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Journal of Korean Neurosurgical Society 2003;34(4): 375-378.
Lhermitte-Duclos Disease Associated with Cowden Disease.
Eun Seok Choi, Bong Arm Rhee, Jun Seok Koh, Tae Sung Kim
Department of Neurosurgery, School of Medicine, Kyung-Hee University, Seoul, Korea. barhee@unitel.co.kr
Lhermitte-Duclos disease, or dysplastic gangliocytoma of the cerebellum, is a peculiar hamartoma arising from the cerebellar cortex. Cowden disease, which is also termed as a multiple hamartoma-neoplasia syndrome, is an unusual autosomal dominant disorder characterized by mucocutaneous lesions, including facial papules, gingival papillomas, and acral keratoses. Other systemic hamartomas are frequently present, and there are high incidence of breast, thyroid, and genitourinary malignancies. Recently, it is accepted that Lhermitte-Duclos disease is a part of Cowden disease, a new phakomatosis. Recognition of this association has clinical significance, because diligent long-term follow-up monitoring of individuals with Lhermitte-Duclos disease and Cowden disease may lead to the early detection of malignancy.
Key Words: Lhermitte-Duclos disease; Cowden disease; Phakomatosis
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