NEUROVISCERAL STORAGE DISEASE

1 May 1968

journal article
Published by American Roentgen Ray Society in American Journal of Roentgenology

Vol. 103 (1) , 149-153
https://doi.org/10.2214/ajr.103.1.149

Abstract

Neurovisceral storage disease is inherited as an autosomal recessive trait.The patients have clinical and roentgenographic features resembling Hurler's disease. There is severe progressive cerebral degeneration with death occurring in the first two years of life. Increased accumulation of ganglioside in the brain has been previously demonstrated.Two patients are reported who show, in addition, the increased synthesis and storage of mucopolysaccharide in skin fibroblast cultures.

Keywords

NEUROVISCERAL STORAGE
STORAGE DISEASE
SYNTHESIS
INHERITED
LIFE
RECESSIVE TRAIT.THE
TRAIT.THE PATIENTS
AUTOSOMAL

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