ASSOCIATION OF LHERMITTE-DUCLOS AND COWDEN DISEASE - REPORT OF A NEW CASE AND REVIEW OF THE LITERATURE

Lhermitte-Duclos disease is a rare entity, and its pathological featur es are unique. Pathological findings are characteristic of the disease , with global hypertrophy of the cerebellum, coarse gyri, and the typi cal ''inverted cortex'' pattern. Several associated lesions were noted in many patients with Lhermitte-Duclos disease. It is only recently t hat an association between Lhermitte-Duclos disease and Cowden disease was reported. Cowden disease, or multiple hamartomas syndrome, is a f amilial disease associating breast cancer, cutaneomucous tricholemmoma s, and various other tumoural and dysplastic conditions. A new case of Lhermitte-Duclos disease associated with Cowden disease is reported. A review of the literature found 72 cases of Lhermitte-Duclos disease; 26 had conditions suggesting Cowden disease and seven were definite c ases of Cowden disease. The association of Lhermitte-Duclos disease an d Cowden disease is probably underestimated. Cowden disease represents a new form of phakomatosis; Lhermitte-Duclos disease may occur as a s poradic disease, or as part of familial Cowden disease. The possibilit y of preneoplastic states in Cowden syndrome stresses the importance o f a thorough screening when Lhermitte-Duclos disease is diagnosed.