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January 1950


Author Affiliations

From the Ophthalmic Department and Laboratories of the Beth Israel Hospital.

Arch Ophthalmol. 1950;43(1):43-65. doi:10.1001/archopht.1950.00910010048005

HISTORICAL DATA AND CLINICAL APPEARANCE  ANGIOMATOSIS of the retina was considered a disease affecting the retina solely, but even in the early period a possible connection with lesions of the central nervous system was suspected. It is to the merit of Lindau1 to have recognized that angiomatosis of the central nervous system, characterized by the occurrence of single or multiple hemangioblastoma below the tentorium—in the cerebellum, the brain stem or the spinal cord—where the picture resembles that of syringomyelia, forms a separate entity and that angiomatosis of the retina is a part of the syndrome. The concept of Lindau threw an entirely new light on the subject, and Schuback2 was the first to refer to angiomatosis of the central nervous system as Lindau's disease, an eponym almost universally accepted. Some authors, especially in the ophthalmologic literature, prefer the designation of von Hippel's disease, or von Hippel-Lindau disease, or

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