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February 1970

Encephalocraniocutaneous Lipomatosis: A New Example of Ectomesodermal Dysgenesis

Arch Neurol. 1970;22(2):144-155. doi:10.1001/archneur.1970.00480200050005

ACONGENITAL neurocutaneous disorder with the distinguishing histopathologic features of dysgenesis and neoplasia of the adipose tissue is presented in a 51/2-year-old epileptic, mentally retarded boy. To our knowledge, no such form of neurocutaneous lipomatosis has been reported. Thus the aim of this communication is threefold: (1) to give an account of the clinical and histopathologic characteristics of the disease; (2) to discuss it in relation to allied conditions; and (3) to entertain a possible pathogenetic mechanism.

Report of a Case 

History.—  A white boy, second in order of birth, was born on Aug 11, 1963. Hereditary and family history revealed no abnormalities. The mother's health during pregnancy was good, and the labor was uneventful. The birth weight was 2,779 gm (6 lb 2 ounces). He cried spontaneously. Abnormality of the right side of head, face, and right eye was noted at birth. Roentgenogram of the skull showed a slight