic pattern and well-recognized predisposing conditions, such as cranial therapeutic radiation. We report the case of a 36-year-old Caucasian female with a history of craniopharyngioma treated with whole-brain radiotherapy 20 years previously. She was admitted to the emergency department with disorientation and imperceptible speech lasting for 1 hour. Upon examination, she had slight motor aphasia, without sensory or motor deficits. However, the neurological deficits worsened on standing position. The computed tomography (CT) angiogram and transcranial Doppler ultrasonography revealed occlusion of the distal portion of the left internal carotid artery (ICA). Mechanical thrombectomy was attempted without success. Head CT was repeated, revealing left periventricular acute ischaemic stroke. The cerebral angiography showed total occlusion of the left ICA with an exuberant network of transdural collateral vessels, confirming MMS. The patient completed a functional rehabilitation program with progressive improvement of deficits and maintained a multidisciplinary follow-up. MMS is a serious late complication from cranial radiation therapy and a well-described risk factor for ischaemic stroke in younger patients. Therefore, early detection and prompt treatment are mandatory, as the consequences can be disastrous, such as cognitive and neurologic decline due to repeated strokes.https://www.ejcrim.com/index.php/EJCRIM/article/view/1337radiotherapymoyamoya syndromecraniopharyngioma
spellingShingle Paulo Almeida
Ana Rocha
Gonçalo Alves
Tiago Parreira
Maria Silva
António Cerejo
Pedro Abreu
Ana Monteiro
Moyamoya Syndrome after Radiation Therapy: a Clinical Report
European Journal of Case Reports in Internal Medicine
radiotherapy
moyamoya syndrome
craniopharyngioma
title Moyamoya Syndrome after Radiation Therapy: a Clinical Report
title_full Moyamoya Syndrome after Radiation Therapy: a Clinical Report
title_fullStr Moyamoya Syndrome after Radiation Therapy: a Clinical Report
title_full_unstemmed Moyamoya Syndrome after Radiation Therapy: a Clinical Report
title_short Moyamoya Syndrome after Radiation Therapy: a Clinical Report
title_sort moyamoya syndrome after radiation therapy a clinical report
topic radiotherapy
moyamoya syndrome
craniopharyngioma
url https://www.ejcrim.com/index.php/EJCRIM/article/view/1337
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