Retinitis Pigmentosa Without Pigment

Pearlman, Jerome T.; Flood, Stuart R.; Seiff, Stuart R.

doi:10.1016/0002-9394(76)90296-8

Cited by 29 publications

(7 citation statements)

References 4 publications

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“…These include pigmented paravenous retinochoroidal atrophy,5 helicoid parapapillary chorioretinal degeneration,6 sector retinitis pigmentosa,7 retinitis punctata albescens,8 and retinitis pigmentosa sine pigmento. 9 Morphological classification of the pigmented retinopathies is a convenient method for separating the various forms of RP clinically, and when a fundus pattern is always inherited in the same fashion it is strong evidence supporting a single disease process. However, subsequent research will have to demonstrate which morphological types consistently have the same aetiology, since difference aetiologies may give rise to the same clinical appearance.…”

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confidence: 99%

Preserved para-arteriole retinal pigment epithelium (PPRPE) in retinitis pigmentosa.

Heckenlively¹

1982

British Journal of Ophthalmology

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SUMMARY Five patients with retinitis pigmentosa (RP) with probable autosomal recessive inheritance have been identified in whom there is relative preservation of retinal pigment epithelium adjacent to and under retinal arterioles despite a panretinal degenerative process. All the patients were hypermetropic, though patients with RP tend to be myopic. This implies that there is a factor associated with retinal arterioles which locally retards the RP process in these patients. It may be appropriate to look for the PPRPE pattern in hypermetropic RP patients.

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confidence: 99%

Preserved para-arteriole retinal pigment epithelium (PPRPE) in retinitis pigmentosa.

Heckenlively¹

1982

British Journal of Ophthalmology

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“…This study aimed to increase the recognition and improve the clinical characterization of RP sine pigmento, a unique condition previously reported in only a limited number of case series or reports. 11,12,18,19 Diagnosing RP sine pigmento based solely on ophthalmoscopic findings is more difficult than the more typical cases of the disease involving BSP deposition. The changes in the fundus appearance in the absence of pigment deposition are remarkably subtle, and they may easily escape detection.…”

Section: Discussionmentioning

confidence: 99%

“…The absence or scarcity of typical BSP, known as "RP sine pigmento," has been described in several RP subtypes, although this finding may also be related to the fact that BSP is sometimes absent during the early stages of RP. 11 The clinical presentation, natural history, and detailed multimodal retinal imaging of RP sine pigmento have rarely been reported. In a recent study, BSP was reported to develop after a median time of 5.4 years in patients diagnosed with RP sine pigmento.…”

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confidence: 99%

Retinitis Pigmentosa Sine Pigmento

Lee

Joong

et al. 2022

Retina

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“…It is unusual to have this duration of symptomatic retinitis pigmentosa in the absence of significant retinal pigment (Pearlman et al, 1976). The association of myopia and choreoretinal degeneration with night blindness is well known and may occur in a sex linked recessive pattern of inheritance (Francois and De Rouck, 1965).…”

Section: Discussionmentioning

confidence: 99%