Acute Macular Neuroretinopathy: A Review of the Literature

doi:10.1016/S0039-6257(02)00398-3

Survey of Ophthalmology

Volume 48, Issue 1, January–February 2003, Pages 1-11

https://doi.org/10.1016/S0039-6257(02)00398-3 Get rights and content

Abstract

Acute macular neuroretinopathy (AMNR) is a rare condition that produces transient or permanent visual impairment. Typical cases have acute onset multifocal scotomas that correspond rather precisely with reddish, flat, or depressed circumscribed lesions in the macula. These lesions are wedge-shaped and generally point toward the fovea. The pathophysiology of AMNR is unclear, the causes are uncertain, and there is no specific treatment for this condition. This review summarizes the presentation, possible risk factors, and prognosis of the 41 cases of AMNR reported in the published, English-language literature from 1975 through April 2002. Possible areas for future research into the etiology of this rare condition are discussed.

Section snippets

Definition, Clinical Diagnosis, and Natural History of AMNR

Acute macular neuroretinopathy (AMNR) is a rare condition characterized by the sudden onset of mild visual impairment from lesions that occur in the macula of the retina.⁴ It occurs unilaterally or bilaterally with normal to mildly decreased visual acuity.1, 4, 11, 19, 24, 26, 28, 30 Patients typically present several days or later after the development of multiple, paracentral scotomata in one or both eyes.4, 11, 24, 28

The clinical diagnosis is readily made on the basis of the distinctive

Summary of Reported Cases

AMNR is rare. Forty-one cases have been reported to date in the English-language medical literature from 1975 through April 2002. These cases are summarized in Table 1. AMNR is seen more frequently in women than men; of the 41 cases, 34 (83%) were in women.1, 4, 10, 11, 17, 19, 24, 26, 29, 30, 31 Women were in their reproductive years, with a mean age of 27 years (range 20–53 years).1, 4, 10, 11, 17, 19, 24, 26, 29, 30, 31 We were unable to identify any published reports of AMNR in children.

Summary

Acute macular neuroretinopathy is a rare condition that had been reported primarily among young women in their reproductive years. The symptoms usually include the onset of temporary or permanent vision loss associated with paracentral scotomas that occur either unilaterally or bilaterally. The hallmark lesions of this condition are dark-reddish brown and wedge- or oval-shaped. The lesions typically point toward the fovea and appear as teardrops or petals. Amsler grid or Goldmann perimetry

Method of Literature Search

This review included all published case reports of AMNR in the English-speaking literature from 1975 through April 2002. The following databases were searched: Pubmed and Medline. The keywords included acute macular neuroretinopathy, macula, etiology, and retinal diseases. Textbooks that included information on diseases of the macula were examined for relevant reports, and published reports identified through the reference lists of other articles were also included. Only non-English language

Acknowledgements

The authors reported no proprietary or commercial interest in any product mention or concept discussed in this article.

References (37)

D.M. Albert
Acute macular neuroretinopathy
Arch Ophthalmol
(1998)
P. Amin et al.
Acute macular neuroretinopathy
Arch Ophthalmol
(1998)
R. Booy
Amaurosis fugax in a young woman
Lancet
(1990)
P.J. Bos et al.
Acute macular neuroretinopathy
Am J Ophthalmol
(1975)
R. Davidoff
MigraineManifestations, Pathogenesis, and Management
(1995)
U.R. Desai et al.
Intravenous epinephrine and acute macular neuroretinopathy
Arch Ophthalmol
(1993)
L. DuBois et al.
Inner retinal layer loss in complicated migraine. Case report
Arch Ophthalmol
(1988)
B. Feigl et al.
Optical coherence tomography (OCT) in acute macular neuroretinopathy
Acta Ophthalmol Scand
(2000)
C.M. Fisher
Transient monocular blindness versus amaurosis fugax
Neurology
(1989)
A. Gandorfer et al.
Scanning laser ophthalmoscope findings in acute macular neuroretinopathy
Am J Ophthalmol
(2002)

J.D.M. Gass et al.

Acute macular neuroretinopathy and multiple evanescent white dot syndrome occurring in the same patients

Arch Ophthalmol

(1989)

J.D.M. Gass

Stereoscopic Atlas of Macular DiseaseDiagnosis and Treatment

(1977)

J.D.M. Gass

Stereoscopic Atlas of Macular DiseaseDiagnosis and Treatment

(1987)

J.D.M. Gass

Stereoscopic Atlas of Macular DiseaseDiagnosis and Treatment

(1997)

M. Gillies et al.

Traumatic retinopathy resembling acute macular neuroretinopathy

Aust NZ J Ophthalmol

(1997)

A.M. Glenn et al.

Complicated migraine resulting in blindness due to bilateral retinal infarction

Br J Ophthalmol

(1992)

M. Gomez-Torreiro et al.

Scanning laser ophthalmoscope findings in acute macular neruoretinopathy

Retina

(2001)

J. Goodwin et al.

Transient monocular visual lossamaurosis fugax versus migraine

Neurology

(1984)

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However, persistent visual field deficits have been reported in more than 50% of patients (Bhavsar et al., 2016). There is no specific treatment and no consensus regarding the discontinuation of risk factors such as hormonal contraceptives after AMN (Bhavsar et al., 2016; Fawzi et al., 2012; Turbeville et al., 2003). The nature of the primary insult in AMN remains uncertain and both ischemic and inflammatory disruption have been suggested.
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Citation Excerpt :
The exact etiology of AMN, however, remains elusive, albeit a wealth of evidence suggests a vasculopathic etiology.22-24 Specifically, Turbeville and associates hypothesized vascular damage as a common end point of vasoactive events including exposure to epinephrine, trauma, hypovolemia, systemic shock, hyperviscosity, contraceptive use, and occlusion of precapillary arterioles by immune complex deposition.4,25 OCTA in AMN has displayed flow deficits at the deepest level of the DCP, suggesting that capillary injury at the level of the collecting venules may be the primary etiology.10,16,26
To analyze imaging characteristics and the clinical course of patients demonstrating coincident lesions of paracentral acute middle maculopathy (PAMM) and acute macular neuroretinopathy (AMN) in the same eye.
Retrospective, observational case series.
Lesions from patients presenting with coincident PAMM and AMN in the same eye were evaluated with multimodal imaging including optical coherence tomography (OCT). The association with ocular and systemic findings was also investigated.
Fifteen subjects (17 eyes) were included in the study. The mean age was 44.4 ± 15.3 years and the follow-up period ranged from 1 to 32 weeks (mean, 11.9 ± 11.4 weeks). The mean visual acuity was 0.8 ± 0.6 logarithm of minimal angle of resolution (Snellen equivalent 20/126) at baseline and 0.3 ± 0.4 logarithm of minimal angle of resolution (Snellen equivalent 20/40) at the last follow-up. PAMM and AMN lesions occurred in the setting of Purtscher's retinopathy (4 eyes, 3 patients), retinal vein occlusion (7 eyes, 7 patients), central retinal artery occlusion (1 eye, 1 patient), and idiopathic retinal vasculitis (1 eye, 1 patient). In 4 eyes (3 patients), an association with other ocular disorders was not identified as evaluated with multimodal imaging. Of the total cohort, 11 eyes (64.7%) showed extension of the AMN hyperreflective bands in Henle's fiber layer with a Z-shaped morphology on OCT B-scan.
The presence of coincident PAMM and AMN suggests a common pathophysiologic etiology. This may be the result of retinal vein impairment and hypoperfusion at the level of the deep retinal capillary plexus possibly leading to injury to the Müller glia or photoreceptors in Henle's fiber layer.
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Citation Excerpt :
Affected patients classically present with acute paracentral scotomas, mild vision loss, and perifoveal reddish, wedge-shaped petalloid lesions on exam. Other risk factors commonly reported with AMN include viral prodrome, exposure to vasoconstrictive epinephrine or ephedrine, caffeine intake, and hypotensive shock.2,3 To date, there has been one other report in the literature describing AMN in the setting of acute lymphoblastic leukemia (ALL).4
To describe the first reported case of acute macular neuroretinopathy (AMN) associated with acute promyelocytic leukemia in a young Asian-Indian male.
We review the clinical and multimodal imaging findings in our patient that are characteristic of AMN.
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Major reviewAcute Macular Neuroretinopathy: A Review of the Literature

Abstract

Section snippets

Definition, Clinical Diagnosis, and Natural History of AMNR

Summary of Reported Cases

Summary

Method of Literature Search

Acknowledgements