Abstract

RICART-MAS, Violeta Nohelí et al. Lhermitte-Duclos disease (LDD): Report of the first case registered in Paraguay with radiological-pathological correlation. Med. clín. soc. [online]. 2023, vol.7, n.3, pp.236-241. ISSN 2521-2281.  https://doi.org/10.52379/mcs.v7i3.328.

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.

Keywords : Lhermitte- Duclos; gangliocitoma; displásico; cerebeloso; neurorradiología..

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