Lhermitte-Duclos disease is a clinical manifestation of Cowden's syndrome

Citation
N. Vantomme et al., Lhermitte-Duclos disease is a clinical manifestation of Cowden's syndrome, SURG NEUROL, 56(3), 2001, pp. 201-204
Citations number
32
Categorie Soggetti
Neurology
Journal title
SURGICAL NEUROLOGY
ISSN journal
00903019 → ACNP
Volume
56
Issue
3
Year of publication
2001
Pages
201 - 204
Database
ISI
SICI code
0090-3019(200109)56:3<201:LDIACM>2.0.ZU;2-C
Abstract
BACKGROUND Lhermitte-Duclos disease (LDD) is a hamartomatous overgrowth of cerebellar ganglion cells, which replace granular cells and Purkinje cells. In recent years several cases involving the association between LDD and Co wden's syndrome (CS), an autosomal dominant condition characterized by mult iple hamartomas and neoplastic lesions in skin and internal organs, have be en reported. METHODS We reviewed the medical records and imaging studies of six patients with LDD who were treated at our institution, and we looked at other possi ble symptoms of CS. RESULTS Other clinical findings suggestive of CS were apparent in five pati ents: These included mucocutaneous lesions, acral keratosis, thyroid adenom a, fibrocystic disease ovarian cyst, intestinal polyposis, and arteriovenou s malformation. Only in the youngest patient, a 5-year-old boy, were no cut aneous or other signs found, despite extensive clinical and ultrasound exam ination. CONCLUSION Our observations strengthen the hypothesis that LDD is a neurolo gical manifestion of CS. Patients with LDD should receive a thorough dermat ological and systemic screening, because some of the lesions (breast, etc.. .) can develop into malignant tumors. (C) 2001 by Elsevier Science Inc.