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Macula Halo Syndrome: Variant of Niemann-Pick Disease

Macula Halo Syndrome: Variant of Niemann-Pick Disease Abstract • The macula halo syndrome is the name proposed to describe patients with a unique ring-form opacity about the foveolas and a histiocytic storage disease. Since sphingomyelinase deficiency has now been found in the three patients in whom it was sought (including two in the present report), the entity may be classified as a variant of Niemann-Pick disease. A secondary hyperlipidemia may also be present. The macula halos consisting of symmetric crystalloid opacities with little or no visual impairment are pathognomonic of the entity. References 1. Cogan DG, Chu F, Barranger J, et al: Macula Halo Syndrome. Read in part before the American Ophthalmological Society, Hot Springs, Va, May 25, 1982. 2. Wewalka F: Zur Frage der Blauen Pigmentmakrophagen im Sternalpunktat . Wien Klin Wochenschr 1950;62:788-791. 3. Cogan DG, Federman DD: Retinal involvement with reticuloendotheliosis of unclassified type . Arch Ophthalmol 1964;71:489-491.Crossref 4. Sebestyn J, Galfi I: Retinal functions in Niemann-Pick lipidosis . Ophthalmologica 1969; 157:349-356.Crossref 5. Saidi P, Azizi SP, Sarlati R, et al: Rare variant of lipid storage disorders . Blood 1970; 35:533-538. 6. Harzer K, Ruprecht KW, Seuffer-Schulze D, et al: Morbus Niemann-Pick Typ B—enzymatisch gesichert—mit unerwarteter retinaler Beteiligung . Albrecht Von Graefes Arch Klin Exp Ophthalmol 1973;206:79-88.Crossref 7. Hartwig H, Kolling G, Best W: Kirschrot Fleck im Makulabereich ohne sicheren Hinweis auf Sphingolipidose . Klin Monatsbl Augenheilkd 1978;173:716-719. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Macula Halo Syndrome: Variant of Niemann-Pick Disease

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References (9)

Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1983.01040020700005
Publisher site
See Article on Publisher Site

Abstract

Abstract • The macula halo syndrome is the name proposed to describe patients with a unique ring-form opacity about the foveolas and a histiocytic storage disease. Since sphingomyelinase deficiency has now been found in the three patients in whom it was sought (including two in the present report), the entity may be classified as a variant of Niemann-Pick disease. A secondary hyperlipidemia may also be present. The macula halos consisting of symmetric crystalloid opacities with little or no visual impairment are pathognomonic of the entity. References 1. Cogan DG, Chu F, Barranger J, et al: Macula Halo Syndrome. Read in part before the American Ophthalmological Society, Hot Springs, Va, May 25, 1982. 2. Wewalka F: Zur Frage der Blauen Pigmentmakrophagen im Sternalpunktat . Wien Klin Wochenschr 1950;62:788-791. 3. Cogan DG, Federman DD: Retinal involvement with reticuloendotheliosis of unclassified type . Arch Ophthalmol 1964;71:489-491.Crossref 4. Sebestyn J, Galfi I: Retinal functions in Niemann-Pick lipidosis . Ophthalmologica 1969; 157:349-356.Crossref 5. Saidi P, Azizi SP, Sarlati R, et al: Rare variant of lipid storage disorders . Blood 1970; 35:533-538. 6. Harzer K, Ruprecht KW, Seuffer-Schulze D, et al: Morbus Niemann-Pick Typ B—enzymatisch gesichert—mit unerwarteter retinaler Beteiligung . Albrecht Von Graefes Arch Klin Exp Ophthalmol 1973;206:79-88.Crossref 7. Hartwig H, Kolling G, Best W: Kirschrot Fleck im Makulabereich ohne sicheren Hinweis auf Sphingolipidose . Klin Monatsbl Augenheilkd 1978;173:716-719.

Journal

Archives of Ophthalmology – American Medical Association

Published: Nov 1, 1983

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