Jun Sugasawa

Visual field defects of optic neuritis in neuromyelitis optica compared with multiple sclerosis

BackgroundNeuromyelitis optica (NMO) is an inflammatory demyelinating disease that predominantly affects the optic nerves and the spinal cord, and is possibly mediated by an immune mechanism distinct from that of multiple sclerosis (MS). Central scotoma is recognized as a characteristic visual field defect pattern of optic neuritis (ON), however, the differing pathogenic mechanisms of NMO and MS may result in different patterns of visual field defects for ON.MethodsMedical records of 15 patients with NMO and 20 patients with MS having ON were retrospectively analyzed. A thorough systemic and neurological examination was performed for evaluating ON. The total number of relapses of ON and visual fields was investigated. Visual fields were obtained by Goldmann perimeter with each ON relapse.ResultsAll MS patients experienced central scotoma, with 90% of them showing central scotoma with every ON relapse. However, 53% of NMO patients showed central scotoma with every ON relapse (p = 0.022), and the remaining 47% of patients experienced non-central scotoma (altitudinal, quadrant, three quadrant, hemianopia, and bitemporal hemianopia). Thirteen percent of NMO patients did not experience central scotoma during their disease course. Altitudinal hemianopia was the most frequent non-central scotoma pattern in NMO.ConclusionsNMO patients showed higher incidence of non-central scotoma than MS, and altitudinal hemianopia may be characteristic of ON occurring in NMO. As altitudinal hemianopia is highly characteristic of ischemic optic neuropathy, we suggest that an ischemic mechanism mediated by anti-aquaporin-4 antibody may play a role in ON in NMO patients.

Orbital apex syndrome associated with herpes zoster ophthalmicus.

We report our findings for a patient with orbital apex syndrome associated with herpes zoster ophthalmicus. Our patient was initially admitted to a neighborhood hospital because of nausea and loss of appetite of 10 days’ duration. The day after hospitalization, she developed skin vesicles along the first division of the trigeminal nerve, with severe lid swelling and conjunctival injection. On suspicion of meningoencephalitis caused by varicella zoster virus, antiviral therapy with vidarabine and betamethasone was started. Seventeen days later, complete ptosis and ophthalmoplegia developed in the right eye. The light reflex in the right eye was absent and anisocoria was present, with the right pupil larger than the left. Fat-suppressed enhanced T1-weighted magnetic resonance images showed high intensity areas in the muscle cone, cavernous sinus, and orbital optic nerve sheath. Our patient was diagnosed with orbital apex syndrome, and because of skin vesicles in the first division of the trigeminal nerve, the orbital apex syndrome was considered to be caused by herpes zoster ophthalmicus. After the patient was transferred to our hospital, prednisolone 60 mg and vidarabine antiviral therapy was started, and fever and headaches disappeared five days later. The ophthalmoplegia and optic neuritis, but not the anisocoria, gradually resolved during tapering of oral therapy. From the clinical findings and course, the cause of the orbital apex syndrome was most likely invasion of the orbital apex and cavernous sinus by the herpes virus through the trigeminal nerve ganglia.

Bilateral, Nearly Simultaneous Anterior Ischemic Optic Neuropathy Complicated by Diabetes and Bilateral, Small, Crowded Optic Discs

BackgroundBilateral simultaneous anterior ischemic optic neuropathy (AION) is uncommon. We report a case of bilateral and nearly simultaneously occurring AION.CaseA 61-year-old man was referred to our hospital with bilateral optic disc edema.ObservationsVisual field testing demonstrated inferior nasal defect OD and inferior defect OS. Fluorescein angiography showed a delay of dye filling in the superior part of the optic disc in both eyes. The patient had poorly controlled diabetes. A mild increase in erythrocyte sedimentation rates and creactive protein was observed, but the results of temporal artery biopsy were negative. His optic discs were small and lacked biological cups, which has been identified as a risk factor for developing AION.ConclusionsThe complications of the structural anomaly, also known as “disc at risk,” and diabetes might have caused the bilateral and nearly simultaneously occurring AION. Jpn J Ophthalmol 2005;49:235–238

Abnormalities of visual-evoked potentials and pupillary light reflexes in a family with autosomal dominant occult macular dystrophy

Occult macular dystrophy (OMD) is an unusual inherited or sporadic macular dystrophy characterized by an essentially normal appearing ocular fundus and normal ocular circulation determined by fluorescein angiography but with a progressive decrease of visual acuity. We present a family with OMD who have a delay in the implicit times of both the visual‐evoked potentials and the pupillary light reflexes. Because these delays are usually indicative of optic nerve disorders, deciding on a diagnosis between OMD and optic nerve disorder should be done carefully. We are not aware of any studies that have reported these findings in patients with OMD.

Vitrectomy for Tractional Retinal Detachment with Twin Retinal Capillary Hemangiomas in a Patient with Von Hippel-Lindau Disease: A Case Report.

Purpose: The purpose of this study was to report a case of Von Hippel-Lindau disease (VHL) with twin retinal capillary hemangiomas that was successfully treated by vitreous surgery for tractional retinal detachment following laser photocoagulation. Case: A 44-year-old male presented at our university hospital after noticing decreased visual acuity in his right eye. The patient had previously undergone multiple operations for cerebellar, thoracic, and lumbar spine hemangioblastomas when he was approximately 19 years old. Upon initial examination, ocular findings revealed twin connected retinal capillary hemangiomas around the temporal upper area of the patient’s right eye. The patient was subsequently diagnosed with VHL based on his medical history and current observations of the ocular fundus. Tractional retinal detachment had occurred as the result of the formation of proliferative membranes following laser photocoagulation. The patient underwent vitreous surgery to treat the tractional retinal detachment, resulting in a successful postoperative outcome. Conclusion: The findings of this study show the possibility that proliferative changes and tractional retinal detachment can arise following photocoagulation for retinal capillary hemangiomas in patients with VHL.

A comparison of sex steroid concentration levels in the vitreous and serum of patients with vitreoretinal diseases

The purpose of this study was to compare steroid hormone concentration levels in the vitreous and serum of vitreoretinal disease patients to elucidate the possibility of neurosteroid production in the retina. Serum and vitreous samples were collected from vitrectomy patients, and estradiol (E2) and testosterone (T) concentrations were measured using electro-chemiluminescence immunoassay. We measured E2 in epiretinal membrane (ERM, n = 14), macular hole (MH, n = 18), proliferative diabetic retinopathy (PDR, n = 20), and retinal detachment (RD, n = 19) cases, and T in ERM (n = 14), MH (n = 17), PDR (n = 13), and RD (n = 17) cases. No statistically significant age differences existed among the groups. Mean respective E2 concentrations (pg/ml) in the male/female vitreous were ERM: 6.67±4.04/18.82±7.10, MH: 10.3±7.02/17.00±4.8, PDR: 4.2±3.05/15.83±3.46, and RD: 10.00±4.58/16.06±4.57, while those in serum were ERM: 31.67±5.51/5.82±1.08, MH: 21.00±8.89/7.53±3.2, PDR: 29.20±7.07/12.75±10.62, and RD: 24.33±6.51/7.5±4.42. E2 concentrations were significantly higher (P<0.001) in the male serum than vitreous, yet significantly higher in the female vitreous than serum. Mean respective T concentrations (ng/ml) in the male/female vitreous were ERM: 0.15±0.03/0.15±0.01, MH: 0.15±0.01/0.15±0.01, PDR: 0.15±0.03/0.16±0.12, and RD: 0.14±0.01/0.17±0.08, while those in serum were ERM: 4.54±1.46/0.16±0.01, MH: 8.04±2.29/0.16±0.10, PDR: 5.14±1.54/0.22±0.11, and RD: 3.24±0.75/0.17±0.10. T concentrations were high in the male serum, yet extremely low in the male and female vitreous and female serum. High concentrations of E2 were found in the vitreous, and women, in particular, exhibited significantly higher concentrations in the vitreous than in the serum. This finding suggests the possibility that in vitreoretinal disease cases, the synthesis of E2 is increased locally only in female eyes.

Long-Term Follow-Up Case of Multiple Retinal Arterial Macroaneurysms Developing Branch Retinal Vein Occlusion following Ruptured Macroaneurysm

Purpose: Retinal arterial macroaneurysm (RAM) has been reported in association with branch retinal vein occlusion (BRVO), and usually BRVO precedes RAM. We present a long-term follow-up case report of unilateral multiple RAMs that developed BRVO following ruptured RAM in the same retinal quadrant. Case Presentation: An 80-year-old woman presented with floaters in her right eye in June 2012. Visual acuity (VA) was 20/25 in her right eye with posterior capsular opacity. Her fundus showed the first ruptured RAM at the superotemporal vascular arcade with subinternal limiting membrane and subretinal hemorrhages not involving the macula. These were absorbed gradually with a VA of 20/20. After 2 years, the second RAM at the proximal superotemporal vascular arcade developed and impending BRVO occurred with macular edema at the distal site of the RAM. With the RAM located close to the arteriovenous crossing, her VA was dropped to 20/60. Intravitreal injection of ranibizumab was performed and macular edema was resolved with improved vision of 20/30. Three months later, she realized a sudden vision loss of 2/200. Her posterior pole showed massive pre- and subretinal hemorrhages, and vitrectomy was performed. The source of bleeding was the third RAM’s rupture in a different artery. Her vision improved to 20/30. The unaffected eye showed no RAMs. Conclusion: We experienced a long-term follow-up case of multiple RAMs showing different courses. We should cautiously note that BRVO can occur following RAM at the arteriovenous crossing.

Vitrectomy for Proliferative Diabetic Retinopathy Associated with Klinefelter Syndrome.

Introduction: We encountered a patient with Klinefelter syndrome (KS) who experienced poor outcomes after vitrectomy for proliferative diabetic retinopathy (PDR). Case: A 44-year-old male with poorly controlled diabetes was diagnosed with KS by chromosome analysis. Ocular findings revealed severe PDR complicated with extensive preretinal hemorrhages and traction retinal detachment in his left eye, and pars plana vitrectomy was subsequently performed for treatment. Results: A clotting hemorrhage developed during surgery and proved difficult to control. Due to postoperative bleeding and redetachment, the vitrectomy was repeated. At the second operation, we performed a silicone oil tamponade; however, the retina was redetached under the silicone oil, and the light perception vision ultimately disappeared. Conclusion: The patient, despite showing increased blood coagulability due to diabetes, presented severe coagulopathy, likely related to KS. In patients with KS and severe PDR, the potential difficulty of vitrectomy should always be kept in mind.

A case of eosinophilic chronic rhinosinusitis associated with optic neuropathy

We report a case of eosinophilic chronic rhinosinusitis (ECRS) associated with optic neuropathy. The visual acuity in the right eye was suddenly reduced to no light perception on awakening in the morning. Fundus examination of both eyes on the same day showed no remarkable changes. Emergency computed tomography showed pan-sinusitis bilaterally and a partial defect of the sphenoid bone on the right side. From the clinical findings, the case was diagnosed as optic neuropathy associated with chronic sinusitis. Endoscopic sinus surgery (ESS) was performed on the same day, and all of the major sinuses were found to be filled with highly viscous fluid. Part of the optic canal had a defect probably due to inflammatory invasion from the adjacent sphenoid bone. Steroid therapy was started immediately postoperatively. Histopathological examination of excised polyps showed that numerous eosinophils had invaded the polyps but no hyphae were present. The patient reported that he had bronchial asthma and had had nasal polypectomy. Six months after the ESS and steroid therapy, the patient had a recurrence of the sinusitis. At that time, laboratory examination showed an elevation of total IgE and eosinophil numbers. From the clinical findings and course, this case was diagnosed as ECRS accompanied by optic neuropathy. Although ECRS rarely has ocular complications, the inflammation can spread and the optic nerve can be affected.

Acute Infarction at the Opto-Chiasmal Junction Detected by Diffusion Weighted Magnetic Resonance Imaging

ABSTRACT A localized infarction at the junction of the optic nerve and chiasm (opto-chiasmal junction) is rare and difficult to detect by conventional neuro-imaging techniques. The purpose of this report is to show the diagnostic potential of diffusion-weighted magnetic resonance imaging for lesions at this location. A 62-year-old woman complained of sudden visual loss in her left eye. Visual field testing strongly suggested that the left opto-chiasmal junction was damaged. Diffusion weighted imaging demonstrated an area of restricted diffusion at the left opto-chiasmal junction, whereas the T2 weighted and fluid-attenuated inversion recovery sequences revealed no abnormality.