Lhermitte-Duclos disease (LDD): Report of the first case registered in Paraguay with radiological-pathological correlation

Authors

  • Violeta Ricart Universidad Nacional de Asunción, Facultad de Ciencias Médicas, San Lorenzo, Paraguay https://orcid.org/0009-0001-5257-4888
  • Bárbara Castellano Universidad Nacional de Asunción, Facultad de Ciencias Médicas, San Lorenzo, Paraguay https://orcid.org/0000-0002-8601-5953
  • Christian Aguayo Universidad Nacional de Asunción, Facultad de Ciencias Médicas, San Lorenzo, Paraguay https://orcid.org/0009-0008-3973-6097
  • Edgar Martínez Barrios Universidad Nacional de Asunción, Facultad de Ciencias Médicas, San Lorenzo, Paraguay
  • Italo Flecha Universidad Nacional de Asunción, Facultad de Ciencias Médicas, San Lorenzo, Paraguay
  • Judith Oertell Universidad Nacional de Asunción, Facultad de Ciencias Médicas, San Lorenzo, Paraguay https://orcid.org/0000-0002-1488-8018

DOI:

https://doi.org/10.52379/mcs.v7i3.328

Keywords:

Lhermitte- Duclos, Gangliocytoma , cerebellar , Neuroradiology

Abstract

Lhermitte-Duclos disease, or cerebellar dysplastic gangliocytoma, is a rare entity that manifests as a slow-growing expansive process in the posterior fossa, specifically in the cerebellum, with a pathogenesis that is not well understood, which may be related to congenital anomalies and PTEN gene mutations (phosphatase and tensin homologue), especially in the form of adult presentation. Documented cases of this disease are scarce, with around 230 records in the medical literature and a prevalence of less than 1 per million people. We present the first case in Paraguay of dysplastic gangliocytoma or Lhermitte-Duclos disease in a 44-year-old male patient, with no relevant medical or family history, who underwent surgery due to an expansive process in the posterior fossa, with characteristic findings on magnetic resonance, which allowed preoperative suspicion of the disease, with differential diagnosis of medulloblastoma, due to restrictions observed in the Diffusion sequence. The resection was complete with adequate evolution and without relapses of the process.

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Published

09/11/2023

Issue

Vol. 7 No. 3 (2023): SETIEMBRE-DICIEMBRE

Section

Case Reports

License

Copyright (c) 2023 Violeta Ricart, Bárbara Castellano, Christian Aguayo, Edgar Martínez Barrios, Italo Flecha, Judith Oertell

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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