PROGRESSIVE FACIAL HEMIATROPHY WITH MULTIPLE BENIGN-TUMORS AND HAMARTOMAS

We report the unusual association of Progressive Facial Hemiatrophy (P arry-Romberg syndrome) with multiple benign tumors (orbital neurinoma, mandibular odontogenous fibroma) and hamartomas. The neurological cli nical features were infantile hemiplegia, mild mental retardation and focal seizures. Brain CT-scan and MRI showed porencephaly and cerebral calcifications ipsilateral to hemifacial atrophy. Immunological inves tigations proved negative. The etiology of the disease and the bridgin g of this case to phakomatoses are discussed.