[Choroidal neovascularization followed in a patient with "Multiple Evanescent White Dot Syndrome" (MEWDS) -- a case report]. / Chorioidale Neovaskularisationsmembran bei einer Patientin mit "Multiple evanescent white dot Syndrome" -- Eine Kasuistik.

BACKGROUND: Since the description of the "multiple evanescent white dot syndrome" (MEWDS) by Jampol et al, choroiditis has been in the focus of interest. But the classical type of MEWDS was an exceptional case in clinical routine. CASE REPORT: A 48-year-old female presented to our hospital with a sudden unilateral visual acuity decrease and an extension of the blind spot. Ophthalmoscopy and fluorescein angiography revealed typical multiple grey-white chorioretinal patches of the same stage with lesion areas of about 100 - 200 microm compatible with the diagnose of MEWDS. Although visual acuity increased continuously the patient developed a classical choroidal neovascularization within 4 weeks. She was treated with PDT and visual acuity as well as the ophthalmoscopic diagnosis remained stable. CONCLUSION: In spite of visual improvement in MEWDS, regular control is recommended. In addition we propose to consider the diagnosis of MEWDS if an enlargement of the blind spot and CNV without lesions of the retinal pigment epithelium are diagnosed.

Melanoma-associated retinopathy: high frequency of subclinical findings in patients with melanoma.

BACKGROUND: Melanoma-associated retinopathy (MAR) is a paraneoplastic syndrome with symptoms of night blindness, light sensations, visual loss, defect in visual fields, and reduced b-waves in the electroretinogram. Patients with MAR often suffer from a sudden onset of ocular symptoms that are believed to result from antibody production against melanoma-associated antigens that cross-react with corresponding epitopes on retinal depolarizing bipolar cells. OBJECTIVES: To correlate the frequency of subclinical symptoms suggestive of MAR in melanoma patients to different stages of disease, patient age, type and thickness of the primary tumour, form of therapy, S-100 level and tumour burden. METHODS: We analysed 28 patients with melanoma in stages I-IV (according to the American Joint Committee on Cancer tumour classification) for the presence of subclinical MAR symptoms using scotopic electroretinography, static and kinetic perimetry and nyctometry. RESULTS: Seven patients had clinical signs and symptoms consistent with MAR, 18 had some indications, while the remaining three had none. We found no correlation between clinical symptoms and stage of disease, tumour burden or S-100 level, but findings suggestive of MAR were observed more frequently in advanced stages of disease. CONCLUSIONS: Subclinical retinal involvement characteristic of MAR appears to be more common than previously suspected in patients with cutaneous malignant melanoma. Our findings in this small cohort seem to indicate that the percentage of patients with symptoms suggestive of MAR is higher in advanced stages of disease. Further clinical studies are required to evaluate if the presence of subclinical symptoms suggestive of MAR is correlated with a worse prognosis and a shortened progression-free and overall survival.

Multifocal electroretinogram with a multiflash stimulation technique in open-angle glaucoma.

PURPOSE: To analyze a multiflash multifocal electroretinogram in 20 patients with open-angle glaucoma (OAG). METHODS: The stimulation sequence consisted of a binary m-sequence (L(max) 200 cd/m(2), L(min) <1 cd/m(2)). Each m-sequence stimulus was followed by three global flashes (luminance: 400 cd/m(2)) at an interval of 26 ms. RESULTS: The presence of a response to the three global flashes indicated an adaptive effect of the response to the preceding m-sequence stimulus. In the nasal retinal response average the relative amplitude contribution of the response to the second global flash in relation to the other two global flash responses was outside the range of normal (10th-90th percentile) in 10 of 20 OAG patients. CONCLUSIONS: The changes in the relative contribution of the response to the second global flash seem indicative of impaired adaptive effects presumably due to inner retinal damage.

Comparison of different high resolution multifocal electroretinogram recordings in patients with age-related maculopathy.

AIM: to compare two different resolutions of multifocal electroretinogram recordings (MF-ERG) in patients with age-related macular degeneration (AMD). PATIENTS AND METHODS: in 20 eyes (14 patients) with early or late stages of AMD, MF-ERGs of 8 min duration were recorded using a low and a high resolution. The central 50 degrees of the retina were stimulated by 103 as well as by 241 stimulus elements. Results were compared with one another and with an age-matched control group. RESULTS: when response averages of equal eccentricity were analysed, amplitudes of the MF-ERG differed significantly from normal within the central 40 degrees. This held true for both low (103) and high (241) resolutions. Sixteen of 20 eyes also showed focal retinal dysfunction at both resolutions. In one case, the high resolution MF-ERGs could detect functional deficits that could not be seen with the lower resolution. However, due to a reduced signal-to-noise ratio three high resolution recordings could not be analysed. CONCLUSION: high resolution MF-ERG seems more sensitive than low resolution MF-ERG. However, the low resolution (103) MF-ERG is recommended for routine application in the clinic because of its better signal-to-noise ratio.

[A differentiated study of the retinal function in segmental retinitis pigmentosa by multifocal electroretinograms]. / Differenzierte Untersuchung der Netzhautfunktion bei regionaler Retinitis pigmentosa mit Hilfe des multifokalen Elektroretinogramms.

PURPOSE: Generalized retinal degenerations such as retinitis pigmentosa may manifest with focal retinal dysfunctions. These may be detected objectively by new electrophysiological techniques, such as multifocal electroretinography (ERG). CASE REPORT: A mother and daughter, aged 81 and 46 years, showed bilateral caudal bone spiculae formations with corresponding cranial visual field defects in the static perimetry of the central visual field (Octopus) and in the kinetic perimetry (Goldmann). RESULTS: Pattern VEP, pattern ERG, EOG, and cone ERG were within the normal range. The scotopic ERG was in the lower normal range. The multifocal cone ERG of the central 50 degrees showed reduced amplitudes and prolonged latencies in the first-order response component. These findings corresponded to the area of the bone spiculae and the scotomata. CONCLUSION: Multifocal ERG enables the detection of focal retinal cone dysfunction in segmental retinitis pigmentosa. It is an additional tool that may aid in the diagnosis and classification of this disease.

The multifocal ERG in open angle glaucoma--a comparison of high and low contrast recordings in high- and low-tension open angle glaucoma.

High and low contrast multifocal ERG (MF-ERG) recordings were obtained from the right eyes of 24 patients with OAG (high-tension OAG: n=16, low-tension OAG: n=8) and compaired to those recorded from 18 healthy volunteers. High contrast MF-ERG recordings were obtained at a mean luminance of 100 cd/m2 with a contrast of 99%, while low contrast MF-ERGs were obtained at a mean luminance of 100 cd/m2 with a contrast of 50%. During MF-ERG recordings the central 50 degrees of the retina were stimulated by 103 hexagons. A MF-ERG recording lasted eight minutes, a M-sequence of 2(15) was used. The first order response component (KI, mean focal flash response) and the first and second slice of the second order response component (mean focal two flash interaction of flashes one, KII. 1, or two, KII.2, base intervals apart) were analyzed for group differences. Group differences were found mainly in latency measures. These included a delay in the central response average of the first positive peak, P1, in KII.2 (p < or = 0.05) in OAG high contrast recordings. Low contrast recordings showed a significant delay in the central response average of the first negative peak, Nl, in KII.2 as well as in the peripheral response average of N1 in KI and of P1 in KII.2 (p<0.05) in OAG. Amplitudes were only affected significantly in KI of the low contrast recordings. Here the amplitude N1P1 was significantly higher in high tension (n=16) than in low tension (n=8) OAG patients. However, an overlap in all of the response parameters tested allowed only group differences to be characterized. Under these stimulus conditions, neither high contrast recordings nor low contrast recordings seem sensitive enough to reliably recognize early glaucomatous retinal dysfunction.

The effect of time of day and repeat reliability on the fast flicker multifocal ERG.

PURPOSE: To investigate the possible influence of different times of the day on multifocal ERG recordings in normals. METHODS: 10 healthy volunteers underwent multifocal ERG recordings (VERIS 3.1.1.) 3 times a day, at 10:30 am, 1:30 pm and 4:30 pm using a fast flicker stimulus of 103 hexagons. Additional night-time recordings were performed in 2 subjects. The first-order and dominant part of the second order response component were analyzed. RESULTS: Neither peak latencies nor peak-to-peak amplitudes showed significant changes during the day (p >0.05). Comparison between night and day time recordings did also not reveal an influence of a circadian rhythm on the MF-ERG. CONCLUSIONS: No significant influence of the time of the day suggestive of a circadian rhythm could be observed. Light adaptation due to preadaptation and the flicker sequence used could be a possible cause for these results.

Das multifokale elektroretinogramm in der diagnostik und verlaufskontrolle lokalisierter Netzhautfunktionsstörungen: fallbericht eines patienten mit chorioretinopathia centralis serosa.

The role of multifocal electroretinography (MF-ERG) in the diagnosis and follow-up of localized areas of retinal dysfunction is discussed. A 42-year-old male with the preliminary diagnosis of optic neuritis in his left eye was referred for evaluation with the MF-ERG. Simultaneous cone ERGs were obtained from 103 locations within the central 50 degrees of the retina. During an 8-month follow-up four MF-ERGs were obtained. Bilaterally reduced paracentral response amplitudes contradicted the preliminary diagnosis. Subsequently central serous chorioretinopathy was diagnosed. Follow-up showed normalization of the MF-ERG responses in the left eye while retinal function in the right eye showed initial worsening. The noninvasive MF-ERG lends itself to follow-up in patients with central serous chorioretinopathy.

[Multifocal electroretinogram (MF-ERG) in diagnosis of macular changes. Example: senile macular degeneration]. / Multifokales Elekroretinogramm (MF-ERG) in der Diagnostik von Makulaveränderungen. Beispiel: Altersabhängige Makuladegeneration (AMD).

PURPOSE: Small areas of retinal pathology may pose diagnostic difficulties. The noninvasive multifocal electroretinogram (MF-ERG) provides a topographical mapping of retinal function. Its role in the diagnosis of macular diseases is examined in age-related macular degeneration (AMD). AMD is a main cause of central visual loss in the elderly population, affecting the second eye in 75%. METHODS: MF-ERG recordings of three patients with AMD were compared to the findings of fundus photography and fluorescein angiography. During the MF-ERG recordings the central 50 degrees of the retina was stimulated. The visual stimulus consisted of 241 hexagons that alternated, independently and pseudorandomly, between black and white according to a special predetermined binary sequence. Local retinal response components were extracted using the Fast m-Transform Algorithm. RESULTS: Three of six eyes had undergone cataract surgery with implantation of a posterior chamber lens (PCL). In accordance with an increase in light transmission through PCLs, these eyes showed an increase in the MF-ERG responses. MF-ERG allowed accurate topographic mapping of focal areas of retinal dysfunction in all patients tested. There was good correspondence to anatomical changes detected by fluorescein angiography. CONCLUSION: The high resolution of the MF-ERG enables detection of small areas of retinal pathology. It thus presents a clinically useful, noninvasive method in the early diagnosis and follow-up of macular disease.

Effects of refractive blur on the multifocal electroretinogram.

A significant difference in the response density of the MF-ERG response has been suggested for every 2 diopter change of refraction. The influence of refractive blur on the MF-ERG was studied in 8 healthy volunteers using either the VERISTM system (Group A: n=5) or Retiscan(TM) (Group B: n=3). For each eye recordings were obtained with a corrective lens of -3 dpt, 0 dpt, +3 dpt and +6 dpt placed in front of the contact lens electrode. The viewing distance was adjusted to compensate for the induced changes in the retinal image size. When the changes in retinal image size due to the refractive lens were compensated for, no influence due to refraction was observed in either latencies or amplitudes of (KI (P > 0.05). This held true for the central response average (four degrees) as well as for the outer 6-25 degrees. In KII.1 only the peripheral amplitudes of Group B showed an influence due to refraction (P < or = 0.05). This may be due to adaptation as the recordings of group B were obtained in succession. As expected, significant differences were observed when the recordings obtained with the different systems were compared (P < or = 0.05).

Mapping of retinal function in diabetic retinopathy using the multifocal electroretinogram.

PURPOSE: To investigate focal abnormalities in the electroretinogram (ERG) signal in diabetic patients, with and without retinopathy, using a multifocal ERG. METHODS: Sixteen patients with diabetes mellitus, 8 of whom had diabetic retinopathy (mean duration of diabetes: 18.5 years) and 19 approximately age-matched healthy volunteers underwent multifocal ERG testing. One hundred three retinal locations within the central 50 degrees were stimulated concurrently, according to a pseudorandom m-sequence. Response components were extracted for each stimulated retinal location. RESULTS: In diabetic patients with retinopathy, the overall amplitudes were reduced (P < 0.01), and peak implicit times were increased (P < 0.01) in the first-order component (mean flash response) and in the first slice of the second-order component (local two flash interaction). In addition, local reductions of amplitude could be seen in the first- and second-order components. In patients without retinopathy, only amplitudes of the second-order component were reduced (P < 0.01). Another salient difference was observed in a special feature of the second-order component that was reduced in diabetic patients, with and without retinopathy (P < 0.05). CONCLUSIONS: Second-order components depend on nonlinear dynamics. Thus our findings indicate changes in the nonlinear dynamics of a fast-gain control in diabetic patients, presumably located in the inner retina. This suggests that early functional changes of the inner retina are evident in diabetic patients before impairment of the outer retina is observed. Multifocal nonlinear analysis permits the detection of subclinical diabetic retinopathy and offers the advantage of topographic mapping of retinal dysfunction.

Eye movement in amyotrophic lateral sclerosis: a longitudinal study.

Eye muscles and the sphincter muscles of the bowel and bladder were formerly thought to be spared in amyotrophic lateral sclerosis (ALS), a neurodegenerative disorder. As frequent subclinical impairment of the anal sphincter muscles in ALS has recently been reported, We suspected an earlier and more frequent, if subclinical, involvement of the oculomotor system than hitherto described. Starting in 1993, we repeatedly studied oculomotor involvement in eight patients with (ALS) using routine orthoptic examination techniques as well as electro-oculographic recordings of ocular movements. Three patients had consistently normal clinical examinations but progressive changes in electro-oculography (EOG). Three patients showed intermittent impairment of eye movements with normal EOG (one patient) or with progressive changes on EOG examination. In two patients, both clinical examination and EOG were progressively pathologic. These findings provide further evidence of early oculomotor involvement, e.g., prior to respiratory failure, in ALS than previously suspected. Since EOG changes seemed to be detectable in all but one patient, EOG changes may allow earlier, subclinical detection of impaired eye movement and thus reveal even an increased frequency of oculomotor impairment in ALS. Due to the heterogeneity of ALS the number of patients examined to settle finally the question of oculomotor involvement in ALS needs to be increased. Newly developed software will allow further interpretation and comparison of more data and, thus, should offer further help in detecting early changes.

The cornea and systemic diseases.

Corneal involvement in systemic diseases is multifold. Dry eye syndrome may be associated with autoimmune diseases. Type I diabetes mellitus shows a positive correlation between HbA1c-level and dry eye syndrome. Autoimmune diseases and occasionally malignancies may be associated with ulcerative keratitis. Opportunistic infections do not only occur in the immunocompromised patient. Storage diseases can cause corneal deposits that may be of great diagnostic halp. Dermatologic diseases can cause various corneal lesions or opacifications.

[Disorders of eye movement in amyotrophic lateral sclerosis--report of 2 patients]. / Augenbewegunsstörungen bei Amyotropher Lateralsklerose--Bericht über zwei Patienten.

BACKGROUND: Amyotrophic lateral sclerosis (ALS), a neurodegenerative disorder of unknown origin, was thought to spare the extraocular muscles. Extraocular involvement has recently been reported to occur in the late stages of ALS following respiratory insufficiency. CASE REPORT: We report on two patients with ALS who were referred for screening of oculomotor impairment in ALS. Orthoptic examination in a 64-year-old woman with peripheral ALS revealed retraction of the upper eye lids as well as impaired abduction and upgaze in both eyes, developing prior to respiratory insufficiency. A 50-year-old man with bulbar ALS was found to have bilateral impairment of upgaze as well as a negative Bell's phenomenon. Horizontal pursuit was interrupted by compensatory saccades, vertical fixating saccades were slightly hypometric. These oculomotor changes were also seen prior to respiratory insufficiency. CONCLUSION: These findings provide further evidence of early oculomotor involvement in ALS, e.g. prior to respiratory failure and prior than previously suspected. Oculomotor impairment may occur in both the peripheral and the bulbar type of ALS.

[Non-syphilitic interstitial keratitis and inner ear deafness in the initial phase of Wegener's granulomatosis]. / Nicht syphilitische interstitielle Keratitis und Innenohrschwerhörigkeit in der Initialphase einer Wegenerschen Granulomatose.

BACKGROUND: The aim of the present report is to briefly discuss the possible underlying diseases in a syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. PATIENT: A 46-year-old patient presented with interstitial keratitis, vestibuloauditory hearing reduction and necrotising atrophy of the nasal septum. RESULTS: Clinical tests for lues, tuberculosis, sarcoidosis and autoimmune diseases were negative. CONCLUSION: For clinical aspects Cogan's syndrome in association with an initial phase of Wegener's granulomatosis are discussed as possible underlying diseases.