‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult
Moyamoya disease is a rare neurological condition that affects children and adults of all ages. It is characterized by chronic, progressive stenosis of the circle of Willis that ultimately leads to the development of extensive collateral vessels. Presenting symptoms are usually due to cerebral ische...
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doaj-4f6dad4407f64b0c90f81342d6e7bce22020-11-25T00:12:09ZengTaylor & Francis GroupJournal of Community Hospital Internal Medicine Perspectives2000-96662015-06-01501410.3402/jchimp.v5.2766427664‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adultImtiaz Ismail0Khalid Al-Khafaji1Monica Mutyala2Saurabh Aggarwal3Nawfal Al-Khafaji4Daniela Kovacs5Sandeep Khosla6Rohit Arora7 Department of Internal Medicine, Rosalind Franklin University of Medicine and Science, Waukegan, IL, USA Department of Internal Medicine, Rosalind Franklin University of Medicine and Science, Waukegan, IL, USA Department of Internal Medicine, Rosalind Franklin University of Medicine and Science, Waukegan, IL, USA Department of Cardiology, Creighton University, Omaha, NE, USA Department of Internal Medicine, Rosalind Franklin University of Medicine and Science, Waukegan, IL, USA Department of Internal Medicine, Rosalind Franklin University of Medicine and Science, Waukegan, IL, USA Department of Internal Medicine, Rosalind Franklin University of Medicine and Science, Waukegan, IL, USA Department of Internal Medicine, Rosalind Franklin University of Medicine and Science, Waukegan, IL, USAMoyamoya disease is a rare neurological condition that affects children and adults of all ages. It is characterized by chronic, progressive stenosis of the circle of Willis that ultimately leads to the development of extensive collateral vessels. Presenting symptoms are usually due to cerebral ischemia or hemorrhage. The Japanese term moyamoya (meaning puffy or obscure) was coined to describe the characteristic ‘smoke in the air’ appearance of these vessels on cerebral angiography. Moyamoya has the highest recorded incidence in Japan (0.28 per 100,000). In the west it is an extremely rare condition with an overall incidence of (0.086 per 100,000) in the Western United States. Etiology for the most part is unknown; however, genetic susceptibility related to RNF213 gene on chromosome 17q25.3 has been suggested. Moyamoya is being diagnosed more frequently in all races with varying clinical manifestations. Moyamoya disease is a rare progressive neurologic condition characterized by occlusion of the cerebral circulation with extensive collaterals recruitment in children and adults. Distinguished radiological findings confirm the diagnosis. Early recognition and swift institution of therapy is vital in order to minimize neurological deficits. We present the case of a 19-year-old African American female who presented with left-sided parastheia, weakness, and headache for 2 days duration.http://www.jchimp.net/index.php/jchimp/article/view/27664/pdf_58MoyamoyaneurologyJapanesecircle of Williscollateralssmoke |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Imtiaz Ismail Khalid Al-Khafaji Monica Mutyala Saurabh Aggarwal Nawfal Al-Khafaji Daniela Kovacs Sandeep Khosla Rohit Arora |
spellingShingle |
Imtiaz Ismail Khalid Al-Khafaji Monica Mutyala Saurabh Aggarwal Nawfal Al-Khafaji Daniela Kovacs Sandeep Khosla Rohit Arora ‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult Journal of Community Hospital Internal Medicine Perspectives Moyamoya neurology Japanese circle of Willis collaterals smoke |
author_facet |
Imtiaz Ismail Khalid Al-Khafaji Monica Mutyala Saurabh Aggarwal Nawfal Al-Khafaji Daniela Kovacs Sandeep Khosla Rohit Arora |
author_sort |
Imtiaz Ismail |
title |
‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult |
title_short |
‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult |
title_full |
‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult |
title_fullStr |
‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult |
title_full_unstemmed |
‘Smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult |
title_sort |
‘smoke in the air’: a rare cerebrovascular cause of neurological signs and symptoms in a young adult |
publisher |
Taylor & Francis Group |
series |
Journal of Community Hospital Internal Medicine Perspectives |
issn |
2000-9666 |
publishDate |
2015-06-01 |
description |
Moyamoya disease is a rare neurological condition that affects children and adults of all ages. It is characterized by chronic, progressive stenosis of the circle of Willis that ultimately leads to the development of extensive collateral vessels. Presenting symptoms are usually due to cerebral ischemia or hemorrhage. The Japanese term moyamoya (meaning puffy or obscure) was coined to describe the characteristic ‘smoke in the air’ appearance of these vessels on cerebral angiography. Moyamoya has the highest recorded incidence in Japan (0.28 per 100,000). In the west it is an extremely rare condition with an overall incidence of (0.086 per 100,000) in the Western United States. Etiology for the most part is unknown; however, genetic susceptibility related to RNF213 gene on chromosome 17q25.3 has been suggested. Moyamoya is being diagnosed more frequently in all races with varying clinical manifestations. Moyamoya disease is a rare progressive neurologic condition characterized by occlusion of the cerebral circulation with extensive collaterals recruitment in children and adults. Distinguished radiological findings confirm the diagnosis. Early recognition and swift institution of therapy is vital in order to minimize neurological deficits. We present the case of a 19-year-old African American female who presented with left-sided parastheia, weakness, and headache for 2 days duration. |
topic |
Moyamoya neurology Japanese circle of Willis collaterals smoke |
url |
http://www.jchimp.net/index.php/jchimp/article/view/27664/pdf_58 |
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