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[Lhermitte-Duclos disease. Case report and review of the literature]
Abstract:
A new case of Lhermitte-Duclos disease was recognized as an accidental finding during the autopsy of a 58-year-old male who had died because of acute enteritis and shock-induced cardiac failure. Gross examination revealed focally disturbed cerebellar cortical structure in two foci. Microscopical investigation resulted in findings typical of Lhermitte-Duclos disease: 1. Broadened molecular layer containing myelinated axonal projections; 2. Disappearance of Purkinje cells; 3. Progressive hypertrophy of the granular cells within the granular layer; 4. Loss of the central white matter core of the cerebellar folia. The etiology and pathogenesis of the lesion is still unclear. With this first report in Hungarian the authors wish to call the attention to this rare disease.
Insights
This report details a rare case of Lhermitte-Duclos disease discovered incidentally during an autopsy. The findings highlight characteristic pathological changes in the cerebellum, contributing to understanding this uncommon neurological condition.
Area of Science:
- Neuropathology
- Cerebellar Diseases
Background:
- Lhermitte-Duclos disease, also known as adult-onset cerebellar gangliomatosis, is a rare condition characterized by cerebellar dysplastic lesions.
- The exact etiology and pathogenesis remain largely unknown, necessitating further research and case reporting.
Observation:
- A 58-year-old male, deceased from unrelated causes (acute enteritis and shock-induced cardiac failure), was found to have incidental cerebellar abnormalities during autopsy.
- Gross examination revealed two distinct foci of disturbed cerebellar cortical structure.
Findings:
- Microscopic analysis confirmed features typical of Lhermitte-Duclos disease.
- Key pathological hallmarks included a broadened molecular layer with axonal projections, Purkinje cell loss, granular cell hypertrophy, and loss of the cerebellar folia's white matter core.
Implications:
- This case contributes to the limited literature on Lhermitte-Duclos disease, particularly from a neuropathological perspective.
- Increased awareness and reporting of such rare conditions are crucial for advancing diagnostic and etiological understanding.