Frank W. Newell

A New Mucolipidosis with Psychomotor Retardation, Corneal Clouding, and Retinal Degeneration

A man now 22 years of age had slow psychomotor development about 6 months after birth and developed intermittent corneal clouding at about 18 months. He developed truncal ataxia, hypotonia of the limbs combined with spasticity, and active deep reflexes. These have not progressed. His skeleton and facies are normal. Between his first and thriteenth year he developed sev ere optic atrophy, absence of retinal blood vessels, and an extinguished electroretinogram. Biochemical analysis of cultured fibroblasts indicated no lysosomal hydrolase deficiency; cellular metachromasia was absent and there was no mucopolysaccharidoses. Ultrastructural studies indicated single membrane vacuoles containing lamellated membranes and a polymorphous substance in tissue cultured cells and conjunctiva.

Pigmentary Degeneration of the Retina in the Hallervorden-Spatz Syndrome

Dizygotic twins developed a progressive neurologic disorder at age 6 months. When examined at age 7 1/2 years each had spastic quadriparesis and dystonia. Neither had ever spoken a complete sentence. The fundi showed bone spicule formation, a conspicuous choroidal circulation, and a striking accumulation of yellowish-white globular masses of varying sizes and shapes. Because our patients developed both the pigmentary degeneration and clinical signs of Hallervorden-Spatz syndrome at a much younger age than patients without retinopathy, we believe this case demonstrated a distinct nosologic entity.

Autosomal Recessive Incomplete Achromatopsia with Deutan Luminosity

Four patients in three different families had a form of autosomal recessive incomplete achromatopsia not previously described. The visual acuity was 6/18 to 6/60 (20/60 to 20/200) with minimal ophthalmoscopic abnormality and normal fluorescein angiogram. The photopic electroretinographic responses were present in all four patients; the fusion rate of 60 Hz was only slightly subnormal. The high-intensity scotopic response was subnormal. The patients failed color screening plates and accumulated over 400 errors with scotopic axis on the Farnsworth-Munsell 100-hue test. The Rayleigh match was abnormal, displaced toward the red primary, but with normal luminance. The photopic luminous efficiency function was similar to that of the deuteranope. Color matching revealed a trichromatic form of color vision mediated by long wavelength and short wavelength cones, and a rhodopsin receptor.

Autosomal Recessive Incomplete Achromatopsia with Protan Luminosity Function

A unique form of dichromatic color vision is described in a family with incomplete achromatopsia. In 1966, incomplete achromatopsia was diagnosed in 4 of 14 children of a consanguineous marriage. The 4 affected had best visual acuities of 6/60 or 6/180, pendular nystagmus, and aversion to bright lights. The ERG showed minimal photopic responses. No abnormality of rod function was present. There was a severe color vision defect. In 1976, one of the patients returned for further color testing. Color tests included measurement of the luminous efficiency function using heterochromatic flicker photometry and colorimetric evaluation. The luminous efficiency function resembled that of the protanope. From the colorimetric measurements, we conclude that the patient has a unique form of dichromatic color vision mediated by two visual photopigments: the normal MWS cone photopigment and a photopigment with the spectral characteristics of rhodopsin.

Nabilone: A Pressure-Reducing Synthetic Benzopyran in Open-Angle Glaucoma

Nabilone is a synthesized crystalline benzopyran that resembles the cannabinols but is not a tetrahydrocannabinol. Oral administration of 0.5 to 2 mg to patients with open-angle glaucoma reduced the intraocular pressure from 10% to 54% with an average of 28%. Administration of a topical ophthalmic nabilone solution (0.1 mg/drop) to both eyes of adult albino rabbits (1.5 to 3.5 kg) lowered the IOP an average of 25%. The peak action of the nabilone administered in this manner was 60 minutes, with a return to normal IOP by 180 minutes. Tolerance developed in rabbits after one week of topical administration.

HISTOCHEMISTRY OF THE RETINA IN THE ALLOXAN-DIABETIC RAT

THERE have been relatively few studies of the carbohydrate metabolism of the retina of the alloxan-diabetic animal and not all have been in agreement. Illing and Gray (1951) described an impairment of glucose utilization in the retina of diabetic rabbits, but these results were not confirmed by Kornblueth, Yardeni-Yaron, and Wertheimer (1953). DeRoetth and Pei (1960), using manometric techniques, found decreased anaerobic glycolysis and increased succinic dehydrogenase activity in the retina of alloxan-diabetic rats compared with normal controls. Heath, Rutter and Beck (1962) demonstrated an increase in the nicotinamide-adenine nucleotide content of the alloxan-diabetic rat and a decrease in the reduced form of this nucleotide. Kurimoto and Newell (1963), using histochemical techniques, have shown an increased phosphorylase activity in the alloxan-diabetic rat retina. Additionally, retinal and renal glycogen which was not mobilized by starvation as was liver glycogen was demonstrated. It was the purpose of this study to investigate histochemically some of the pathways of glucose metabolism in the normal and the alloxan-diabetic rat. The metabolic pathways of the retina may be studied by means of enzymatic, micro-enzymatic, or histochemical techniques. The usual enzymatic techniques involve the incubation of large segments of the retina with appropriate substrates and measurement of the amount of oxygen used or metabolic products formed. The micro-enzymatic method, as developed by Lowry, Roberts, and Lewis (1956) and Lowry, Roberts, Schulz, Clow, and Clark (1961) provides the most accurate quantitative estimate of enzyme activity in the retina, but permits study solely of one individual layer at a time. Histochemical techniques provide a visualization of a cross-section of the distribution of an enzyme system. These latter techniques, however, depend upon interaction of more than one enzyme and during incubation enzymes may diffuse into adjacent retinal layers so that specific localization enzyme may not be accurate.

The American Journal of Ophthalmology 1862–1864

TheAmerican Journal of Ophthalmology was the first specialty periodical to be published in the Western hemisphere; the first issue appeared in New York City July 1862. Its editor and publisher was Julius Homberger, M.D., aged 22 years, who had emmigrated from Germany in January 1861. Six issues were published the first year andThe Journal ceased publication after two issues in 1864. Possibly, the American Ophthalmological Society, the first national medical specialty society, was founded in 1864 in a reaction to Homberger, his journal, and his strong belief that specialists, but not other practitioners, should be permitted to advertise their skills. In 1866, Homberger submitted his resignation to the American Medical Association, which he had served a secretary of the Section on Surgery, 1864–1865. His resignation was refused and he was expelled from membership in 1868. He moved to New Orleans to practice ophthalmology in 1867, and died in 1872. The second series ofThe Journal began in St. Louis in 1884 with Adolf Alt, A graduate of Heidelberg University, who trained in ophthalmology in New York City, with Hermann Knapp, founder, editor, and publisher of theArchives of Ophthalmology. In 1918, the current third series of theAmerican Journal of Ophthalmology, consolidated five ophthalmic publications, with Edward Jackson of Denver as editor.

Edward Jackson, MD--a historical perspective of his contributions to refraction and to ophthalmology.

Edward Jackson died October 29, 1942, at 86 years of age. He served as president of the major national ophthalmologic organizations and was professor and chairman of the Department of Ophthalmology at the University of Colorado. He established the first graduate course for ophthalmologists, suggested the formation of the instruction courses of the American Academy of Ophthalmology, and was the principal founder of the American Board of Ophthalmology. He founded and edited the Yearbook of Ophthalmology and Ophthalmic Literature. In 1918, he became editor of the third series of the American Journal of Ophthalmology, which consolidated five ophthalmic periodicals. In 1885, he popularized retinoscopy in the United States and was mainly responsible for making it a practical refraction tool. Two years later, he described the cross cylinder to determine the presence or absence of astigmatism. In 1907, he described the use of the cross cylinder to refine the axis of a correcting cylinder in astigmatism. He lectured and wrote widely and published over 700 scientific articles, book chapters, and books.

The origins of the National Eye Institute 1933–1968

Many organizations and individuals prompted the authorization of the National Eye Institute by the United States Congress. The United States Marine Hospital and Public Health Service established a Laboratory of Hygiene in 1887, which became the research center of the National Institute of Health in 1930. The Albert D. and Mary Lasker Medical Foundation was mainly responsible for the 1945 conversion of the American Society for the Control of Cancer to the American Cancer Society, dedicated to medical research. Mildred Weisenfeld, a patient with retinitis pigmentosa, founded The Fight for Sight! in 1946 to provide funds for eye research. The Laskers invited Miss Weisenfeld to testify in support of a National Institute of Neurology, and her appeal was so persuasive that it emerged as the National Institute of Neurological Diseases and Blindness. Congressman John Fogarty and Senator Lister Hill directed the attention of the Congress and the public to the need for the Federal support of medical research. In 1960 Jules Stein, the legendary founder of the Music Corporation of America (MCA), established a new philanthropy, Research to Prevent Blindness, which provided skilled leadership in detailing the need for research in blinding disease and obtaining Congressional and Presidential approval of a new institute. The Committee for Research in Ophthalmology and Blindness was instrumental in bringing the groups interested in the welfare of the blind into harmony with groups concerned with medical research in blinding disease. The Association of University Professors of Ophthalmology was the first medical society to support the need for a National Eye Institute. The National Eye Institute was formally approved 8 August 1968 and celebrated its 25th anniversary in 1993.

The origins of the National Eye Institute 1933-1968. The Fifth Charles B. Snyder Lecture.

Many organizations and individuals prompted the authorization of the National Eye Institute by the United States Congress. The United States Marine Hospital and Public Health Service established a Laboratory of Hygiene in 1887, which became the research center of the National Institute of Health in 1930. The Albert D. and Mary Lasker Medical Foundation was mainly responsible for the 1945 conversion of the American Society for the Control of Cancer to the American Cancer Society, dedicated to medical research. Mildred Weisenfeld, a patient with retinitis pigmentosa, founded The Fight for Sight! in 1946 to provide funds for eye research. The Laskers invited Miss Weisenfeld to testify in support of a National Institute of Neurology, and her appeal was so persuasive that it emerged as the National Institute of Neurological Diseases and Blindness. Congressman John Fogarty and Senator Lister Hill directed the attention of the Congress and the public to the need for the Federal support of medical research. In 1960 Jules Stein, the legendary founder of the Music Corporation of America (MCA), established a new philanthropy, Research to Prevent Blindness, which provided skilled leadership in detailing the need for research in blinding disease and obtaining Congressional and Presidential approval of a new institute. The Committee for Research in Ophthalmology and Blindness was instrumental in bringing the groups interested in the welfare of the blind into harmony with groups concerned with medical research in blinding disease. The Association of University Professors of Ophthalmology was the first medical society to support the need for a National Eye Institute. The National Eye Institute was formally approved 8 August 1968 and celebrated its 25th anniversary in 1993.