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Neu-Laxova Syndrome: Report of Two Cases
Authors:Gershon G. Ejeckam   Jasbir K. Wadhwa  John P. Williams  Atilano G. Lacson
Affiliation: a Department of Pathology, Memorial University of Newfoundland, St. John's, Newfoundland, Canada
Abstract:
Two cases of Neu-Laxova syndrome are reported. The pathological features are described. There is marked swelling of the palms and soles, which is due to massive fat and myxomatous connective tissue proliferation in addition to edema. Lissencephaly with hypoplasia of cortical descending fibers is observed, along with cerebellar hypoplasia. The occurrence of 2 micro cephalic male and female infants dying shortly after birth in the same sibship with 1 normal baby in between confirms similar findings by others and supports an autosomal-recessive inheritance. The relevance of pathological examination in further delineation of the phenotypic appearance is discussed.
Keywords:Neu-Laxova syndrome  neurocctodermal dysplasia  intrauterine growth retardation  ichthyosis
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