Epidemiologic and Clinical Characteristics of Optic Neuritis in Japan.

PURPOSE: To elucidate the clinical and epidemiologic characteristics of optic neuritis in Japan. DESIGN: Multicenter cross-sectional, observational cohort study. PARTICIPANTS: A total of 531 cases of unilateral or bilateral noninfectious optic neuritis identified in 33 institutions nationwide in Japan. METHODS: Serum samples from patients with optic neuritis were tested for anti-aquaporin-4 antibodies (AQP4-Abs) and anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) using a cell-based assay and were correlated with the clinical findings. MAIN OUTCOME MEASURES: Antibody positivity, clinical and radiologic characteristics, and visual outcome. RESULTS: Among 531 cases of optic neuritis, 12% were AQP4-Ab positive, 10% were MOG-Ab positive, 77% were negative for both antibodies (double-negative), and 1 case was positive for both antibodies. Pretreatment visual acuity (VA) worsened to more than a median 1.0 logarithm of the minimum angle of resolution (logMAR) in all groups. After steroid pulse therapy (combined with plasmapheresis in 32% of patients in AQP4-Ab-positive group), median VA improved to 0.4 logMAR in the AQP4-Ab-positive group, 0 logMAR in the MOG-Ab-positive group, and 0.1 logMAR in the double-negative group. The AQP4-Ab-positive group showed a high proportion of females, exhibited diverse visual field abnormalities, and demonstrated concurrent spinal cord lesions on magnetic resonance imaging (MRI) in 22% of the patients. In the MOG-Ab-positive group, although posttreatment visual outcome was good, the rates of optic disc swelling and pain with eye movement were significantly higher than those in the AQP4-Ab-positive and double-negative groups. However, most cases showed isolated optic neuritis lesions on MRI. In the double-negative group, 4% of the patients had multiple sclerosis. Multivariate logistic regression analysis of all participants identified age and presence of antibodies (MOG-Ab and AQP4-Ab) as significant factors affecting visual outcome. CONCLUSIONS: The present large-scale cohort study revealed the clinicoepidemiologic features of noninfectious optic neuritis in Japan. Anti-aquaporin-4 antibody-positive optic neuritis has poor visual outcome. In contrast, MOG-Ab positive cases manifested severe clinical findings of optic neuritis before treatment, but few showed concurrent lesions in sites other than the optic nerve and generally showed good treatment response with favorable visual outcome. These findings indicate that autoantibody measurement is useful for prompt diagnosis and proper management of optic neuritis that tends to become refractory.

Early intervention using high-precision radiotherapy preserved visual function for five consecutive patients with optic nerve sheath meningioma.

BACKGROUND: There has been a paradigm shift in the treatment for optic nerve sheath meningioma (ONSM) from surgery to fractionated stereotactic radiotherapy (FSRT) in other countries. However, FSRT has seldom been performed in Japan. The purpose of this retrospective study is to reconfirm the effectiveness of early intervention with precision radiotherapy for ONSM reported in our previous study. METHODS: Five consecutive patients with ONSM were retrospectively analyzed. All patients underwent intensity-modulated radiotherapy (IMRT) or FSRT. They received the early interventions between 1.5 and 7 months after deterioration of the disease. The median dose was 52.8 Gy (range 46.0-59.4 Gy) and the median number of fractions was 25 (range 22-33). RESULTS: All patients experienced reestablishment of vision at the median follow-up time of 36 months (range 18-54 months). Four of them noted early improvement of visual deficits during the treatment course (range 2-4 weeks) and the remaining patient improved 3 weeks after completion of IMRT. The median tumor reduction was 53% (range 39-75%). One patient with diabetes mellitus developed retinal bleeding as a result of radiation retinopathy 16 months after IMRT, although the doses were acceptable. The remaining 4 patients have no late toxicity at the follow-up time of 31-54 months. CONCLUSIONS: A paradigm shift is necessary from surgery to early intervention using precision radiotherapy for the treatment of ONSM in Japan.

[A Case of a Corneal Disorder after Breast Cancer Treatment with Nab-paclitaxel].

BACKGROUND: We report a case of a corneal disorder after breast cancer treatment with a microtubule inhibitor, nab-paclitaxel (Abraxane). CASE: A 55-year-old woman, who was referred to our clinic, presented with blurred vision. 2 weeks previously, she had been undergoing treatment with nab-paclitaxel, trastuzumab and pertuzumab. Visual acuity was 20/60 in right eye and 20/40 in left eye. Slit-lamp examination showed an atypical epithelium invasion of the cornea in both eyes and small epithelial defect in left eye. After the nab-paclitaxel treatment was discontinued the corneal findings improved. 4 months later, visual acuity was 20/15 in right eye and 20/20 in left eye. CONCLUSIONS: Nab-paclitaxel can cause severe corneal disorders, but the possibility that trastuzumab and pertuzumab combination therapy is associated with these disorders cannot be denied. Clinicians should consider the possibility of the side effects caused by systemic administration in cases of corneal disorders of uncertain etiology.

[Clinical features of 19 cases of Fisher syndrome].

PURPOSE: To determine the clinical symptoms of Fisher syndrome (FS). METHODS: We performed a retrospective study of 19 patients who had been diagnosed as having FS. RESULTS: The study involved 19 patients (13 men and 6 women) whose mean age of onset was 42.0 years. Ophthalmoplegia included 16 cases of abducens nerve palsy, 2 cases of total external ophthalmoplegia, and 1 combined case of abducens nerve palsy and impaired eye elevation. Although all cases of abducens nerve palsy were bilateral, in 5 cases the degree of the affliction was asymmetric. Other neurologic symptoms included 5 cases of nystamus, 5 cases of pain on eye movement, 5 cases of pupillary defects, 6 cases of blepharoptosis, 2 cases of facial palsy, 9 cases of dysesthesia in the limbs, 3 cases of ataxia and 1 case of bulbar palsy. Anti-GQ1b antibodies were detected in 74% of the patients. Sixteen patients were followed without treatment while 1 patient, was given oral vitamin B12 and 2 patients received intravenous immunoglobulin. Double vision improved in all cases, and the mean duration of double vision was approximately 70 days. CONCLUSIONS: FS develops in people of all ages and is more common in men. Also, abducens nerve palsy is common in FS. While it is rare to observe the triad of ophthalmoplegia, ataxia and areflexia, the frequency in which the disorder is complicated by neurologic symptoms other than the triad is high. Double vision has a good prognosis and improves even without treatment.

[Designation criteria for Leber's hereditary optic neuropathy].

Designation criteria for Leber's hereditary optic neuropathy (LHON) have been established by a working group for retino-choroidal and optic atrophy funded by the Ministry of Health, Labor, and Welfare (MHLW) of Japan in collaboration with the Japanese Neuro-ophthalmology Society. The criteria are composed of three major symptoms and three ancillary test findings. According to the number and the combination of these symptoms and findings, subjects are classified into definite, probable, and possible LHON cases and asymptomatic carriers. The major symptoms include bilateral involvement with a time-lag, a papillomacular bundle atrophy, both characteristic optic disc findings at the acute phase. In the ancillary testings, mitochondrial DNA mutations specific for LHON are detailed with a table listing the mutation loci being attached. To enhance readers' understanding of description of the major symptoms and ancillary test findings, explanatory remarks on 11 parameters are supplemented. The establishment of the criteria facilitates epidemiological survey of LHON by MHLW and contributes to improvement of welfare for patients with LHON in Japan.

[National clinical investigation of anti-aquaporin-4 seropositive optic neuritis].

PURPOSE: We carried out a national clinical investigation of anti-aquaporin-4 seropositive optic neuritis which is resistant to steroid pulse therapy. METHODS: A questionnaire of clinical training institutions authorized by the Japanese Ophthalmological Society regarding anti-aquaporin-4 seropositive optic neuritis. RESULTS: We received responses from 44 facilities (40%). There were 188 anti-aquaporin-4 seropositive cases (24 men, 162 women), with a mean age of 52.4 +/- 15.5 (14-88 years old, median 54). Visual acuity at the time of the first medical examination was 3.4 to -0.18 logMAR (median 1.30) and at the last 3.4 to -0.18 logMAR (median 0.70). The average number of times of recurrence and steroid pulse therapy were 1.5 +/- 2.3 (median 1) and 3.3 +/- 4.1 (median 2) respectively. There was no significant difference in visual acuity between patients who received additional therapy such as plasma exchange therapy and immunoadsorption therapy and patients who were not treated additionally. CONCLUSIONS: Clinical characteristics of anti-aquaporin-4 seropositive optic neuritis were revealed. A well-designed prospective study is needed to obtain evidence of availability of plasma exchange therapy and other related issues.

Assessment of objective visual function following idebenone administration in patients with leber hereditary optic neuropathy.

PURPOSE: To objectively assess visual function in Leber's Hereditary Optic Neuropathy (LHON) patients; this study evaluated pre- and post-idebenone treatment changes in primary visual cortical (V1) responses using functional magnetic resonance imaging (fMRI), given the challenges in subjective testing due to central retinal ganglion cell damage. STUDY DESIGN: A descriptive study involving four confirmed LHON patients. METHODS: Four patients received 900 mg/day of oral idebenone for 24 weeks. Baseline and post-treatment visual acuity, visual fields, and BOLD fMRI responses while passively viewed drifting contrast pattern visual stimuli were compared with self-reported symptoms. RESULTS: Post-idebenone, one patient showed positive trends across subjective tests, reported symptoms, and fMRI. Two patients had stable symptoms and fMRI responses; one improved on subjective tests, and another worsened slightly. Another patient improved in visual field tests despite worsening symptoms and fMRI trends. CONCLUSION: fMRI may offer a valuable objective measure of visual functions in LHON and appears to be more relevant in assessing symptoms. Further research with more participants is needed to ascertain fMRI's role in developing objective visual assessments and treatment evaluation.

Phosphorylation of ezrin/radixin/moesin (ERM) protein in spinal microglia following peripheral nerve injury and lysophosphatidic acid administration.

Peripheral nerve injury activates spinal glial cells, which may contribute to the development of pain behavioral hypersensitivity. There is growing evidence that activated microglia show dynamic changes in cell morphology; however, the molecular mechanisms that underlie the modification of the membrane and cytoskeleton of microglia are not known. Here, we investigated the phosphorylation of ezrin, radixin, and moesin (ERM) proteins in the spinal cord after peripheral nerve injury. ERM is known to function as membrane-cytoskeletal linkers and be localized at filopodia- and microvilli-like structures. ERM proteins must be phosphorylated at a specific C-terminal threonine residue to be in the active state. The nature of ERM proteins in the spinal cord of animals in a neuropathic pain model has not been investigated and characterized. In the present study, we observed an increase in the phosphorylated ERM in the spinal microglia following spared nerve injury. The intrathecal administration of lysophosphatidic acid induced the phosphorylation of ERM proteins in microglia along with the development of mechanical pain hypersensitivity. Intrathecal administration of ERM antisense locked nucleic acid suppressed nerve injury-induced tactile allodynia and decreased the phosphorylation of ERM, but not the Iba1 staining pattern, in spinal glial cells. These findings suggest that lysophosphatidic acid induced the phosphorylation of ERM proteins in spinal microglia and may be involved in the emergence of neuropathic pain. These findings may underlie the pathological mechanisms of nerve injury-induced neuropathic pain.

Ischemic stroke brain sends indirect cell death signals to the heart.

BACKGROUND AND PURPOSE: Ischemic stroke is a leading cause of mortality and morbidity in the world and may be associated with cardiac myocyte vulnerability. However, it remains uncertain how an ischemic brain contributes to cardiac alternations. Here, we used experimental stroke models to reveal the pathological effects of the ischemic brain on the heart. METHODS: For the in vitro study, primary rat neuronal cells were subjected to 90-minute oxygen-glucose deprivation (OGD). Two hours after OGD, the supernatant was collected and cryopreserved until further biological assays. Primary rat cardiac myocytes were exposed to ischemic-reperfusion injury and subsequently to the supernatant derived from either the OGD or non-OGD-exposed primary rat neuronal cells for 2, 6, 24, or 48 hours. Thereafter, we measured cell viability and mitochondrial activity in rat cardiac myocytes. For the in vivo study, we subjected adult rats to transient middle cerebral artery occlusion, and their brains and hearts were harvested for immunohistochemical analyses at 3 months later. RESULTS: The supernatant from the OGD, but not the non-OGD-exposed primary rat neuronal cells, caused significant reduction in cell viability and mitochondrial activity in rat cardiac myocytes. Ischemic stroke animals displayed phenotypic expression of necrosis, apoptosis, and autophagy in their hearts, which paralleled the detection of these same cell death markers in their brains. CONCLUSIONS: Ischemic stroke was accompanied by cardiac myocyte death, indicating a close pathological link between brain and heart. These results suggest a vigilant assessment of the heart condition in stroke patients, likely requiring the need to treat systemic cardiac symptoms after an ischemic brain episode.

Vasculogenesis in experimental stroke after human cerebral endothelial cell transplantation.

BACKGROUND AND PURPOSE: Despite the reported functional recovery in transplanted stroke models and patients, the mechanism of action underlying stem cell therapy remains not well understood. Here, we examined the role of stem cell-mediated vascular repair in stroke. METHODS: Adult rats were exposed to transient occlusion of the middle cerebral artery and 3 hours later randomly stereotaxically transplantated with 100K, 200K, or 400K human cerebral endothelial cell 6 viable cells or vehicle. Animals underwent neurological examination and motor test up to day 7 after transplantation then euthanized for immunostaining against neuronal, vascular, and specific human antigens. A parallel in vitro study cocultured rat primary neuronal cells with human cerebral endothelial cell 6 under oxygen-glucose deprivation and treated with vascular endothelial growth factor (VEGF) and anti-VEGF. RESULTS: Stroke animals that received vehicle infusion displayed typical occlusion of the middle cerebral artery-induced behavioral impairments that were dose-dependently reduced in transplanted stroke animals at days 3 and 7 after transplantation and accompanied by increased expression of host neuronal and vascular markers adjacent to the transplanted cells. Some transplanted cells showed a microvascular phenotype and juxtaposed to the host vasculature. Infarct volume in transplanted stroke animals was significantly smaller than vehicle-infused stroke animals. Moreover, rat neurons cocultured with human cerebral endothelial cell 6 or treated with VEGF exhibited significantly less oxygen-glucose deprivation-induced cell death that was blocked by anti-VEGF treatment. CONCLUSIONS: We found attenuation of behavioral and histological deficits coupled with robust vasculogenesis and neurogenesis in endothelial cell-transplanted stroke animals, suggesting that targeting vascular repair sets in motion a regenerative process in experimental stroke possibly via the VEGF pathway.

A case with transient refractive change after removal of pituitary tumor.

BACKGROUND: Refractive change can be caused by systemic illnesses such as Lupus erythematosus, thyroid deficiency, and diabetes mellitus. However, refractive change after pituitary tumor removal has so far not been reported. CASE PRESENTATION: A 62-year-old woman presented with blurred near vision 10 days after trans-sphenoidal surgery (TSS) for a pituitary tumor. Around the same time, she experienced intercurrent hyponatremia. The corrected visual acuity of both eyes was 20/20, the spherical equivalent of the right eye was -2.125 diopters, and of the left eye was -2.0 diopters before TSS. However, 11 days after TSS, the spherical equivalent of the right eye changed to -0.75 diopters, and that of left eye changed to -1.125 diopters without hyperglycemia. There were no changes in the corrected visual acuity during the follow-up. CONCLUSION: We demonstrated a case with transient refractive change after TSS. The following mechanism is proposed: Hyponatremia induced by the pituitary tumor removal causes an osmotic change in the aqueous humor with lens swelling. This case report is a reminder to both ophthalmologists and neurosurgeons that ophthalmological factors such as lens thickness and axial length should be taken into account when conducting preoperative examinations especially for patients undergoing TSS.

[Viewing 3D stereoscopic images in children and adults with and without strabismus: multicenter study in Japan].

PURPOSE: To investigate the ability of patients with strabismus and/or amblyopia to see 3D images. METHODS: A questionnaire survey conducted for children aged 6 to 19 years and adults aged 20 to 39 years on their experience of viewing 3D images (movies, motion attractions, television, games), asking whether they could see stereoscopically, with or without adverse effects. A retrospective investigation of ophthalmological examinations was followed. RESULTS: Of 507 cases, 342 had had the experience of veiwing 3D images. In 212 (62%) cases of strabismus and/or amblyopia, stereopsis was lacking in 17 to 18% of the subjects for movies, in 6 to 7% for attractions, in 32% in children and 50% in adults for television and 23% in children and 17% in adults for games. Adults complained of a higher rate of adverse effects, 65% for movies and 75% for games, as compared with 34% for movies and 26% for games in children(p < 0.01). The lack of stereopsis for games and movies was higher in the subgroup of Fly (-) and convergence insufficiency (p < 0.05). CONCLUSIONS: Since many patients with strabismus and/or amblyopia found some difficulty in stereoscopic viewing, precise examinations for stereopsis and convergence are needed to assess individual aptitude for 3D viewing.

A Case of Bilateral Papilledema With Improved Clinical Symptoms by Venous Stenting for Superior Sagittal Sinus Stenosis.

Superior sagittal sinus (SSS) obstruction causes intracranial hypertension, often requiring surgical stenting. Consensus on treating brain venous sinus stenosis, another cause, is lacking. This study reports a case of SSS stenosis and intracranial hypertension treated with venous stenting, improving bilateral papilledema. A 51-year-old with a headache and visual disturbance had papilledema and visual field loss. MR venography showed SSS stenosis, leading to a neurosurgery referral. Lumbar puncture confirmed intracranial hypertension (>35 cmH2O), prompting venous stenting. Post-procedure, papilledema, headache, and visual field loss improved. Venous stenting could be effective for SSS stenosis with clinically proven or recurrent pressure differences. Further cases are needed for standardization.

Contribution of IL-33 to induction and augmentation of experimental allergic conjunctivitis.

IL-33, a member of the IL-1 family of cytokines, is the ligand for ST2 (IL-33Ralpha chain). IL-33 has the capacity to induce T(h)2 cytokine production from T(h)2 cells, mast cells and basophils, indicating that IL-33 has the potential to induce T(h)2 cytokine-mediated allergic inflammation of the eye. Thus, we tested the pathological role of IL-33 in allergic conjunctivitis (AC). As reported elsewhere, animals immunized with ragweed pollen (RW)/alum and boosted with RW/PBS developed AC promptly (within 15 min) and conjunctival eosinophilic inflammation after a delay (within 24 h) in response to eye drop challenge with RW. Furthermore, RW-immunized mice, when topically challenged with both RW and IL-33, developed more striking eosinophilia in their conjunctiva without exacerbation of the clinical AC score. This in vivo IL-33 treatment significantly increased the capacity of T cells in the cervical lymph nodes of RW-immunized mice to produce IL-4, IL-5 and IL-13 upon challenge with anti-CD3 and anti-CD28 antibodies in vitro. Furthermore, the infiltrating cells were largely eosinophils and a small proportion of CD4(+) T cells, both of which express ST2. We also found that even splenic eosinophils express ST2 and show increased expression in response to IL-5, granulocyte-macrophage colony-stimulating factor (GM-CSF) or IL-33. Eosinophils, stimulated with IL-5 and/or GM-CSF, are responsive to IL-33, which induces production of IL-4 and chemokines. Finally, we showed that conjunctival tissues constitutively express biologically active IL-33, suggesting that IL-33 might play a crucial role in the induction and augmentation of AC.

[A case of idiopathic orbital inflammatory disease with variable ocular manifestations].

PURPOSE: We present a case of idiopathic orbital inflammatory disease with dilatation and tortuosity of the retinal veins. CASE: A 74-year-old man presented at his local eye hospital with left conjunctival edema and pain. He was referred to our hospital. At the initial examination, the intraocular pressure was 19 mmHg OD and 40 mmHg OS. Examination of the left eye revealed conjunctival edema with dilated and tortuous blood vessels, and dilatation and tortuousity of the retinal veins. Magnetic resonance imaging showed marked thickening of the left extraocular muscles, and suspected compression of the left superior and inferior ophthalmic veins. We diagnosed left idiopathic orbital inflammatory disease, and administered a tapering course of prednisolone starting at 40 mg daily. Thirteen days later, the conjunctival findings improved, but the left fundus showed signs of non-ischaemic central retinal vein occlusion. Subsequently, the dilatation and tortuousity of the retinal veins gradually improved. At the final examination, recurrence was not noted. CONCLUSION: We report a case of idiopathic orbital inflammatory diseases with central retinal vein occlusion. The dilatation and tortuousity of the conjunctiva and central retinal vein occlusion improved with steroid therapy only. These Conditions improved because the inflammatory swelling of the extraocular muscle disappeared and the pressure to the vein was relieved.

Intravenous immunoglobulin treatment for steroid-resistant optic neuritis: a multicenter, double-blind, randomized, controlled phase III study.

PURPOSE: To evaluate the efficacy and safety of intravenous "freeze-dried sulfonated human normal immunoglobulin (GGS)" in patients with steroid-resistant optic neuritis (ON). STUDY DESIGN: Multicenter, prospective, double-blind, parallel-group, randomized controlled trial. METHODS: Patients with steroid-resistant acute ON were randomly assigned to receive either intravenous GGS (GGS group) or intravenous methylprednisolone (steroid pulse [SP] group). Visual acuity (logarithm of the minimum angle of resolution [logMAR]), mean deviation (MD) value of the Humphrey Field Analyzer, and critical flicker fusion frequency were measured as efficacy endpoints; adverse events (AEs) were assessed as the safety endpoint. RESULTS: Thirty-two patients (16 patients/group) received the study drugs. The primary endpoint, change in logMAR at week 2 compared to baseline, showed no statistically significant intergroup difference. However, compared with the SP group, change in the GGS group was increasingly indicative of visual improvement, with least squares mean difference of > 0.3 logMAR. On post-hoc analyses, the percentage of patients in the GGS and SP groups with improvement by ≥ 0.3 logMAR at week 2 were 75.0% and 31.3%, respectively. Changes in MD values at week 2 compared to baseline were 9.258  ±  8.296 (mean ± standard deviation) dB and 3.175  ±  6.167 dB in the GGS and SP groups, respectively. These results showed statistically significant intergroup differences (visual acuity improvement, P = 0.032; change in MD values, P = 0.030). No clinically significant AEs were observed. CONCLUSION: Our results suggest that intravenous immunoglobulin could be a safe and efficacious therapeutic option for prompt treatment of steroid-resistant acute ON. TRIAL REGISTRATION: JapicCTI-132080.

Characteristics of Japanese patients with Leber's hereditary optic neuropathy and idebenone trial: a prospective, interventional, non-comparative study.

PURPOSE: Leber's hereditary optic neuropathy (LHON) is a mitochondrial neuropathy that causes acute vision loss. Idebenone, a short-chain ubiquinone analog that preserves mitochondrial function is thought to suppress disease progression in early-onset LHON patients. We investigated the effects of idebenone in Japanese LHON patients. STUDY DESIGN: Prospective, interventional, non-comparative study in patients with definite LHON diagnosis, under trial registration number UMIN000017939. METHODS: Fifty-seven patients received 900 mg/day idebenone for 24 weeks. We measured baseline best-corrected visual acuity, visual fields, critical fusion frequency and retinal ganglion cell layer complex thickness; we assessed efficacy at 24 and 48 weeks, and safety throughout. RESULTS: Patients were predominantly male (91.2%) and most had an mt.11778G>A mutation (94.7%). All patients tolerated idebenone therapy well. Data from the 51 mt.11778 patients were compared with their baseline data. At 48 weeks, significant improvement in best-corrected visual acuity was observed in 17 patients (33.3%). Furthermore, 25.5% of patients showed improvements in visual fields and 33.3% in critical fusion frequency. However, retinal ganglion cell layer complex thickness was significantly reduced. Among patients who started idebenone >1 year after disease onset, visual improvement was found in 12 (38.7%). Among patients who developed LHON before 19 years of age, visual improvement was found in 11 (42.3%). CONCLUSION: Idebenone's potential and favorable safety profile were confirmed in Japanese LHON patients. However, this study had no placebo group; therefore, we need to undertake a prospective intervention study to further investigate the therapeutic effects of Idebenone in Japanese LHON patients.

[Clinico-statistical study on availability of Esterman disability score for assessment of mobility difficulty in patients with visual field loss].

OBJECTIVE: The visual efficiency scale currently adopted to determine the legal grade of visual disability associated with visual field loss in Japan is not appropriate for the evaluation of disability regarding daily living activities. We investigated whether Esterman disability score (EDS) is suitable for the assessment of mobility difficulty in patients with visual field loss. METHODS: The correlation between the EDS calculated from Goldmann's kinetic visual field and the degree of subjective mobility difficulty determined by a questionnaire was investigated in 164 patients with visual field loss. The correlation between the EDS determined using a program built into the Humphrey field analyzer and that calculated from Goldmann's kinetic visual field was also investigated. RESULTS: The EDS based on the kinetic visual field was correlated well with the degree of subjective mobility difficulty, and the EDS measured using the Humphrey field analyzer could be estimated from the kinetic visual field-based EDS. CONCLUSION: Instead of the currently adopted visual efficiency scale, EDS should be employed for the assessment of mobility difficulty in patients with visual field loss, also to establish new judgment criteria concerning the visual field.

[A case of acute lymphoblastic leukemia with variable appearance].

BACKGROUND: A case of acute lymphoblastic leukemia in which a variety of ophthalmic findings were observed. CASE: A 27-year-old woman with acute lymphoblastic leukemia. Ocular findings were Roth's spot and serous retinal detachment. Fluorescein angiography revealed no clear leak point. Subsequently, the serous retinal detachment disappeared. Within 4 months after the initial diagnosis, cytomegalovirus (CMV) retinitis occurred, and further keratoconjunctivitis and serous retinal detachment recurred due to Graft-versus-Host Disease (GVHD), thus resulting in death 4 months later. CONCLUSIONS: Cases in which patients with leukemia complicated by serous retinal detachment during the course often result in death. In the present case, CMV retinitis and keratoconjunctivitis occurred due to GVHD after serous retinal detachment, thus resulting in death 8 months after the initial diagnosis, therefore if serous retinal detachment is observed in a leukemia patient, it is necessary to perform periodical ophthalmologic examinations even after the ocular symptoms disappear.