Heredodegeneration of the Macula Lutea

McFarland, Corley B.

doi:10.1001/archopht.1955.00930010226009

Cited by 30 publications

(3 citation statements)

References 5 publications

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“…These observers agree that the degenerative process takes its origin where it is finally observed at its maximum, i. e. in the neuro-epithelium. This hypothesis is weakened by the observation by Vossius (1921), McFarland (1955) and Klien (1958) that visual acuity remains unaltered for a long time in spite of vast ophthalmoscopic alterations. My material includes 23 cases (54 eyes) with vision amounting to at least 5/10 in spite of central degenerations of at least one p. d.…”

Section: Discussion a N D Conclusionmentioning

confidence: 98%

“…As far as I have found, Hutchinson (1875), Harms (1904), Behr (1921), Klien (1950) andMcFarland (1955) are the sole contributors to the discussion of the formal pathogenesis of the hereditary macular degenerations. None of the published cases concerned persons younger than 60 years and the alterations were in their final stages.…”

Section: Discussion a N D Conclusionmentioning

confidence: 99%

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A Clinical Study of a Central Tapetoretinal Degeneration

Barkman

1961

Acta Ophthalmologica

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Iq 1956 a woman came to my office with the single complaint of a slightly fainting vision. She had central macular depigmentations and visual acuity 0. D. = 0,4; 0. S. = 0,8 (V: 49 in pedigree chart, Figure 1). On a direct question the patient told me that one or two of her four sons had poor vision. Further investigations revealed several cases of cent r d tapetoretinal alterations in residents of one parish, wAr, in the Swedish province of Dalecarlia. A causal relationship between the cases observed was sought and is tentatively reported here. MATERIAL AND METHODSTo secure patients for this study my original office patients were asked for information concerning their families. Additional cases were obtained through the files of the Ophthalmiatric Department of the Falu Hospital, where 10 cases had been observed by my predecessor. 59 cases were traced and observed by me at the ophthalmiatric outpatients department and followed up during half a year to four years. Ten of these patients were admitted for neurological and medical observation by me and my colleagues at the Medical Department.Relationship between patients with macular alterations was sought for by interviews and by means of objective genealogic methods, resorting to birth and parish registers. RESULTSA total of 69 persons were observed to have central retinal alterations, 39 men and 30 women. In 63 instances, 36 men and 27 women, the patients belonged to six frequently intermarrying kindreds from parish >>AN, they are represented in Figure 1, where the Roman numerals refer to generations, the Arabic to individuals. In the following report patients are denoted accordingly. 663

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Section: Discussion a N D Conclusionmentioning

confidence: 98%

Section: Discussion a N D Conclusionmentioning

confidence: 99%

A Clinical Study of a Central Tapetoretinal Degeneration

Barkman

1961

Acta Ophthalmologica

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“…[21][22][23][24][25] In the later stages, loss of RPE cells as well as photoreceptors occurs, and extracellular subretinal material can be found. [26][27][28] In the present disorder, biomicroscopic examination suggests that the serpentine-like gray deposits are located either within enlarged RPE cells or within the interphotoreceptor matrix between the photoreceptor layer and the RPE cells. A definite decision, however, is impossible without histologic evaluation.…”

Section: Commentmentioning

confidence: 99%

Cone-Rod Dystrophy With Serpentine-like Retinal Deposits

Kellner¹

1998

Arch Ophthalmol

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To describe the clinical and electrophysiologic findings in a novel retinal dystrophy. Methods: Ophthalmologic and electrophysiologic examinations were performed in 3 affected members of 1 family: a 10-year-old girl, her 30-year-old mother, and her 59-year-old maternal grandfather. Electrooculography (EOG) and electroretinography (ERG) were performed according to the standards of the International Society for Clinical Electrophysiology of Vision. Results: In all 3 family members, gray, serpentine-like deposits were seen at the level of the retinal pigment epithelium (RPE). These were most distinct in the child, less prominent in her mother, and barely visible in the grandfather. Visual acuity was 20/25 OU in the child and 20/ 200 OU in both adults. Visual field testing showed normal outer limits and small paracentral scotomas in both adults. Electroretinographic recording revealed slightly reduced amplitudes in the 10-year-old girl, cone dysfunction in her mother, and cone-rod dysfunction in the grandfather. Multifocal ERG responses were reduced but recordable in the mother. The EOG light peak amplitude was normal in both females and borderline in the grandfather. The light peak was delayed in all 3 patients. Conclusions: Similar deposits at the level of the RPE have not been described in other inherited retinal dystrophies. This family appears to have a novel form of conerod dystrophy with deposits at the level of the RPE and probable autosomal dominant inheritance.

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Diseases of the Retina and Optic Nerve

Wagener¹

1955

Archives of Ophthalmology

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Heredodegeneration of the Macula Lutea

Cited by 30 publications

References 5 publications

A Clinical Study of a Central Tapetoretinal Degeneration

A Clinical Study of a Central Tapetoretinal Degeneration

Cone-Rod Dystrophy With Serpentine-like Retinal Deposits

Diseases of the Retina and Optic Nerve

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