Three cases of acute-onset bilateral photophobia

Ueno, Shinji; Inooka, Daiki; Meinert, Monika; Ito, Yasuki; Tsunoda, Kazushige; Fujinami, Kaoru; Iwata, Takeshi; Ohde, Hisao; Terasaki, Hiroko

doi:10.1007/s10384-018-00649-0

Cited by 3 publications

(4 citation statements)

References 31 publications

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“…Additionally, some patients developed visual field loss in the affected eye, which was especially severe under light-adapted conditions. Interestingly, photopsia was not observed in any of the patients, and bilateral cases also experienced acuteonset photophobia in both eyes [78].…”

Section: Symptomsmentioning

confidence: 87%

“…The full-field ERGs of the affected eyes were characterized as having severe cone-rod system dysfunction with negative ERGs in all patients [76][77][78]. The ERGs of the fellow eyes were either normal or mildly abnormal, and the rod ERGs (DA 0.01), cone, and 30 Hz flicker ERGs were severely reduced or undetectable.…”

Section: Electroretinogrammentioning

confidence: 98%

“…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%

“…The patient was elderly at the time of onset and was aware of the sudden photophobia at the time of onset [76][77][78]. No obvious genetic mutations have been revealed as far as we could examine [76].…”

Section: Pathophysiologymentioning

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: Symptomsmentioning

confidence: 87%

Section: Electroretinogrammentioning

confidence: 98%