. 2013 Oct;8(4):341–350.

Table 2.

History of classification of Stargardt’s Disease

	Description of Stargardt's Disease				Criteria for diagnosis
Stargardt¹ 1909	1- A disease of probable autosomal recessive inheritance with age of onset in the 1^st or 2^nd decade. 2- Initial loss of vision before definite retinal changes are seen. 3- The eventual appearance of both an atrophic macular degeneration and prominent yellowish flecks in the macular and often posterior polar areas as well. The area of degeneration becomes larger with time and prominent flecks disappear. 4- Essentially normal peripheral visual fields and night vision throughout the patient's life. 5- A rather mild loss of color vision even with fairly severe visual loss.

Stargardt¹⁷ 1913	Conditions designated as Stargardt's disease
Stargardt¹⁷ 1913	Vitelliruptive macular degeneration	X-chromosome-linked juvenile retinoschisis	Cone degenerations	Fundus flavimaculatus	Age of onset (1^st and 22^nd decade) Absence of peripheral retinal changes

Franceschetti³ 1962	The same clinical picture reported by Stargardt (1909) is designated as Fundus flavimaculatus				Electroretinography dark adaptation

Krill and Deutman⁹ 1972	Stargardt's disease as a part of Fundus flavimaculatus				Eyeground changes Complete retinal function
	Group I	Group II		Group III
	Pure form without atrophy, central visual loss due to invasion of the fovea with one of the typical flecks	Atrophy, macular degeneration followed by flecks (frequent) or flecks preceding macular degeneration (rare). No loss of peripheral retinal function		(rare) Progressive deterioration of peripheral retinal function (4^th-5^th decade), Macular atrophy
		Subgroup A (frequent) No diffuse cone abnormality (clinical picture reported by Stargardt 1909)	Subgroup B (rare) Diffuse severe cone disease on the ERG such as described in cone degenerations

Fishman⁷ 1976	Stage I	Stage II	Stage III	Stage IV	The extent of fundus fleck-like lesions and their degree of resorption The extent of choroidal atrophy
Fishman⁷ 1976	Central RPE and possibly choriocapillaris disease, often associated with a discrete ring of perimacular flecks	Macular atrophy with flecks often extending to the equator. Atrophy of choriocapallaris and RPE within the macula.	Extensive fleck resorption within the posterior pole and marked RPE atrophy.	Diffusely resorbed flecks, extensive choriocapillaris atrophy

Gass¹⁸ 1987	Group I	Group II	Group III	Group IV	Macula appearance
Gass¹⁸ 1987	Vermillion fundi and hidden choroidal fluorescence	Atrophic maculopathy with or without flecks	Atrophic maculopathy with late signs and symptoms of retinitis pigmentosa	Flecks not associated with macular atrophy	Macula appearance

Noble and Carr¹³ 1979	Group I	Group II	Group III	Group IV	Macula appearance
Noble and Carr¹³ 1979	Macular degeneration without flecks	Macular degeneration with flecks	Macular degeneration with diffuse flecks	Diffuse flecks without macular degeneration	Macula appearance

Aaberg¹⁴ 1986	Stage I	Stage II	Stage III	Stage IV	The degree of damage to the retina
Aaberg¹⁴ 1986	Purely central macular degeneration with or without perifoveal flecks	Central macular degeneration and pericentral flecks extending outside the posterior fundus	Centroperipheral retinal pigmentary degeneration with an intact peripheral visual field but pigment migration, depigmentation and normal retinal vessel size	Centroperipheral retinal pigmentary degeneration with peripheral visual field defects and "bone trabeculae" pigmentation with attenuated retinal vessels	The degree of damage to the retina

Lois et al⁸ 2001	Group I	Group II		Group III	Electrophysiological attributes
Lois et al⁸ 2001	Normal rod and cone-mediated ERGs	Relative loss of generalized cone function		Both abnormal rod and cone ERGs	Electrophysiological attributes

The term “group” does not allow the progression of a patient from one category to another while the term ”stage” is defined as a period or distinct phase in the course of a disease or any biologic process.