Acute unilateral inner retinal dysfunction with photophobia: importance of electrodiagnosis

Hirakata, Toshiaki; Fujinami, Kaoru; Saito, Wataru; Kanda, Atsuhiro; Hirakata, Akito; Ishida, Susumu; Murakami, Akira; Tsunoda, Kazushige; Miyake, Yoichi

doi:10.1007/s10384-020-00780-x

Cited by 2 publications

(12 citation statements)

References 29 publications

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“…AIR is characterized by an older age of onset. In unilateral cases, five males and three females, seven Japanese, and one Caucasian were reported by Hirakata et al [76], and two males and two females, four Japanese, were reported by Kido et al [77]. Patients' demographic of unilateral retinopathy is shown in Table 1.…”

Section: Patients' Demographicmentioning

confidence: 93%

“…AIR often affects the outer retinal layers; however, recently, a rare type of AIR has been reported, which damages the inner retinal layers is suspected. Moreover, Hirakata et al reported eight cases of unilateral retinopathy [76], Kido et al reported four cases [77], and Ueno et al reported three cases of bilateral retinopathy [78]. All these cases have been reported in Japan.…”

Section: Acute Inner Retinal Dysfunctionmentioning

confidence: 99%

“…Patients' demographic of unilateral retinopathy is shown in Table 1. Additionally, the mean age at the onset of symptoms was 62.2 ± 7.9 years (range 48-76), and three patients had a history of systemic autoimmune diseases, including asthma, palmoplantar pustulosis, and polymyalgia rheumatic [76,77].…”

Section: Patients' Demographicmentioning

confidence: 99%

“…The majority of the patients experienced sudden and severe photophobia in one eye [76,77], and some patients required dark glasses to walk under light-adapted conditions. Additionally, some patients developed visual field loss in the affected eye, which was especially severe under light-adapted conditions.…”

Section: Symptomsmentioning

confidence: 99%

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Retinal Dysfunction Caused by Autoimmune Mechanisms

Hirakata¹

2023

Medical and Surgical Retina - Recent Innovation, New Perspective, and Applications

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Autoimmune retinal disorders have been identified, including acute zonal occult outer retinopathy (AZOOR), AZOOR complex, autoimmune retinopathy (AIR) comprising paraneoplastic AIR (pAIR), cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and non-paraneoplastic AIR (npAIR). Patients with autoimmune retinal disorders typically present with sudden or acute onset of photopsia, photophobia, night blindness, rapid visual loss, and visual field abnormalities. The combination of multimodal imaging and electrophysiology is crucial because these diseases are challenging to diagnose. In particular, electroretinograms (ERGs) are essential for diagnosis. However, no treatment has been established to date. Additionally, a case of inner retinal dysfunction, thought to be a type of AIR, was recently reported. The diagnosis is difficult because most cases occur in one eye, and although the patient complains of severe photophobia, retinal imaging is almost normal, vision is preserved and there is almost no progression. The ERG is very characteristic, with cone-rod dysfunction and negative ERG. This chapter describes in detail the characteristics of AZOOR, AIR, and acute inner retinal dysfunction as new phenotypes of AIR.

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Section: Patients' Demographicmentioning

confidence: 93%

Section: Acute Inner Retinal Dysfunctionmentioning

confidence: 99%

Section: Patients' Demographicmentioning

confidence: 99%

Section: Symptomsmentioning

confidence: 99%

See 2 more Smart Citations

Retinal Dysfunction Caused by Autoimmune Mechanisms

Hirakata¹

2023

Medical and Surgical Retina - Recent Innovation, New Perspective, and Applications

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“…A review of cases with unilateral electronegative ERGs (that were not due to vascular occlusion or Birdshot chorioretinopathy) frequently found evidence of inflammatory changes in the affected eye, and the authors postulated an autoimmune aetiology [ 85 ]. Recently a clinical entity of acute unilateral inner retinal dysfunction has been described: the aetiology is unclear, and it is possible that inflammatory or autoimmune processes play a role [ 86 ].…”

Section: Acquired Causesmentioning

confidence: 99%

Negative electroretinograms: genetic and acquired causes, diagnostic approaches and physiological insights

Jiang

Mahroo

2021

Eye

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The dark-adapted human electroretinogram (ERG) response to a standard bright flash includes a negative-going a-wave followed by a positive-going b-wave that crosses the baseline. An electronegative waveform (or negative ERG) results when the b-wave is selectively reduced such that the ERG fails to cross the baseline following the a-wave. In the context of a normally sized a-wave, it indicates a site of retinal dysfunction occurring after phototransduction (commonly at the photoreceptor to bipolar cell synapse). This is an important finding. In genetic disease, the pattern of ERG abnormality can point to variants in a small group of genes (frequently those associated with congenital stationary night blindness and X-linked retinoschisis, but negative ERGs can also be seen in other conditions including syndromic disease). In acquired disease, there are numerous causes, but specific features may point to melanoma-associated retinopathy (MAR). In some cases, the visual symptoms precede the diagnosis of the melanoma and so the ERG findings can initiate investigations facilitating early detection and treatment. Negative ERGs can occur in other paraneoplastic conditions, and in a range of other diseases. This review will outline the physiological basis for the negative ERG, report prevalences in the literature from different cohorts, discuss the range of causes, displaying examples of a number of ERG phenotypes, highlight features of a clinical approach to patients, and briefly discuss further insights relating to current flows shaping the a-wave trough and from single-cell transcriptome analysis.

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Acute unilateral inner retinal dysfunction with photophobia: importance of electrodiagnosis

Cited by 2 publications

References 29 publications

Retinal Dysfunction Caused by Autoimmune Mechanisms

Retinal Dysfunction Caused by Autoimmune Mechanisms

Negative electroretinograms: genetic and acquired causes, diagnostic approaches and physiological insights

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