Altered Functional Connectivity of the Primary Visual Cortex in Patients With Iridocyclitis and Assessment of Its Predictive Value Using Machine Learning.

Purpose: To explore the intrinsic functional connectivity (FC) alteration of the primary visual cortex (V1) between individuals with iridocyclitis and healthy controls (HCs) by the resting-state functional magnetic resonance imaging (fMRI) technique, and to investigate whether FC findings be used to differentiate patients with iridocyclitis from HCs. Methods: Twenty-six patients with iridocyclitis and twenty-eight well-matched HCs were recruited in our study and underwent resting-state fMRI examinations. The fMRI data were analyzed by Statistical Parametric Mapping (SPM12), Data Processing and Analysis for Brain Imaging (DPABI), and Resting State fMRI Data Analysis Toolkit (REST) software. Differences in FC signal values of the V1 between the individuals with iridocyclitis and HCs were compared using independent two-sample t-tests. Significant differences in FC between two groups were chosen as classification features for distinguishing individuals with iridocyclitis from HCs using a support vector machine (SVM) classifier that involved machine learning. Classifier performance was evaluated using permutation test analysis. Results: Compared with HCs, patients with iridocyclitis displayed significantly increased FC between the left V1 and left cerebellum crus1, left cerebellum 10, bilateral inferior temporal gyrus, right hippocampus, and left superior occipital gyrus. Moreover, patients with iridocyclitis displayed significantly lower FC between the left V1 and both the bilateral calcarine and bilateral postcentral gyrus. Patients with iridocyclitis also exhibited significantly higher FC values between the right V1 and left cerebellum crus1, bilateral thalamus, and left middle temporal gyrus; while they displayed significantly lower FC between the right V1 and both the bilateral calcarine and bilateral postcentral gyrus (voxel-level P<0.01, Gaussian random field correction, cluster-level P<0.05). Our results showed that 63.46% of the participants were correctly classified using the leave-one-out cross-validation technique with an SVM classifier based on the FC of the left V1; and 67.31% of the participants were correctly classified based on the FC of the right V1 (P<0.001, non-parametric permutation test). Conclusion: Patients with iridocyclitis displayed significantly disturbed FC between the V1 and various brain regions, including vision-related, somatosensory, and cognition-related regions. The FC variability could distinguish patients with iridocyclitis from HCs with substantial accuracy. These findings may aid in identifying the potential neurological mechanisms of impaired visual function in individuals with iridocyclitis.

Subfoveal Choroidal Thickness and Its Intereye Differences in Fuchs Uveitis Syndrome Evaluated Using Optical Coherent Tomography.

Purpose: To measure the subfoveal choroidal thickness (SFCT) and assess intereye subfoveal choroidal thickness difference (ISFCTD) in patients with unilateral Fuchs Uveitis Syndrome (FUS) compared with healthy controls.Methods: Forty-two patients with unilateral FUS were included in this observational retrospective study. SFCT in both eyes was measured in patients and controls using optical coherent tomography. The measurements were analyzed and compared as follows: for SFTC-affected eye vs fellow eye (FUS); affected eye (FUS) vs right control eye; fellow eye (FUS) vs left control eye; for ISFCTD - FUS patients vs controls. In addition, measurement error analysis was performed.Results: No significant differences in SFCT between the compared eyes were found (p > .05). The mean ISFCTD was 57.24 ± 40.8 µm in FUS patients and 30.33 ± 25.48 µm in controls (p < .,001).Conclusion: The ISFCTD was higher in FUS patients than in controls. There were no statistically significant differences in SFCT between the compared eyes.

Predictive Factors of Intraocular Pressure Level Evolution Over Time and Glaucoma Severity in Fuchs' Heterochromic Iridocyclitis.

Purpose: To investigate the clinical and virologic-associated and predictive factors of intraocular pressure (IOP) evolution over time and its severity in Fuchs' heterochromic iridocyclitis (FHC). Methods: Consecutive patients with both clinical FHC and intraocular synthesis of rubella virus (RV)-specific antibodies were included in this study. Specific ocular production of RV antibodies was confirmed using the quotient of serum/aqueous humor ratio of RV IgGs (Crv) and control antiviral IgGs (Cctl), using quantitative serology methods. Epidemiologic, clinical, biological, and virologic data at referral were collected and correlated with IOP values over time, occurrence, and severity of glaucoma. Results: Sixty-eight eyes of 68 patients were included. Mean age at diagnosis was 40.7 ± 11.1 years. Mean follow-up was 4.3 ± 4.3 years. Mean baseline Crv and Cctl values were 12.34 ± 14.67 and 216.70 ± 98.4, respectively. Mean baseline IOP was 17.2 ± 7.2 mm Hg (range, 9-40) and 15.6 ± 5.6 (range, 3-30) 5 years after referral. The predictive factors for pejorative IOP evolution over time and glaucoma severity were male sex (P = 0.03) and decreased Crv (P = 0.04) and presence of iris nodules (P < 0.001) and decreased Cctl (P = 0.02), respectively. Diagnostic delay was associated with increased likelihood of undergoing glaucoma surgery (P = 0.02). Conclusions: Time to diagnosis, male sex, presence of iris nodules at baseline, and decreased Crv and Cctl ratios were associated with increased likelihood of pejorative IOP evolution over time. Given the aggressiveness of glaucoma in FHC, these results provide interesting insight into what category of patients should need the closest screening.

The characteristic of intraocular pressure dynamic change in patients with glaucomatocyclitic crisis.

PURPOSE: To investigate the characteristic of intraocular pressure (IOP) dynamic change from episode to intermittent period in patients with Posner-Schlossman syndrome (PSS). PATIENTS AND METHODS: Seventeen cases of typical PSS were collected in this study. Both their random IOP in episodes/intermittent period and 24-h IOP in intermittent period were measured. The mean IOP as well as the peak and the valley value of 24-h IOP were calculated. Those data were statistically analyzed. RESULTS: The IOP in affected eye in episodes is higher than that of the contralateral eye statistically; while in intermittent period the mean IOP (p = 0.001), the peak (p = 0.029) and the valley (p = 0.004) value of 24-h IOP in affected eye are statistically different with that of the contralateral eye. All of these parameters of the affected eye in intermittent period are lower than that of the contralateral eye obviously. CONCLUSION: The dynamic observations of IOP in episodes and intermittent period confirmed the IOP crossover phenomenon inpatients with typical PSS and this observation may be important in the differential diagnosis of PSS.

Fuchs heterochromic iridocyclitis-associated glaucoma: a retrospective comparison of primary Ahmed glaucoma valve implantation and trabeculectomy with mitomycin C.

Background: The aim of this study was to compare the safety and efficacy of primary trabeculectomy with mitomycin C and Ahmed glaucoma valve (AGV) implantation in patients with Fuchs heterochromic iridocyclitis (FHIC)-related glaucoma, a rare complication of an uncommon form of uveitis. Methods : In this retrospective comparative case series, 26 FHIC-associated glaucoma patients received trabeculectomy (n=12) or an AGV (n=14). Primary outcome measures were surgical success, defined as intraocular pressure (IOP) ≤21 mmHg, decreasing ≥20% from baseline, and no secondary glaucoma surgery. Secondary outcome measures were the number of glaucoma medications, complications, best corrected visual acuity (BCVA), and IOP. Results: The follow-up was 34.0±17.7 months in patients that received trabeculectomy and 33.4±18.6 months in AGV (P= 0.837). The cumulative probability of success rate was 41.7% for trabeculectomy and 85.7% for AGV, with no significant difference in complications (P>0.05). The IOP in patients that received trabeculectomy dropped from 23.4±3.3 mmHg to 21.6±5.2 mmHg at the final visit (P= 0.041). In patients that received AGV, the IOP decreased from 24±7.8 to 17.1±2.6 mmHg (P= 0.003). The number of glaucoma medications at baseline were 3.3±0.5 in those that received trabeculectomy and 3±0.6 in those that received AGV (P=0.233), and decreased to 2.4±1.0 (P=0.008) and 1.7±0.6 (P=0.002), respectively. BCVA was equal in both groups and did not change (P>0.05). Conclusion: Primary AGV had a higher success rate than trabeculectomy, with patients also needing fewer medications for the management of FHIC-associated glaucoma.

Posner-Schlossman syndrome in Wenzhou, China: a retrospective review study.

AIM: To describe the incidence of Posner-Schlossman syndrome (PSS) in Lucheng District, Wenzhou, China, over a 10-year period. METHODS: We reviewed retrospectively the medical records of all inpatient and outpatient patients diagnosed with PSS during the years 2005-2014 in the Eye Hospital of Wenzhou Medical University. The keywords of 'glaucomatocyclitic crisis', 'Posner-Schlossman syndrome' and 'PSS' were used for the retrieval. Only patients with registered residing address in Lucheng District where the hospital located were finally selected. The cumulative incidence and annual incidence of PSS were calculated based on the sum of household registered population and temporary resident population in Lucheng District. RESULTS: A total of 576 patients with PSS (339 men and 237 women) met the retrieval criteria. The mean age of these subjects at the first clinic visit was 40±15 years. Intraocular pressure (IOP) of the initial record was 31.91±15.37 mm Hg. The 10-year cumulative incidence of PSS in Lucheng District was 39.53 per 100 000 population, whereas the mean annual incidence of PSS in this area was 3.91 per 100 000 population. The majority of these patients were aged 20-59 years (83.9%). Men showed a significantly higher cumulative incidence of PSS than women (p=0.010). Higher rate of newly onset cases was found in spring (31%) than in other seasons (p=0.006). CONCLUSIONS: Our results suggest a relatively high incidence of PSS in Wenzhou, a southeastern city in China. Young, male adults are prone to be affected in spring. However, the aetiology and other risk factors are still waited to be clarified.

Are There Any Changes in Corneal Biomechanics and Central Corneal Thickness in Fuchs' Uveitis?

PURPOSE: To compare corneal biomechanics, intraocular pressure (IOP) and central corneal thickness (CCT) of 38 patients with unilateral Fuchs' uveitis (FU) with 42 healthy controls. METHODS: Corneal hysteresis (CH), corneal resistance factor (CRF), Goldmann-correlated and corneal-compensated IOP (IOPg and IOPcc, respectively) and CCT were measured. RESULTS: The mean CH, CRF, and IOPg of the involved FU eyes were significantly lower (9.5 ± 1.6, 9.0 ± 1.9 and 13.1 ± 4.3 mmHg) than contralateral eyes (10.1 ± 1.7, 9.9 ± 1.7 and 14.6 ± 3.4 mmHg), and controls (10.5 ± 1.5, 10.3 ± 1.5 and 14.8 ± 2.5 mmHg), respectively. There was no significant difference for mean IOPcc between involved FU or contralateral eyes and controls (14.8 ± 4.1, 15.5 ± 3.4 and 15.0 ± 2.7 mmHg). The CCT values correlated with CH and CRF in the involved and contralateral eyes. CONCLUSIONS: Involved FU eyes had lower CH, CRF, and IOPg than contralateral eyes and controls, with no difference regarding IOPcc.

Comparison between Outcomes of Vitrectomy in Granulomatous and Nongranulomatous Uveitis.

PURPOSE: The aim of this study was to compare the outcomes of vitrectomy in granulomatous uveitis and nongranulomatous uveitis insufficiently managed by immunosuppressive therapy. METHODS: Thirty-eight eyes with granulomatous uveitis and 17 eyes with nongranulomatous uveitis that underwent vitrectomy for ocular complications between July 2006 and August 2012 were reviewed retrospectively. Visual acuity and ocular inflammation scores before and 6 months after surgery were compared. Patients treated with vitrectomy alone and those in whom vitrectomy was combined with phacoemulsification were analyzed separately. RESULTS: The mean visual acuity improved significantly both in granulomatous and nongranulomatous uveitis. In granulomatous uveitis, the mean inflammation scores decreased significantly both in the anterior segment and in the posterior segment. In nongranulomatous uveitis, the mean inflammation score in the posterior segment decreased significantly, although it did not change in the anterior segment. CONCLUSION: Vitrectomy was effective for treating ocular complications both in granulomatous uveitis and nongranulomatous uveitis, with favorable outcomes of improved visual acuity and decreased uveitis activity.

Optic neuritis associated with adalimumab in the treatment of uveitis.

BACKGROUND: Tumor necrosis factor (TNF) inhibitors such as adalimumab are increasingly used in the treatment of ocular inflammatory disease refractory to standard therapies. There is evidence that TNF inhibitors are associated with demyelinating neurologic events. OBJECTIVE: The authors report a case of optic neuritis and multiple sclerosis developing in association with the use of adalimumab for the treatment of bilateral chronic granulomatous iridocyclitis and multifocal choroiditis. They also review the available literature on cases of optic neuritis occurring in the setting of TNF inhibition. RESULTS: The authors identified 21 cases of optic neuritis associated with TNF inhibition; 36% of cases with available MRI results had evidence of other demyelinating lesions in the CNS. CONCLUSIONS: The strong association between TNF inhibitors and CNS demyelination may represent an adverse effect of these drugs. Ophthalmologists should recognize the relationship between TNF inhibitors and demyelinating disease, as it can present initially with visual symptoms.

Peripheral vascular endothelial dysfunction in glaucomatocyclitic crisis: a preliminary study.

PURPOSE: Glaucomatocyclitic crisis (GCC) is a syndrome of recurrent unilateral increased intraocular pressure associated with mild cyclitis and few clinical symptoms. This study was undertaken to assess brachial artery endothelium-dependent flow-mediated vasodilation (FMD) as an indicator of vascular endothelial function, to describe the association between GCC and endothelial dysfunction excluding age effects. METHODS: This prospective study was conducted from January 2007 to April 2009 and included 12 patients with GCC and 15 age-matched normal control subjects. Detailed clinical parameters were reviewed, including highly sensitive C-reactive protein (hs-CRP), homocysteine, anti-SSA, anti-cardiolipin antibodies, and HLA type. Brachial artery FMD and endothelium-independent, nitroglycerin-mediated vasodilation (NMD) were studied by using high-resolution, two-dimensional (2-D) ultrasonic imaging. RESULTS: Twelve patients with GCC were evaluated. The mean age of the patients was 36.3 years including 5 (41.6%) women and 7 (58.4%) men. There were no significant differences between patients with GCC and control subjects with regard to basal data, including body mass index, smoking, blood pressure, complete blood count, and routine blood biochemistries. Homocysteine and hs-CRP were within normal limits. Two (16.7%) patients were positive for HLA-B27, anti-SSA, and anti-cardiolipin antibodies. The nitroglycerin-mediated vasodilation in the patients with GCC was not significantly different from that of the control group. The FMD was much lower in the GCC group than in the control groups (mean 4.81% vs. 7.89%, P < 0.01). CONCLUSIONS: The significantly lower FMD in patients with GCC implies peripheral vascular endothelial dysfunction. However, in the 16.7% positive for the HLA-B27, anti-SSA, and anti-cardiolipin antibodies, these parameters are associated with GCC and abnormal FMD.

Aqueous flare and cells in Fuchs syndrome.

PURPOSE: To quantitatively evaluate aqueous flare and cells in patients with Fuchs syndrome. METHODS: The medical records of 40 patients (47 eyes) diagnosed with Fuchs syndrome between February 2006 and January 2007 at the Uveitis Study Center of Sun Yat-sen University were retrospectively reviewed. Aqueous flare and cells were clinically evaluated and quantified with laser flare-cell meter. Statistical analysis was performed to investigate the relationship between flare values and cell counts, and clinical parameters including patients' age, sex, duration of disease, best-corrected visual acuity, keratic precipitate, iris depigmentation, intraocular pressure, and posterior subcapsular lens opacities. RESULTS: Aqueous flare values (photon counts/ms) were significantly higher in Fuchs syndrome (9.40+/-5.85) than in normal controls (5.77+/-1.89, P=0.000). Aqueous cell counts (cells/0.5 mm(3)) were also significantly higher in Fuchs syndrome (5.09+/-4.84) than in normal controls (1.14+/-1.03, P=0.000). The flare values were positively correlated with the cell counts (r=0.331, P=0.001). Both flare values and cell counts were higher in eyes with keratic precipitates scored 2+ or 3+ as compared to those with a 1+ score. Higher flare values and cell counts were also observed in eyes with a 2+ or 3+ iris depigmentation score as compared to those with a 1+ score. No difference was found between flare values and cell counts and other parameters. CONCLUSION: Breakdown of blood-aqueous barriers and increased cell counts are present in the affected eyes in patients with Fuchs syndrome. These changes are positively associated with the degree of keratic precipitates and iris depigmentation.

Posner-Schlossman syndrome (glaucomatocyclitic crisis).

Posner-Schlossman syndrome (PSS) or glaucomatocyclitic crisis is a rare, typically unilateral recurrent inflammatory ocular hypertensive disease in which diagnosis can be challenging. An acute elevation of intraocular pressure is accompanied by or followed within a few days by a mild, often symptomless uveal inflammation. The mild nature of the uveitis at presentation of the first attack may go undetected. Medical treatment is indicated to prevent pressure-related optic nerve damage and to reduce inflammation. This report details a patient with Posner-Schlossman syndrome whose unilateral pressure elevation was initially treated as acute angle-closure glaucoma. He subsequently had several episodes of increased pressure over a two-year period. Diagnostic difficulties in this case are discussed.

Ampolla filtrante secundaria a cirugía de ciclodiálisis / Filtering bleb after surgical cyclodialysis

Caso clínico: Hombre de 35 años, miope magno, que recibe un balonazo en su ojo izquierdo. El traumatismo ocular le origina una ciclodiálisis y una hipotonía ocular con aparición de pliegues corioretinianos. Discusión: La hipotonía persistente no cedió al tratamiento tópico, ni a la fotocoagulación, por lo que se procedió al tratamiento quirúrgico mediante ciclopexia directa. En una de las ciclodiálisis se observó la existencia de vítreo, que consideramos pudiera ser la causa de su cierre incompleto. En el postoperatorio se comprobó el cierre completo de la ciclodiálisis, pero apareció una ampolla de filtración inesperada que pudo haber contribuido al buen control de la presión intraocular (AU)

Case report: A 35-year-old man with degenerative myopia suffered blunt ocular trauma due to being hit in the face by a ball. The injury caused four cyclodialysis clefts in his left eye which resulted in hypotony and chorioretinal folds. Discussion: The persisting hypotony was unsuccessfully treated with topical therapy and with laser photocoagulation. Therefore, a surgical treatment with direct cyclopexy was performed. In one of the cyclodialysis clefts, incarcerated vitreous was found, and this was considered to be the reason for its incomplete closure. In the postoperative period complete closure of the cyclodialysis cleft was observed; however an unexpected filtering bleb appeared, which could have affected good control of intraocular pressure (AU)

Chorioretinal lesions in nonfatal cases of West Nile virus infection.

OBJECTIVE: West Nile virus (WNV) disease is a zoonotic infection with recent outbreaks in the United States. Recent reports have highlighted the intraocular findings associated with WNV disease. We describe the intraocular findings observed in two patients infected by the West Nile virus. DESIGN: Observational case reports. METHODS: During an outbreak of WNV disease in Southwest Ohio, two patients with an acute onset of a systemic febrile illness accompanied by myalgia, arthralgia, headache, and a maculopapular rash were referred for blurred vision. Complete ophthalmologic examination, fundus photographs, and fluorescein angiograms were obtained on both patients. Both patients underwent serologic testing for viruses and cultures for bacteria, viruses, and fungi. RESULTS: Ophthalmologic examination in each patient revealed anterior segment and vitreous inflammatory cells and multiple partially atrophic and partially pigmented chorioretinal lesions clustered in the peripheral fundus. Fundus examination in case 2 also revealed mild disc edema in both eyes. Intracranial pressure as measured by lumbar puncture was borderline elevated. The chorioretinal lesions in both patients showed a striking similarity and appeared hypofluorescent centrally and hyperfluorescent around the edges on a fluorescein angiogram. Serologic testing for the WNV was positive in both patients, and tests for all other bacteria, fungi, and viruses were negative. CONCLUSIONS: WNV usually causes mild symptoms, but it occasionally causes neurologic illness with fatal outcome or severe morbidity. We present the cases of two patients with serology-proven WNV disease who developed chorioretinal lesions with a targetlike appearance and iridocyclitis.

Optic nerve head behavior in Posner-Schlossman syndrome.

PURPOSE: To analyze the effect of glaucomatocyclitic crisis on the optic nerve head, as well as its consequent structural and hemodynamic changes. To evaluate the surgical indication for Posner-Schlossman syndrome. MATERIALS AND METHODS: Four Posner-Schlossman syndrome cases were evaluated for an analysis of the phenomena occurring in the optic disc by means of retinal confocal tomography and Scanning Laser Doppler Flowmetry, and their correlation with visual function as measured with automated computerized perimetry. In all cases the images were obtained in the mediate period after the attack, while in case 1, measurements were performed in the immediate and mediate periods before, during and after the attack. RESULTS: Significant differences were found between optic nerve head parameters and retinal flow measurements in the different phases evaluated. No permanent optic nerve head damage was demonstrated in any of the cases studied, except for case 4 (Posner-Schlossman syndrome associated with traumatic glaucoma). All the variables analyzed returned to normal values after remission of the acute rise in intraocular pressure. CONCLUSIONS: During the attacks of ocular hypertension the optic nerve head experiences significant morphologic changes and hemodynamic variations; nevertheless since they are transient, they fail to cause permanent damage. There was only one case, in which there was optic disc damage and visual field loss. It can be concluded that surgery should be restricted to those cases with severe and disabling symptoms (relative surgical indication) or to cases with progressive optic neuropathy with visual field loss, when the syndrome is associated with glaucoma (absolute surgical indication).

[Analysis of the blood aqueous barrier by measurement of aqueous flare in 31 eyes with Fuchs' heterochromic uveitis with and without secondary open-angle glaucoma]. / Lasertyndallometrische Analyse der Blut-Kammerwasser-Schranke bei 31 Augen mit Heterochromiezyklitis Fuchs mit und ohne sekundäres Offenwinkelglaukom.

BACKGROUND: Fuchs' heterochromic uveitis is characterized by low-grade intraocular inflammation and a relatively benign clinical course. It was the aim of this study to quantitatively determine alterations of the blood-aqueous barrier in this disease by measuring the aqueous flare. METHODS: 31 affected eyes of 31 patients with the characteristic clinical picture of Fuchs' heterochromic uveitis, 31 apparently unaffected contralateral eyes, and 120 age and sex-matched normal control eyes were included in this retrospective study. Five of the eyes with Fuchs' heterochromic uveitis showed signs of secondary open-angle glaucoma. Determination of aqueous flare was performed in all eyes using the laser flare-cell meter FC-1000 (Kowa, Japan) after pupillary dilation. RESULTS: Aqueous flare values were moderately but significantly increased in eyes with Fuchs' heterochromic uveitis (mean 12.1 +/- 3.6 photon counts/ms) in comparison to normal control eyes (4.1 +/- 1.3 photon counts/ms, p < 0.001). Flare values of apparently unaffected contralateral eyes (mean 3.9 +/- 1.1 photon counts/ms) did not differ from normal control eyes (p = 0.5). In the group of eyes with Fuchs' heterochromic uveitis, aqueous flare was comparable in eyes with and without secondary open angle glaucoma (11.9 +/- 2.5 versus 12.1 +/- 3.8 photon counts/ms, p = 0.9). CONCLUSION: The alteration of the blood-aqueous barrier in patients with Fuchs' heterochromic uveitis is unilateral and relatively mild, corresponding to the well-known clinical picture of the disease. Secondary open-angle glaucoma appears not to be associated with additional increase of aqueous flare. The unilaterality and the relatively homogeneous distribution of the degree of increased aqueous flare values support the clinical impression that Fuchs' heterochromic uveitis is a distinct clinical entity that should be differentiated from other variants of chronic anterior uveitis.

[Periphlebitis retinae, uveitis and cystoid maculopathy in a patient with multiple sclerosis]. / Periphlebitis retinae, Uveitis sowie zystoide Makulopathie bei einem Patienten mit Multipler Sklerose.

PATIENT: A 44-year-old woman has a history of multiple sclerosis since 20 years. Besides recurrent numbness she had many instances of optic nerve neuritis which has led to a pronounced optic atrophy on her left eye. Additionally, she has developed recurrent iridocyclitis on her right eye. This was the reason why she was referred to our outpatient department for evaluation. Ophthalmic examination revealed an iridocyclitis associated with a multifocal retinal periphlebitis and severe cystoid macular edema in her right eye. CONCLUSION: Patients with multiple sclerosis develop in approximately 30% of cases retinal periphlebitis which may rarely be associated with anterior uveitis or cystoid macular edema as observed in our patient.

Unilateral pigmentary degeneration of the retina associated with heterochromia iridis.

BACKGROUND: For the past 5 years, a 56-year-old patient has been displaying monocular progressive pigmentary changes in the left eye. Heterochromy of the left eye has been known since childhood. The other eye is clinically and functionally normal. The patient was adopted and he has no children. Therefore, we have no family history. METHODS: The patient was examined clinically and by means of electroretinography, electrooculography, perimetry, computer tomography, pulsatile ocular blood flow (POBF) measurement, serology and Doppler sonography. RESULTS: Electrophysiology displayed a considerable reduction of scotopic and photopic ERGs, a reduced dark-through, and a reduced light-rise in the left eye, whereas the fellow eye was normal. The visual field was limited to 5 deg around the fixation point, and a peripheral crescent-shaped arch encircled the temporal-inferior quadrant concomitant to the pigmentary changes. By computer tomography and Doppler sonography a vascular affection was excluded. The left eye displayed lower POBF values. All serological tests were found negative. CONCLUSION: The clinical picture and negative exclusion criteria indicate a unilateral retinitis pigmentosa. However, with regard to the literature an unequivocal diagnosis can only be made upon hereditary evidence.