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...
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Thieme Medical and Scientific Publishers Pvt. Ltd.
2021
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oai:doaj.org-article:8532e86d0a0041849157e4e4778ea6962021-11-13T23:32:31ZLabrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts0971-30261998-380810.1055/s-0041-1736398https://doaj.org/article/8532e86d0a0041849157e4e4778ea6962021-07-01T00:00:00Zhttp://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1736398https://doaj.org/toc/0971-3026https://doaj.org/toc/1998-3808Leukoencephalopathy, 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.Anagha R. JoshiKiran KulkarniAnkita U. ShahThieme Medical and Scientific Publishers Pvt. Ltd.articlelabrune syndromeleukoencephalopathycerebral calcificationscystsMedical physics. Medical radiology. Nuclear medicineR895-920ENIndian Journal of Radiology and Imaging, Vol 31, Iss 03, Pp 772-775 (2021) |
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labrune syndrome leukoencephalopathy cerebral calcifications cysts Medical physics. Medical radiology. Nuclear medicine R895-920 |
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labrune syndrome leukoencephalopathy cerebral calcifications cysts Medical physics. Medical radiology. Nuclear medicine R895-920 Anagha R. Joshi Kiran Kulkarni Ankita U. Shah Labrune Syndrome: A Very Rare Association of Leukoencephalopathy, Cerebral Calcifications, and Cysts |
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 |
article |
author |
Anagha R. Joshi Kiran Kulkarni Ankita U. Shah |
author_facet |
Anagha R. Joshi Kiran Kulkarni Ankita U. Shah |
author_sort |
Anagha R. Joshi |
title |
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_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_sort |
labrune syndrome: a very rare association of leukoencephalopathy, cerebral calcifications, and cysts |
publisher |
Thieme Medical and Scientific Publishers Pvt. Ltd. |
publishDate |
2021 |
url |
https://doaj.org/article/8532e86d0a0041849157e4e4778ea696 |
work_keys_str_mv |
AT anagharjoshi labrunesyndromeaveryrareassociationofleukoencephalopathycerebralcalcificationsandcysts AT kirankulkarni labrunesyndromeaveryrareassociationofleukoencephalopathycerebralcalcificationsandcysts AT ankitaushah labrunesyndromeaveryrareassociationofleukoencephalopathycerebralcalcificationsandcysts |
_version_ |
1718430086262161408 |