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Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan
BACKGROUND: Moyamoya is a rare cerebrovascular disease of unknown etiology. The data on moyamoya disease from Pakistan is sparse. We report a case series of 13 patients who presented with moyamoya disease to a tertiary care hospital in Pakistan with a national referral base. METHODS: We conducted a...
Autores principales: | , , , , |
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Formato: | Texto |
Lenguaje: | English |
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BioMed Central
2009
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2678270/ https://www.ncbi.nlm.nih.gov/pubmed/19368734 http://dx.doi.org/10.1186/1471-2377-9-15 |
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author | Shoukat, Sana Itrat, Ahmed Taqui, Ather M Zaidi, Moazzam Kamal, Ayeesha K |
author_facet | Shoukat, Sana Itrat, Ahmed Taqui, Ather M Zaidi, Moazzam Kamal, Ayeesha K |
author_sort | Shoukat, Sana |
collection | PubMed |
description | BACKGROUND: Moyamoya is a rare cerebrovascular disease of unknown etiology. The data on moyamoya disease from Pakistan is sparse. We report a case series of 13 patients who presented with moyamoya disease to a tertiary care hospital in Pakistan with a national referral base. METHODS: We conducted a retrospective review of thirteen patients who presented to The Aga Khan University and diagnosed with "Moyamoya Disease" during the period 1988 – 2006. These patients were identified from existing hospital database via ICD-9 codes. A predesigned questionnaire containing information about clinical presentation, management and neuroimaging was administered to all identified patients. RESULTS: There were seven males and six females. Mean age at presentation was 16.5 years and a female predominance was found in the pediatric age group (n = 10, 71.4%). Stroke (n = 11, 84.2%) was the most common presentation with motor deficit being the universal cortical symptom. Fever was a common symptom in the lower age groups (n = 4, 51.7%). Cerebral Angiography and Magnetic Resonance Angiography showed bilateral involvement of the vessels in eleven patients while unilateral in two. Subarachnoid and interventricular haemorrhage appeared in 2(15.4%) adults. Twelve (92.3%) patients were discharged as independent with minor deficits regardless of therapeutic modality. Only three (23.0%) patients underwent surgery whereas the remaining were managed conservatively. CONCLUSION: Physicians when dealing with childhood strokes and characteristic deficits in adult population should consider Moyamoya disease. |
format | Text |
id | pubmed-2678270 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2009 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-26782702009-05-08 Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan Shoukat, Sana Itrat, Ahmed Taqui, Ather M Zaidi, Moazzam Kamal, Ayeesha K BMC Neurol Research Article BACKGROUND: Moyamoya is a rare cerebrovascular disease of unknown etiology. The data on moyamoya disease from Pakistan is sparse. We report a case series of 13 patients who presented with moyamoya disease to a tertiary care hospital in Pakistan with a national referral base. METHODS: We conducted a retrospective review of thirteen patients who presented to The Aga Khan University and diagnosed with "Moyamoya Disease" during the period 1988 – 2006. These patients were identified from existing hospital database via ICD-9 codes. A predesigned questionnaire containing information about clinical presentation, management and neuroimaging was administered to all identified patients. RESULTS: There were seven males and six females. Mean age at presentation was 16.5 years and a female predominance was found in the pediatric age group (n = 10, 71.4%). Stroke (n = 11, 84.2%) was the most common presentation with motor deficit being the universal cortical symptom. Fever was a common symptom in the lower age groups (n = 4, 51.7%). Cerebral Angiography and Magnetic Resonance Angiography showed bilateral involvement of the vessels in eleven patients while unilateral in two. Subarachnoid and interventricular haemorrhage appeared in 2(15.4%) adults. Twelve (92.3%) patients were discharged as independent with minor deficits regardless of therapeutic modality. Only three (23.0%) patients underwent surgery whereas the remaining were managed conservatively. CONCLUSION: Physicians when dealing with childhood strokes and characteristic deficits in adult population should consider Moyamoya disease. BioMed Central 2009-04-15 /pmc/articles/PMC2678270/ /pubmed/19368734 http://dx.doi.org/10.1186/1471-2377-9-15 Text en Copyright © 2009 Shoukat et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Research Article Shoukat, Sana Itrat, Ahmed Taqui, Ather M Zaidi, Moazzam Kamal, Ayeesha K Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan |
title | Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan |
title_full | Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan |
title_fullStr | Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan |
title_full_unstemmed | Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan |
title_short | Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan |
title_sort | moyamoya disease: a clinical spectrum, literature review and case series from a tertiary care hospital in pakistan |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2678270/ https://www.ncbi.nlm.nih.gov/pubmed/19368734 http://dx.doi.org/10.1186/1471-2377-9-15 |
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