ArticleJournal of neurosciences in rural practice
Lhermitte-Duclos disease: A series of six cases.
Article in Journal of neurosciences in rural practice. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 7 citations in OpenAlex.
- Recognizing genetic association between Lhermitte-Duclos Disease and Autism Spectrum Disorder in PTEN-related patients: a case report and literature comprehension.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026Review
- Recurrent multiloculated cystic variant of Lhermitte-Duclos disease managed with cyst fenestration and Ommaya reservoir placement: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Case Report: Bilateral tonic-clonic seizures as initial presentation of Lhermitte-Duclos Disease and Cowden Syndrome.Frontiers in oncology · 2026Article
- Two illustrative cases of adult Lhermitte-Duclos disease and a systematic review of literature related to surgical management.Brain & spine · 2025Article
- Case Report: A case series of Lhermitte-Duclos disease with surgical intervention.Frontiers in oncology · 2025Article
- Lhermitte-Duclos Disease: A Case Series.Cureus · 2023Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare lesion characterized by variable enlargement of cerebellar folia. The pathological basis of LDD has long been debated, as it has overlapping features of both, a neoplasm and hamartoma. Association between LDD and Cowden syndrome (CS) has been established based on the presence of phosphatase and tensin homologue germline mutation in both. We present a series of six cases of LDD: Four females and two males, aged between 16 and 38 years, presenting with headache and imbalance on walking of 1-7 months duration. Histomorphology showed thickening and vacuolation of the molecular layer, loss of Purkinje cells, and replacement of granular cell layer by large dysplastic ganglion cells. Awareness of histological features of this rare entity and a higher level of suspicion is required for the correct diagnosis, which, in turn, should prompt thorough investigations to exclude features of associated CS. LDD is a rare entity, awareness of its histological features and correlating them with radiology is essential, especially in tiny biopsies; to render the correct diagnosis. Diagnosis of LDD warrants further clinical workup and close follow-up for the associated features of CS.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.