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Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy

Cystic bone lesions are the hallmark of skeletal abnormalities in patients with congenital generalized lipodystrophy (CGL). However, their pathophysiology is still unclear and theories about their origin remain largely speculative. This article reports on a patient with CGL and cystic bone lesions,...

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Autores principales: de Oliveira, TFT, Natal, MRC, Teixeira, AA, Machado, BB
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9841538/
https://www.ncbi.nlm.nih.gov/pubmed/36348607
http://dx.doi.org/10.4103/jpgm.jpgm_1031_21
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author de Oliveira, TFT
Natal, MRC
Teixeira, AA
Machado, BB
author_facet de Oliveira, TFT
Natal, MRC
Teixeira, AA
Machado, BB
author_sort de Oliveira, TFT
collection PubMed
description Cystic bone lesions are the hallmark of skeletal abnormalities in patients with congenital generalized lipodystrophy (CGL). However, their pathophysiology is still unclear and theories about their origin remain largely speculative. This article reports on a patient with CGL and cystic bone lesions, some of them with unusual magnetic resonance imaging (MRI) findings that include elevated signal intensity on T1-weighted images and fluid–fluid levels, the latter evolving to a more “classic” cystic appearance on follow-up. Even though similar findings were first described almost 30 years ago, little attention was given to them back then; furthermore, other than the present report, no other study has performed sequential exams to follow their evolution in serial MRI. The authors conduct a review of the literature, hypothesizing that these remarkable findings may reflect an intermediate stage in the process of cystification of the abnormal bone marrow, incapable to perform adipose conversion, lending factual support to the modern theories about this issue.
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spelling pubmed-98415382023-01-17 Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy de Oliveira, TFT Natal, MRC Teixeira, AA Machado, BB J Postgrad Med Case Report Cystic bone lesions are the hallmark of skeletal abnormalities in patients with congenital generalized lipodystrophy (CGL). However, their pathophysiology is still unclear and theories about their origin remain largely speculative. This article reports on a patient with CGL and cystic bone lesions, some of them with unusual magnetic resonance imaging (MRI) findings that include elevated signal intensity on T1-weighted images and fluid–fluid levels, the latter evolving to a more “classic” cystic appearance on follow-up. Even though similar findings were first described almost 30 years ago, little attention was given to them back then; furthermore, other than the present report, no other study has performed sequential exams to follow their evolution in serial MRI. The authors conduct a review of the literature, hypothesizing that these remarkable findings may reflect an intermediate stage in the process of cystification of the abnormal bone marrow, incapable to perform adipose conversion, lending factual support to the modern theories about this issue. Wolters Kluwer - Medknow 2022 2022-11-04 /pmc/articles/PMC9841538/ /pubmed/36348607 http://dx.doi.org/10.4103/jpgm.jpgm_1031_21 Text en Copyright: © 2022 Journal of Postgraduate Medicine https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
de Oliveira, TFT
Natal, MRC
Teixeira, AA
Machado, BB
Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy
title Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy
title_full Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy
title_fullStr Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy
title_full_unstemmed Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy
title_short Unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy
title_sort unusual magnetic resonance imaging findings of cystic bone lesions in congenital generalized lipodystrophy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9841538/
https://www.ncbi.nlm.nih.gov/pubmed/36348607
http://dx.doi.org/10.4103/jpgm.jpgm_1031_21
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