Abraham Kornzweig

Born in New York City, Kornzweig graduated from Columbia University in 1922, and from the New York University School of Medicine in 1925. From 1925 to 1928, He trained as an intern at Mount Sinai Hospital, New York, including study in pathology under the well-known Bernard Samuels, after which he entered private practice until 1934. He returned to Mount Sinai for a residency in ophthalmology and in 1936, received a diplomate. For 17 years, he taught at NYU-Bellevue Medical School, where he was an Associate Clinical Professor; he subsequently taught at Mount Sinai School of Medicine, where he was Clinical Professor, and eventually Emeritus Professor, of Ophthalmology. He was also Director of Research and Chief of Ophthalmology at the Jewish Home and Hospital for the Aged.[3]

He retired in poor health in 1972,[4] although he continued to work on his research projects until his death in 1982.[5]

He married Chifra Goldberg, a naturalized citizen, born in Yass, Rumania and they had one daughter.[6]

Bassen-Kornzweig disease, also called Bassen-Kornzweig Syndrome, is a rare congenital disorder in which the body fails to produce chylomicrons, a low density lipoprotein (LDL) and very low density lipoprotein (VLDL). Individuals with this condition are unable to properly digest fats. Symptoms include ataxia, peripheral neuropathy and other forms of nerve dysfunction. Treatment includes vitamin E.[7]

Characteristics of the syndrome include the presence of acanthocytes (burr-cell malformation of the erythrocytes), and the reduction or even absence of B-lipoproteins. Complications include retinitis pigmentosa,[8] degenerative changes in the central nervous system involving the cerebellum and long tracts, fatty diarrhea, ataxia, areflexia, demyelination, defective intestinal lipid absorption with low serum cholesterol level, intestinal malabsorption, amaurosis, retarded growth, and steatorrhea. Intellectual development may be slightly retarded.[9] Many afflicted with the syndrome are unable to walk.[10]

The syndrome appears in infancy. Affected children appear normal at birth but usually fail to thrive during their first year. The syndrome may predominate in males (71%). Most cases occur in children of Jewish descent, especially among Ashkenazi Jews. The disease is transmitted as an autosomal recessive trait.[9] It is also commonly recognized as a betalipoprotein deficiency[11] or abetalipoproteinemia.[10]

Kornzweig's publications include over 50 articles and books, beginning in 1948 with a series of articles on the "Eye in Old Age", and concluding with, in 1980, "New Ideas for the Old Eye."[3]

• Neuroacanthocytosis