Late Dislocation of In-the-Bag Intraocular Lens in Three Patients with Multiple Chorioretinal Atrophy Associated with Sarcoidosis.

PURPOSE: A late dislocation of an in-the-bag intraocular lens (IOL) is strongly associated with pseudoexfoliation and less with retinitis pigmentosa, prior vitreoretinal surgery, and uveitis. We present our findings of late in-the-bag IOL dislocation in three patients with multiple chorioretinal atrophy associated with sarcoidosis. METHODS: Observational study of three elderly female Japanese patients with a history of uveitis from sarcoidosis who presented with a late dislocation of an in-the-bag IOL. RESULTS: The late in-the-bag IOL dislocation occurred in the convalescent/quiescent stage of the sarcoidosis. Peripheral multifocal chorioretinal atrophy was the main manifestation in all patients who were diagnosed with definite or presumed sarcoidosis. The dislocated IOLs were successfully removed and new IOLs were implanted with scleral suture fixation followed by no remarkable active uveitis. CONCLUSION: Clinicians should be aware that patients with peripheral multifocal chorioretinal atrophy associated with sarcoidosis can have a late in-the-bag IOL dislocation.

Macular Hole Formation in Eye After Cryotherapy and Intravitreal Bevacizumab Treatment for Vasoproliferative Tumor.

PURPOSE: To report a case of a full-thickness macular hole (MH) that developed after cryotherapy and intravitreal bevacizumab injection (IVB) to treat a retinal vasoproliferative tumor (VPT). METHODS: Case report of a man with a retinal VPT. RESULTS: A 64-year-old Japanese man complained of blurred vision in his right eye. At the initial examination, his best-corrected visual acuity (BCVA) was 20/25 in the right eye and 20/20 in the left eye. Ophthalmoscopy showed a VPT in the lower peripheral retina of the right eye. An exudative retinal detachment and hard exudates were seen around the tumor. Cryotherapy and intravitreal injections of bevacizumab (IVB) were performed. Although the exudative changes were reduced, a MH developed two months after the initial IVB treatment. He underwent 25-gauge pars plana vitrectomy, and the MH was closed. His postoperative BCVA was 20/32 and the VPT was inactive. The reduced BCVA was due to damage of the outer retinal layers. CONCLUSION: Our findings indicate that cryotherapy and IVB are effective treatments for VPT although the possibility of developing a MH should be considered.

Stereopsis After Unilateral Macular Hole Surgery with Internal Limiting Membrane Peeling.

PURPOSE: We have shown distinct morphological changes of the foveal retina such as horizontally asymmetrical retinal thicknesses and displacements of the fovea toward the optic disc after successful idiopathic macular hole (MH) closure by MH surgery with internal limiting membrane (ILM) peeling. The purpose of this study was to determine whether these morphological changes will alter the stereoacuity. SETTING: This was a non-randomized, retrospective study conducted on patients who underwent MH surgery at the Matsumoto Dental University Hospital, Nagano, Japan. METHODS: Forty patients (22 women, 18 men) who underwent MH surgery with ILM peeling for a unilateral MH were studied. At >6 months after the PPV, the stereoacuity was measured with the Titmus stereotest (TST) and the TNO stereotest (TNO). The relationship between the stereoacuity and the age, MH size, best-corrected visual acuity (BCVA), foveal retinal thickness, and amount of nasal shift of the retina was determined. RESULTS: The mean postoperative BCVA was -0.05 ± 0.11 logarithm of the minimum angle of resolution (logMAR) units. The percentage of cases with good stereoacuity of ≤100 sec of arc was 87.5% with the TST and ≤240" was 90% with the TNO test. A better stereoacuity was significantly correlated with a younger age (r = 0.36, P <0.05; with TNO), smaller MH (r = 0.51, P < 0.001, with TST), better postoperative BCVA (r = 0.43 P <0.01 with TST; r = 0.49, P <0.01 with TNO), but not with the parafoveal retinal thickness or degree of nasal shift. CONCLUSION: The postoperative stereopsis is relatively good in patients with better postoperative BCVA after MH surgery with ILM peeling, even though foveal morphological changes had occurred.

Comparisons of foveal thickness and slope after macular hole surgery with and without internal limiting membrane peeling.

BACKGROUND: We have shown that the foveal contour was asymmetrical after idiopathic macular hole (MH) closure by pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling. The purpose of this study was to determine whether these morphological changes differ in eyes after PPV without ILM peeling. METHODS: Ten eyes of 10 patients that underwent PPV without ILM peeling and 12 eyes of 11 patients with ILM peeling were studied. The MH in all eyes was <400 µm in diameter. Six months after the PPV, the macular thickness and foveal slope around the closed MH were determined by spectral-domain optical coherence tomography. The thickness of the ganglion cell complex was measured by another spectral-domain optical coherence tomography instrument >6 months after the surgery. RESULTS: The mean parafoveal retinal thickness in the non-peeled group was 367.1 µm in the nasal (N), 353.0 µm in the temporal (T), 366.9 µm in the superior (S), and 357.3 µm in the inferior (I) sectors. The T, S, and I sectors were significantly thicker than the corresponding sectors in the ILM peeled group (p=0.0008, 0.003, and 0.03, respectively). The mean ganglion cell complex was thicker not only in the N sector but also in the T sector in the non-peeled group. The mean retinal slopes in the non-peeled group (N, 40.2°; T, 37.6°; S, 41.2°; I, 39.5°) were flatter than those in the peeled group (N, 52.3°; T, 43.6°; S, 50.8°; I, 51.9°; p=0.009, 0.09, 0.008, and 0.017, respectively). CONCLUSION: The symmetrical fovea after MH surgery in the non-ILM peeled eyes indicates that the asymmetrical fovea after ILM peeling was probably due to the ILM peeling.

Displacement of fovea toward optic disk after macular hole surgery with internal limiting membrane peeling.

PURPOSE: The purpose of this study was to demonstrate a displacement of the foveal depression toward the optic disk after idiopathic macular hole (MH) surgery with internal limiting membrane (ILM) peeling. METHODS: Two patients with a unilateral MH developed an MH in the fellow eyes. Vitrectomy with ILM peeling was performed on the fellow eye to close the MH. Images of spectral-domain optical coherence tomography (SD-OCT) were used to measure the disk-to-fovea distances pre MH formation, after MH formation, and 6 months after the closure of the MH. RESULTS: The disk-to-fovea distance was shorter at 6 months than after the development of the MH (4,109 µm and 4,174 µm in Case 1 and 4,001 µm and 4,051 µm in Case 2). CONCLUSION: These results indicate that the fovea moves nasally after the MH surgery with ILM peeling.

Transient Increase of Retinal Nerve Fiber Layer Thickness after Vitrectomy with ILM Peeling for Idiopathic Macular Hole.

Purpose. The purpose of this study was to determine the long-term changes in the circumpapillary retinal nerve fiber layer (RNFL) thickness following macular hole surgery with internal limiting membrane (ILM) peeling combined with phacoemulsification. Methods. Thirty-eight eyes of 37 patients who had pars plana vitrectomy (n = 36) between 2010 and 2014 were studied. The average thicknesses of the global and the six sectors of the RNFL were determined before and at 1, 3, 6, 12, and 24 (n = 22) months (M) after the surgery by spectral-domain optical coherent tomography. The postoperative mean RNFL thickness at each time was compared to that before the surgery by paired t-tests. Results. The RNFL of the operated eyes was significantly thicker at 1 month (1 M) and 3 M in all but the inferior-nasal sectors. The significant increase remained until 12 M in the superior-temporal and superior-nasal sectors. In addition, the RNFL was also significantly thicker in the temporal-inferior sector at 12 M based on the findings in 38 eyes. Conclusions. The postoperative RNFL was thicker in all but the nasal-inferior sector for at least 12 M after surgery. This prolonged increase of the RNFL thickness may indicate damage and mild edema of the RNFL.

Asymmetrical steepening of the foveal contour after macular hole surgery with internal limiting membrane peeling.

PURPOSE: We have previously shown that the thickness of the parafoveal retina was asymmetrical in spectral-domain optical coherence tomographic (SD-OCT) images after idiopathic macular hole (MH) closure by pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling. The aim of this study was to determine whether the foveal slopes around the closed MH were also asymmetrical. METHODS: Forty-three eyes with an MH treated with PPV with ILM peeling at Matsumoto Dental University Hospital were studied. The foveal slope was determined with ImageJ software in both the horizontal and vertical images obtained by SD-OCT at 6 months after the surgery. The possible related factors were also compared. RESULTS: The mean angles of the foveal slopes of the nasal, superior, inferior, and temporal sectors were 31.4° ± 6.2°, 31.2° ± 5.4°, 31.6° ± 7.4°, and 29.9° ± 6.6°, respectively, in the fellow eyes. The corresponding values in the operated eyes were significantly steeper at 50.4° ± 9.1°, 46.9° ± 11.3°, 48.6° ± 11.3°, and 41.3° ± 11.2°, respectively (P < 0.00001). Both the nasal and inferior slopes were significantly steeper than the temporal slopes of the same operated eye (P = 0.002 and P = 0.023, respectively). There was no significant correlation between each slope and the visual acuity, MH size, area of peeled ILM, postoperative parafoveal retinal thickness, defect of the retinal outer layers, disc-foveal distance, stage, and use of dye for ILM peeling. CONCLUSIONS: Although the postoperative foveal contour was markedly changed asymmetrically, the significance of the change was not determined.

Rotigotine Transdermal Patch Does Not Make Parkinson Disease Patients Sleepy During Daytime.

OBJECTIVES: To assess quantitatively the influence of rotigotine transdermal patch on daytime sleepiness, the most common adverse event by non-ergot dopamine agonists (DAs), in Parkinson disease (PD) patients. METHODS: An open-label study enrolled PD patients with unsatisfactory control of motor symptoms. Treatment with rotigotine transdermal patch was titrated to optimal dose (4-8 mg/24 hours) over 2 to 4 weeks. Primary outcome was Epworth Sleepiness Scale (ESS) for daytime sleepiness. Secondary outcomes included Hoehn&Yahr stage, time spent with dyskinesia, Clinical Global Impression of Improvement (CGI-I) of motor symptoms, adverse events, and compliance. RESULTS: The subjects were 31 PD patients (age 72 ± 8, Hoehn &Yahr stage 2.7 ± 0.9, mean ± SD). The ESS did not increase after rotigotine treatment (7.2 ± 4.9 before treatment, 6.2 ± 4.0 with 4 mg/24 hour, and 8.1 ± 6.4 with 8 mg/24 hour). The CGI-I score improved after treatment; responder rate reached 88.9% with 8 mg/24 hours. No patients showed worsening in other secondary outcomes. In 13 patients treated with equivalent doses of rotigotine switched from other DAs (pramipexole, ropinirole, and cabergoline), ESS did not increase after treatment (10.0 ± 4.6 before and 8.6 ± 4.5 after treatment) and decreased without worsening of CGI-I in 54% patients. Other secondary outcomes did not worsen after treatment. CONCLUSIONS: Twenty four-hour transdermal delivery of rotigotine at doses up to 8 mg/24 hours does not worsen the daytime sleepiness in PD patients and often improves it when switched from other non-ergot DAs. This is achieved together with satisfactory improvement in motor symptoms, demonstrating that this new modality of non-ergot DA is well tolerated and beneficial in PD patients.

Retinal angiomatous proliferation in an eye with cuticular drusen.

PURPOSE: To report the presence of retinal angiomatous proliferation (RAP) in an eye with cuticular drusen detected by fluorescein and indocyanine green angiography by confocal scanning laser ophthalmoscopy and by spectral-domain optical coherence tomography (SD-OCT). METHODS: Case report of a 65-year-old Japanese woman with cuticular drusen. RESULTS: At her first ophthalmic examination, her visual acuity was 20/20 in both eyes. An ophthalmoscopy showed many small subretinal pigment epithelial deposits in both eyes. These deposits had a 'saw-tooth pattern' in the SD-OCT images. During the follow-up examination, retinal hemorrhages were observed, and fluorescein angiography showed a 'stars-in-the-sky' appearance and intraretinal neovascularization. The patient was diagnosed with cuticular drusen associated with RAP. CONCLUSION: We suggest that the cuticular drusen were associated with RAP, so periodic follow-up examinations are needed for patients with cuticular drusen for the early detection and treatment of RAP.

Retinal involvement in uveitis associated with Hodgkin disease.

PURPOSE: To describe a patient with Hodgkin disease with posterior uveitis who also had a thinning of the retina and an antiretinal autoantibody in his serum. METHODS: Our patient was a 58-year-old man who had been diagnosed with Hodgkin disease. He had a complete ophthalmologic examination including fluorescein angiography, electroretinography, perimetry, and spectral-domain optical coherence tomography. A search for antiretinal antibodies in the serum was made by Western blot analysis, and the retinal sites reactive to the antibodies were determined by immunohistochemistry. RESULTS: The ocular signs were mild cellular infiltration in the anterior chamber and vitreous, and small, round chorioretinal lesions in the peripheral retina. The electroretinograms were slightly reduced. Small ring-like scotomas were detected in the Goldmann visual fields. An antiretina-specific 116-kDa antibody was detected in the serum by Western blot analysis, and the antibody reacted with the ganglion cell and inner nuclear layers of mice retinas. Although the visual acuities were maintained for over eight years, the macular thickness measured in the spectral-domain optical coherence tomography images was reduced. CONCLUSION: The presence of an antiretinal autoantibody, granulomatous uveitis, and retinal thinning in a patient with Hodgkin disease suggests that the patient had a granulomatous uveitis associated with Hodgkin disease or lymphoma-associated uveitis with retinal involvement.

Screening for impaired cognitive domains in a large Parkinson's disease population and its application to the diagnostic procedure for Parkinson's disease dementia.

BACKGROUND: Dementia is a new focus of research on improved treatment for Parkinson's disease (PD). In 2007, a screening tool for PD dementia (PD-D) was developed by the Movement Disorder Society (Level I testing), which still requires verification by a large population study. METHODS: We conducted a cross-sectional and multicenter study including 13 institutions administering the Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) to 304 PD patients (mean age: 70.6 ± 8.3 years; mean Hoehn and Yahr stage: 2.7 ± 0.7). RESULTS: In all, 34.5% of the patients had MMSE scores <26; 94.3% of these patients had impairments in ≥2 cognitive domains and met the criteria for probable PD-D by Level I testing. Executive dysfunction combined with attention and memory impairment was most common (51.4%). In the Level I subtests of executive function, the score for phonemic fluency declined by <50% in patients with high MoCA scores (24-30 points) and lacked specificity for PD-D. No patient had visuospatial impairment (measured by the pentagon copying subtest) alone, and the score for pentagon copying stayed at ≥70% even in patients with low MMSE scores (12-25 points), therefore lacking sensitivity for PD-D. CONCLUSIONS: Level I testing with administration of the MMSE and MoCA is a practical and efficient screening tool for PD-D. However, the phonemic fluency and pentagon copying tests should be replaced by more specific/sensitive ones when screening for PD-D.

Clinical features and varieties of non-motor fluctuations in Parkinson's disease: a Japanese multicenter study.

OBJECTIVE: This multicenter cross-sectional study aimed to investigate the clinical features and varieties of non-motor fluctuation in Parkinson's disease (PD). METHODS: To identify motor and non-motor fluctuation, we employed the wearing-off questionnaire of 19 symptoms (WOQ-19) in 464 PD patients. We compared the frequency of levodopa-related fluctuation as identified by the WOQ-19 with recognition by neurologists. We compared patients with both motor and non-motor fluctuations with those who only had motor fluctuations. Non-motor fluctuations were separated into psychiatric, autonomic, and sensory categories for further analysis. RESULTS: The patients' average age was 70.8 ± 8.4 years (mean ± SD) and disease duration was 6.6 ± 5.0 years. The frequency of motor fluctuations was 69% and for non-motor fluctuation 40%. Fifty-three percent of patients with motor fluctuations also had non-motor fluctuations, whereas 93% of patients with non-motor fluctuations also had motor fluctuations. The WOQ-19 showed a sensitivity of 82% but a specificity of only 40%. The patients with both non-motor and motor fluctuations exhibited more severe motor symptoms, more non-motor symptoms and higher levodopa daily doses (p < 0.05). Patients had significantly higher fluctuation rates if they had psychiatric (49%) and sensory (45%) symptoms than patients with autonomic symptoms (32%, p < 0.01). Forty-eight percent of patients with non-motor fluctuations exhibited more than one type of non-motor fluctuation. CONCLUSION: Forty percent of PD patients presented with non-motor fluctuations, and almost half of these exhibited more than one type. Appropriate recognition of levodopa-related fluctuations, both motor and non-motor, can lead to treatment modifications in PD patients.

Retinal thickness in eyes with idiopathic macular hole after vitrectomy with internal limiting membrane peeling.

PURPOSE: To compare the thickness of each retinal layer in the parafoveal and perifoveal regions of eyes after successful closure of a macular hole (MH) by pars plana vitrectomy with internal limiting membrane (ILM) peeling to the corresponding areas of the normal fellow eyes. METHODS: Twenty-two eyes of 22 patients with an idiopathic MH who underwent PPV with ILM peeling at the Matsumoto Dental University Hospital were studied. The retinal thickness was measured manually with the volume scan mode of the Spectralis HRA + OCT (Heidelberg Engineering, Germany). RESULTS: The average postoperative parafoveal thickness of the temporal sector was 314.8 µm, and that of the corresponding area of the fellow eyes was 325.0 µm (P = 0.01). The parafoveal thickness of the nasal sector was 360.7 µm, and that of the fellow eyes was 339.6 µm (P < 0.0001). Changes in the inner retinal layer thicknesses contributed to the decreased temporal and increased nasal sectors. The perifoveal retinal thickness was significantly increased in all sectors after PPV, probably due to an increase in the outer nuclear layer thickness. CONCLUSIONS: PPV with ILM peeling for MHs can cause microstructural changes in wide areas of the macula region, suggesting a loss of longitudinal support of the Müller cells.

Age-related decrease of the retinal vasculature area identified with a novel computer-aided analysis system.

Fundus photographs enable non-invasive analysis of the status of the microcirculation by directly observing the retinal vasculature. Retinal microvascular abnormalities are important clinical markers of hypertension and arteriosclerosis, but retinal microvascular changes can be observed in older individuals without hypertension. In this study, our goal is to elucidate the effects of aging on fundus vessels in the retinal photograph. We analyzed retinal vessels of 161 healthy volunteers (49.5 ± 18.7 years, range 18-87) using in-house computer-aided measurement system to measure areas and diameters of all retinal vessels across the entire area of a retinal photograph. The vessels were segmented according to color, and then their area, size, length and thickness were measured by image processing. We also analyzed the correlation between total blood vessel area, age and mean arterial blood pressure (MABP). The decrease in total blood vessel area was dependent on both age and MABP. Moreover, decrease in blood vessel area was also correlated with age for the normotensive group. Furthermore, the slope of the regression line for retinal vessel area with MABP was significantly higher in participants aged ≤ 60 years than in those aged over 60 years. Changes in retinal vessel area with aging were observed in both arterioles and venules. In conclusion, we found the significant decrease in retinal vessel area that is correlated well with calendar age. Therefore, we need to carefully apply traditional classifications of fundus examination for hypertensive retinopathy in older individuals.

REM sleep behavior disorder in Japanese patients with Parkinson's disease: a multicenter study using the REM sleep behavior disorder screening questionnaire.

REM sleep behavior disorder (RBD) is known to be observed more frequently in patients with an α-synucleinopathy such as Parkinson's disease (PD) than in the general population. The precise prevalence of RBD in Japanese PD patients is not known. Therefore, we investigated the prevalence and the clinical characteristics of patients with RBD in a large population of Japanese patients with PD. We investigated various clinical features and employed the Japanese version of the RBD screening questionnaire on 469 non-demented Japanese PD patients in this multicenter study. Probable or possible RBD was detected in 146 patients (31.1%) and was significantly associated with longer PD duration, higher Hoehn and Yahr stage, higher Unified Parkinson's Disease Rating Scale part III subscale (7 items), more motor fluctuations, and a higher levodopa-equivalent daily dose (p < 0.01). As to the major autonomic dysfunctions, severe constipation was significantly more frequent in PD patients with RBD than in those without it (p < 0.01). The RBD symptoms of 53 patients (39.0%) preceded the onset of PD motor symptoms. The median interval from the onset of RBD symptoms to PD motor symptoms was 17.5 years, and 3 patients had intervals of over 50 years. This large-scale multicenter study revealed that RBD is a frequent non-motor symptom in Japanese patients with PD, which may precede the onset of motor symptoms. Moreover, RBD that increases with the duration and severity of PD may be associated with autonomic dysfunction.

Retinoschisis detected by spectral-domain optical coherence tomography in von hippel-lindau disease.

PURPOSE: To report the presence of retinoschises in a case of von Hippel-Lindau disease detected by spectral-domain optical coherence tomography. METHODS: Case report of a 23-year-old man with von Hippel-Lindau disease who was seen for a regular follow-up examination. RESULTS: Ophthalmoscopy showed several retinal capillary hemangioblastomas in both eyes. Spectral-domain optical coherence tomography showed retinoschises around the retinal capillary hemangioblastoma and also around the feeder and draining vessels of the peripheral larger hemangioblastoma. The retinoschises were found under the internal limiting membrane and also in the inner and outer retinal layers. Intraretinal columns were observed in the retinoschises. CONCLUSION: Our observations show that retinoschises can develop in eyes with von Hippel-Lindau disease. The extensiveness of the retinoschises suggests that the retina is fragile in von Hippel-Lindau disease.

Cancer-associated retinopathy in a patient with seminoma.

PURPOSE: The purpose of this study was to report our findings on a patient with cancer-associated retinopathy who had seminoma. METHODS: A 43-year-old man, who had been diagnosed with Stage 1 seminoma, complained of blurred vision. Fundus examination revealed attenuated retinal arteries, mild optic disk pallor, and a thinning of the outer nuclear layer. Ring scotomas were found in the visual fields of both eyes. The amplitudes of the electroretinograms were moderately reduced. The patient's serum was analyzed for antibodies. RESULTS: Antibodies against recoverin, Hu, and Ma-2 antigens were not detected. However, Western blots of the serum detected 2 bands at 41 and 64 kDa. The 41-kDa band was an anti-retina-specific antibody. Immunoreactivity was identified in the photoreceptor layer of mice retinas. From these findings, we diagnosed the patient with cancer-associated retinopathy. The visual functions remained stable after the removal of the primary lesion at an early stage, treatment with steroids, and intravenous immunoglobulin. CONCLUSION: This is the first case of cancer-associated retinopathy because of seminoma. The progression of the disease was reduced by removing the seminoma at an early stage, treatment with steroids, and intravenous immunoglobulin.

Camptocormia in Japanese patients with Parkinson's disease: a multicenter study.

OBJECTIVE: The aim of this work was to investigate the prevalence of camptocormia and the clinical characteristics of patients with camptocormia in a large population of PD patients. BACKGROUND: Although camptocormia has been recognized as a prominent phenomenon in PD, the previous epidemiological reports were limited, especially in terms of sample size. METHODS: We evaluated 531 PD patients (disease duration: 7.0 ± 5.5 years, mean ± standard deviation). We examined their clinical features and the prevalence of camptocormia. RESULTS: Camptocormia was detected in 22 patients (4.1%) and found in patients who were older and had more severe motor symptoms and a higher levodopa (L-dopa) dose (P < 0.05), compared to the patients without camptocormia. Patients with camptocormia showed significantly higher frequencies of autonomic symptoms, such as constipation and urinary incontinence (P < 0.05). CONCLUSIONS: Camptocormia is uncommon in PD and is associated with disease severity, higher L-dopa dose and higher frequencies of autonomic symptoms.