Clinical Characteristics of Acute Zonal Occult Outer Retinopathy

PURPOSE: To investigate the clinical characteristics of patients with acute zonal occult outer retinopathy (AZOOR). METHODS: Medical records of 13 patients who visited Seoul National University Bundang Hospital from May, 2003 to May, 2015 and diagnosed with AZOOR were retrospectively reviewed. RESULTS: Thirteen patients (11 women and 2 men), with a mean age of 28.5 ± 11.4 years were followed for 42.8 ± 30.3 months. Visual field loss, photopsia, and blurred vision were common complaints. Initially, four patients had bilateral disease and seven patients showed bilateral involvement at the last visit. Mean best corrected visual acuity of involved eyes (BCVA) was 0.75 ± 0.32 (log MAR). Among 20 eyes with AZOOR, BCVA was 20/40 or better in 17 eyes (85.0%). The mean spherical equivalent was -4.59 ± 3.23 diopters (D), and 15 eyes (75.0%) had myopia less than -2.00 D. Nine eyes of seven patients (34.6%) had abnormal fundus findings. All patients underwent full field electroretinogram (ERG) or multifocal ERG and a visual field test. Thirteen patients (100.0%) showed a decreased response in ERG and visual field defects presented in every patient. With respect to the visual field test, 10 eyes (50.0%) showed improvement, 6 eyes (30.0%) had stationary status, and the progression of the visual field defect was observed in 4 eyes (20.0%). Among 13 patients, 4 (30.8%) patients showed flu-like symptom, 4 (30.8%) patients had fatigue, 2 (15.4%) patients had nausea, and 2 (15.4%) patients showed headache. CONCLUSIONS: AZOOR should be considered as one of the differential diagnoses, especially in female patients with myopia who show photopsia or visual field defects. ERG and visual field tests are necessary to confirm a decrease in retinal function and visual field loss. Central vision is preserved in most cases and recovery of visual field defect occurs often.

Current research of acute zonal occult outer retinopathy / 中华实验眼科杂志

Acute zonal occult outer retinopathy (AZOOR) is a relatively rare ocular disease that is characterized by the rapid loss of one or more zones of outer retinal function.AZOOR usually occurs in young women.AZOOR patients typically present with the sudden onset of photopsia and acute scotomas related to loss of sectors of outer retinal function.Early in the course of AZOOR,funduscopic appearance was often normal;however,most patients developed zones of retinal pigment epithelial atrophy or pigment clumping over time.Changes in electroretinogram (ERG) and persistent visual field defects were usually observed.Optical coherence tomography (OCT) showed that inner segment (IS)/outer segment (OS) regional abnormal changes corresponding to the visual field defect location area,at the same time the morphology and position of the visual field defect and multifocal electroretinogram (mfERG) shown in retinal amplitude density decreased regions coincide.The visual function of some patients can permanently damage.Fundus examination,mfERG and fluorescein angiography (FA),indocyanine green angiography (ICGA),autofluorescence (AF) and frequency domain OCT imaging techniques has an important significance for the diagnosis of AZOOR.Ever since the lack of understanding,AZOOR is often missed or misdiagnosed.In order to improve the understanding of the disease,this paper reviewed the definition,etiology,the epidemiological characteristics,clinical manifestation,classification,diagnosis,differential diagnosis,treatment and prognosis of AZOOR.

Acute Zonal Occult Outer Retinopathy, Responsive to an Immunosuppressive Agent: A Case Report

PURPOSE: To report a case of acute zonal occult outer retinopathy (AZOOR), initially misdiagnosed as retrobulbar optic neuritis, which was responsive to an immunosuppressive agent. CASE SUMMARY: A 28-year-old female with photopsia and a visual field defect in the left eye was referred to a hospital. There were no fundus abnormalities to explain her left visual field defect. Neurologic examination and brain MRI were normal. The patient was diagnosed with retrobulbar optic neuritis and treated with high-dose steroids. Four months after the onset of symptoms, she visited our hospital. Visual acuity was hand motion in the left eye. No relative afferent pupillary defect in the left eye and no anterior segment or fundus abnormalities were observed. A visual field examination of the right eye was normal and revealed field defect in the left eye. No abnormality was noted in the visual evoked potential test or fluorescein angiography. All examinations of the right eye were normal. In the left eye, fundus autofluorescence showed a hyperautofluorescent spot at the posterior pole, there was a decreased response in electroretinography and spectral domain optic coherence tomography showed that the junction between the photoreceptor inner and outer segments (IS/OS) was faintly visible only in the fovea. With the presumptive diagnosis of AZOOR, the patient was treated with an immunosuppressive agent. Visual acuity improved to 20/80 in the left eye at 10 months after the onset of symptoms. CONCLUSIONS: Electroretinogaphy is essential to diagnose AZOOR in patients with photopsia, visual field defect and minimal or no fundus changes, especially in a young women. There is currently no proven standard treatment, however immunosuppressive agents may be helpful.

Cefaleas de localización y compromiso oculares / Headches with ocular localization and involvement

SE REVISAN las cefaleas primarias más frecuentes que comprometen las estructuras oculares y vecinas al globo ocular, las variantes de la migraña y las alteraciones propias del nervio óptico que alteran la función visual y que se acompañan no solamente de síntomas dolorosos sino también de trastornos vasomotores debidos a alteraciones del sistema nervioso autónomo que suelen ser comunes en algunos procesos neurálgicos llamados ahora cefaleas con compromiso disautonómico. En este artículo se comentan los elementos epidemiológicos de las diferentes enfermedades craneales, faciales y oculares que cursan con cefalea; las diferencias clínicas entre las mismas y su presentación semiológica, al igual que algunos elementos terapéuticos. Se discuten además algunos elementos fisiopatológicos de gran utilidad en el análisis de los procesos dolorosos craneofaciales y el perfil temporal de los mismos; también se comentan algunas cefaleas secundarias que comparten con las primarias elementos que se prestan a confusión en la clínica. Finalmente, se hace un análisis de las neuralgias faciales comunes.

The most frequent primary headaches, including migraine variants, and intrinsic optic nerve disorders that produce headache, are reviewed. The latter are often accompanied by autonomic nervous system alterations which lead to vasomotor changes, frequently present in neuralgic processes known as headaches with disautonomic involvement. Epidemiological, semiological, clinical, and therapeutical aspects of different cranial, facial and ocular diseases that produce headache are included. Some physiopathological elements that may help to analyze painful craniofacial processes are discussed. Secondary headaches and common facial neuralgias, on the other hand, are also considered because they may confuse the differential diagnosis.

A Case Report of Acute Zonal Occult Outer Retinopathy

PURPOSE: We present a case of acute zonal occult outer retinopathy (AZOOR) that has not been reported in Korea. METHODS: A 26-year-old female with photopsia and visual field defect in the left eye was referred to our hospital. There were no fundus abnormalities to explain the visual field defect. Electroretinography, indocyanine green angiography, neurologic examination, cerebrospinal fluid tapping and brain magnetic resonance imaging were performed for further evaluation. RESULTS: Visual acuity was 20/20 in both eyes. Visual field examination revealed a peripheral scotoma in the right eye and marked constriction of visual field in the left eye. Electroretinography showed normal response in the right eye and no response in the left eye. There was an afferent pupillary defect in the left eye and vitreous cells in both eyes. Indocyanine green angiography, neurologic examination, cerebrospinal fluid tapping and brain magnetic resonance imaging showed no abnormal findings. CONCLUSIONS: The possibility of AZOOR should be considered in a patient with photopsia, visual field defect, acute loss of outer retinal function confirmed with electroretinography, and minimal or no fundus and angiographic changes, especially in a young woman.