Cargando…
Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts
Leukoencephalopathy, cerebral calcifications, and cysts (LCC) form a very rare association which is named as “Labrune syndrome” after Labrune who reported the first case in 1996. To the best of our knowledge only eight to 10 cases have been reported in literature to date. We report a case of a 26-ye...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Thieme Medical and Scientific Publishers Pvt. Ltd.
2021
|
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8590554/ https://www.ncbi.nlm.nih.gov/pubmed/34790336 http://dx.doi.org/10.1055/s-0041-1736398 |
_version_ | 1784598998823206912 |
---|---|
author | Joshi, Anagha R. Kulkarni, Kiran Shah, Ankita U. |
author_facet | Joshi, Anagha R. Kulkarni, Kiran Shah, Ankita U. |
author_sort | Joshi, Anagha R. |
collection | PubMed |
description | Leukoencephalopathy, cerebral calcifications, and cysts (LCC) form a very rare association which is named as “Labrune syndrome” after Labrune who reported the first case in 1996. To the best of our knowledge only eight to 10 cases have been reported in literature to date. We report a case of a 26-year-old male with onset of neurological symptoms in late adolescence (at 19 years of age) and presented with complains of continued seizures for 7 years, giddiness with imbalance, and slowly progressive motor symptoms. MRI brain revealed multiple calcifications in bilateral basal ganglia, cerebral white matter, multiple cystic lesions in the supratentorial white matter, and abnormal diffused bilateral white matter T2 hyperintensity suggesting leukoencephalopathy. Histopathological evaluation revealed prominent congested blood vessels suggestive of angiomatous changes and cystic areas suggestive of secondary gliosis. |
format | Online Article Text |
id | pubmed-8590554 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Thieme Medical and Scientific Publishers Pvt. Ltd. |
record_format | MEDLINE/PubMed |
spelling | pubmed-85905542021-11-16 Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts Joshi, Anagha R. Kulkarni, Kiran Shah, Ankita U. Indian J Radiol Imaging Leukoencephalopathy, cerebral calcifications, and cysts (LCC) form a very rare association which is named as “Labrune syndrome” after Labrune who reported the first case in 1996. To the best of our knowledge only eight to 10 cases have been reported in literature to date. We report a case of a 26-year-old male with onset of neurological symptoms in late adolescence (at 19 years of age) and presented with complains of continued seizures for 7 years, giddiness with imbalance, and slowly progressive motor symptoms. MRI brain revealed multiple calcifications in bilateral basal ganglia, cerebral white matter, multiple cystic lesions in the supratentorial white matter, and abnormal diffused bilateral white matter T2 hyperintensity suggesting leukoencephalopathy. Histopathological evaluation revealed prominent congested blood vessels suggestive of angiomatous changes and cystic areas suggestive of secondary gliosis. Thieme Medical and Scientific Publishers Pvt. Ltd. 2021-11-13 /pmc/articles/PMC8590554/ /pubmed/34790336 http://dx.doi.org/10.1055/s-0041-1736398 Text en Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ) https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License, which permits unrestricted reproduction and distribution, for non-commercial purposes only; and use and reproduction, but not distribution, of adapted material for non-commercial purposes only, provided the original work is properly cited. |
spellingShingle | Joshi, Anagha R. Kulkarni, Kiran Shah, Ankita U. Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts |
title | Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts |
title_full | Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts |
title_fullStr | Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts |
title_full_unstemmed | Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts |
title_short | Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts |
title_sort | labrune syndrome: a very rare association of leukoencephalopathy, cerebral calcifications, and cysts |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8590554/ https://www.ncbi.nlm.nih.gov/pubmed/34790336 http://dx.doi.org/10.1055/s-0041-1736398 |
work_keys_str_mv | AT joshianaghar labrunesyndromeaveryrareassociationofleukoencephalopathycerebralcalcificationsandcysts AT kulkarnikiran labrunesyndromeaveryrareassociationofleukoencephalopathycerebralcalcificationsandcysts AT shahankitau labrunesyndromeaveryrareassociationofleukoencephalopathycerebralcalcificationsandcysts |