Deficiency of beta-galactosidase causing abnormal accumulation of GM1 ganglioside in the liver, spleen, nerves, and renal glomerular epithelium. Symptoms present shortly after birth and include failure to thrive, retarded psychomotor development, gargoyle-like facies, and Hurler-like bone defects (dysostosis mutiplex). These symptoms lead to blindness, deafness, and quadriplegia with death in 1st two years of life from bronchopneumonia. Condition is autosomal recessive with defect mapped to short arm of chromosome 3.