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A New Example of Ectomesodermal Dysgenesis

Catherine Haberland, MD; Maurice Perou, MD

Arch Neurol. 1970;22(2):144-155.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

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 . . . [Full Text PDF of this Article]

Author Affiliations
Chicago; Dixon, Ill

From the Department of Neuropathology, Illinois State Psychiatric Institute, Chicago (Dr. Haberland), and Dixon State School, Dixon, Ill (Dr. Perou).

Footnotes
Submitted for publication July 16, 1969; accepted Aug 27.

Reprint requests to the Department of Neuropathology, Illinois State Psychiatric Institute, 1601 W Taylor St, Chicago 60612 (Dr. Haberland).

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