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Lhermitte-Duclos disease. A case report.
Andrea Giorgianni1, Carlo Pellegrino, Alessandro De Benedictis
1Department of Neuroradiology, Circolo Fondazione Macchi Hospital; Varese, Italy - pellegrino.carlo82@gmail.com.
The Neuroradiology Journal
|December 21, 2013
Summary
Lhermitte-Duclos disease, a rare cerebellar condition, involves gangliocytoma. Neuroimaging aids diagnosis, revealing characteristic cerebellar folia hypertrophy and white matter atrophy.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Lhermitte-Duclos disease is a rare cerebellar disorder characterized by dysplastic gangliocytoma.
- It is associated with phacomatosis and Cowden's syndrome, an autosomal dominant neoplastic disorder.
Observation:
- This case study details a 31-year-old woman with Lhermitte-Duclos disease.
- Morphologic and metabolic features were assessed using conventional MRI, diffusion imaging, and spectroscopy.
Findings:
- Neuroimaging demonstrated characteristic hypertrophy of cerebellar folia.
- White matter atrophy was also observed, correlating with the disease's pathology.
Implications:
- Modern neuroimaging techniques are crucial for accurate diagnosis and management of Lhermitte-Duclos disease.
- Understanding the neuroradiologic characteristics aids in preoperative and postoperative evaluations.
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