Blindness due to leptomeningeal carcinomatosis as an initial manifestation of recurrent acute lymphoblastic leukemia.

Leptomeningeal carcinomatosis (LC) is an uncommon presentation of acute lymphoblastic leukemia (ALL), and it is a devastating and life-threatening complication. The disease affects all levels of the central nervous system, and most patients present with different multifocal neurological symptoms. This case was a 34-year-old male who had acute bilateral blindness secondary to recurrent ALL with meningeal infiltration. Diagnosis of LC is made based on the clinical symptoms and the test results including cranial and spinal magnetic resonance imaging and cerebrospinal fluid (CSF) survey. The differential diagnosis of meningeal enhancement and early treatment are also important for prognosis. This case had a good visual recovery after treatment.

Clinical Course and Treatment Response of Neuromyelitis Optica Spectrum Disease: An 8-Year Experience.

PURPOSE: To report the clinical course and treatment response in a cohort of patients with neuromyelitis optica spectrum disorder (NMOSD) from a single referral center in Taiwan. DESIGN: Case series. METHODS: All NMOSD patients who received treatment and follow-up checks at National Taiwan University Hospital for at least 2 years between January 2008 and December 2016 were recruited. Demographic data and detailed characteristics of clinical course including acute episodes, maintenance therapies, and visual outcome were collected. Patients were examined every 6 months and during each episode of relapse. RESULTS: A total of 96 patients were identified; of them, 68 (70.8%) had optic neuritis. Optic neuritis was the initial presentation of NMOSD in 44 patients. Among the patients with optic neuritis, 32.4% had recurrent optic neuritis within 1 year from the first episode. Compared with the group without optic neuritis, the group with optic neuritis had a younger age of presentation (34.4 ± 15.9 vs 42.4 ± 14.8 years, P = 0.02) and a higher 1-year recurrence rate (64.7% vs 17.9%, P < 0.001). Among the patients with optic neuritis, 51.5% had a final visual acuity of worse than 20/200. Multiple regression analysis showed that the presence of aquaporin-4-IgG antibodies and poor initial visual acuity were the risk factors for worse visual outcome. CONCLUSIONS: In this study, patients with optic neuritis were younger and had a higher 1-year recurrence rate. Despite using steroid therapy during the acute phase and immunosuppressive agents as long-term treatment, visual prognosis was poor. Aquaporin-4-IgG antibodies and initial visual acuity were associated with final visual outcome.

Update on ocular myasthenia gravis in Taiwan.

Myasthenia gravis (MG) is an autoimmune disease involving the neuromuscular junction. Autoantibodies to the acetylcholine receptor or, less frequently, to muscle-specific kinase, attack against the postsynaptic junctional proteins, resulting in fluctuating and variable weakness of muscles. Extraocular, levator palpebrae superioris, and orbicularis oculi muscles are particularly susceptible. The majority of patients with MG present with purely ocular symptoms including ptosis and diplopia initially. About half of these patients progress to generalized disease within 2 years. The prevalence of MG in Taiwan is 140 per million with male to female ratio of 0.7. The incidence rate is higher in the elderly. Several immune-related diseases such as lymphoid malignancy, diabetes, and thyroid diseases are associated with MG in the national population-based studies in Taiwan. Ice pack test, rest test, Tensilon/neostigmine test, circulating antibody measurement, and electrophysiological studies are useful diagnostic tools with variable sensitivity and specificity. For the patients with ocular MG, acetylcholinesterase inhibitors are usually the first-line treatment. Corticosteroids and immunosuppressant could provide better disease control and may reduce the risk of conversion to generalized form although there is still some controversy. A thymectomy is also beneficial for ocular MG, especially in refractory cases. The correction of ptosis and strabismus surgery could improve the visual outcome but should be performed only in stable disease.

Bilateral Acute Proptosis as Initial Manifestation of Acute Myeloid Leukemia.

The myeloid sarcoma is an extramedullary finding of acute myeloid leukemia (AML), and orbital leukemic tumors occur most commonly during the first decade of life. To our knowledge, we report the youngest patient with bilateral proptosis of both eyes as an initial manifestation of AML. This case highlights the need for peripheral blood smear and neuro-image work-up for acute proptosis in infancy. AML should be considered in the differential diagnosis of an orbital mass, even in the absence of typical leukemic symptoms.

Bilateral optic neuritis related to chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a condition that mainly affects the peripheral nervous system; however, the central nervous system has also been involved in rare cases. Herein, we describe the case of a 33-year-old man with CIDP who presented with progressively blurred vision and pain with eye movement in both eyes for 1 month. Ocular examination revealed reduced visual acuities of 0.15 (oculus unitas or OU) and unremarkable fundi (OU). Furthermore, bitemporal visual field defects and prolonged visually evoked potentials were evident. Brain magnetic resonance imaging revealed nothing remarkable along the optic nerve and chiasm. These findings were compatible with the diagnosis of bilateral optic neuritis. The patient's symptoms and visual acuity improved after 5 days of intravenous (IV) corticosteroid pulse therapy, which was subsequently replaced by oral prednisolone therapy with a tapering schedule. The patient's visual acuity returned to 1.0 (OU) 6 months after treatment. However, bilateral optic neuritis recurred in 7 months while the patient was on oral prednisolone and azathioprine. IV corticosteroid pulse therapy was subsequently reinitiated and the patient's visual acuity returned gradually to 1.0 (OU). Bilateral optic neuritis is a rare manifestation of CIDP. It responded well to IV corticosteroid therapy in our case.

Focal foveal atrophy of unknown etiology: clinical pictures and possible underlying causes.

BACKGROUND/PURPOSE: Focal foveal atrophy is defined as the presence of a small, focal, ill-defined, hypopigmented foveal or juxtafoveal lesion, with the remaining retina unaffected. The purpose of this study was to report the clinical characteristics and optical coherence tomography (OCT) in patients with focal foveal atrophy of unknown etiology. METHODS: The study was a retrospective observational case series. Data collected included complete ocular examination results for best corrected visual acuity (BCVA), ophthalmoscopy, fundus photography, fluorescein angiography, color sense discrimination tests, visual field tests, and OCT examinations. RESULTS: Twenty-three eyes in 21 patients were examined. The mean patient age was 49.2 ± 15.4 years. The mean BCVA was 20/25. The 21 patients were divided into three groups according to OCT results. Group 1 eyes (n = 10) had intact inner and outer hyperreflective layers (HRLs), with the signal of the inner HRL corresponding to the junction between the inner and outer photoreceptor segments and the outer HRL corresponding to the retinal pigment epithelium (RPE). Group 2 eyes (n = 9) had small hyporeflective defects with defects in the inner HRL at the fovea but an intact outer HRL. Group 3 eyes (n = 4) had small hyporeflective defects in both the inner and outer HRLs at the fovea. Groups 3 eyes had significantly lower visual acuity compared to Group 1 eyes and Group 2 eyes. There was no significant difference in visual acuity between Group 1 and Group 2 eyes. There were no significant differences among the groups with respect to color vision or foveal thickness. CONCLUSION: This is the first report of clinical presentations for patients with focal foveal atrophy of unknown etiology. OCT aided in the diagnosis and assessment of the degree of retinal structural abnormalities, but the real etiology of foveal atrophy remains unclear.

Clinical characteristics and visual function tests with retinal tomographic correlation in patients with Stargardt's disease in Taiwan.

BACKGROUND/PURPOSE: Stargardt's disease is the most common form of juvenile macular dystrophy. The purpose of this study is to report the clinical characteristics, visual function, and retinal tomography of patients diagnosed with Stargardt's disease in Taiwan. METHODS: Retrospective case series; data collected include results of complete ophthalmic examinations: best-corrected visual acuity (BCVA), slit-lamp biomicroscopy, indirect ophthalmoscopy, fundus photography, fluorescein angiography, color sense discrimination test, electroculography (EOG), electroretinography (ERG), dark adaptation, visual field test, and optical coherence tomography (OCT) examinations focused on the macular area. RESULTS: Twenty patients were enrolled in this study. The mean age of the patients was 32.1 years. The mean logMAR-converted visual acuity of all patients was 0.96. Maculopathy with or without flecks was evident in all patients. Fluorescein angiography revealed hyperfluorescence in the macula of all cases. Varying degrees of color sense discrimination dysfunction were noted. Electrophysiologic studies showed subnormal to abnormal responses in most patients. Decreased foveal thickness was evident on OCT and foveal thickness was inversely associated with logMAR visual acuity. Also, logMAR visual acuity was associated with total error scores on the color sense discrimination test and the Arden ratio of EOG. The Arden ratio of EOG correlated with the thickness of the outer ring of the macula. CONCLUSION: Typical clinical presentations of Stargardt's disease were demonstrated in our case series. OCT findings showed a correlation between foveal thickness and visual acuity, making OCT a useful predictor for other visual function tests. Our results indicate that OCT is helpful in the evaluation of visual acuity and the progression of maculopathy in patients with Stargardt's disease.

Clinical prospective study of visual function in patients with acute optic neuritis.

BACKGROUND/PURPOSE: To investigate the clinical features and visual outcomes of acute optic neuritis in adult patients. METHODS: We prospectively collected ninety-nine adult patients, who were found to have acute optic neuritis between 2005 and 2007 at National Taiwan University Hospital. A total of 30 cases, aged ranging from 21 to 55 years old (average 36.4 ± 9.9), that followed up at least 6 months were enrolled in our study. Baseline clinical features and visual function results were analyzed. RESULTS: The mean follow-up period was 15.6 months. Twenty three (76.7%) cases were female. Twenty-seven cases were unilateral involved, one was simultaneously bilateral involved and two was sequentially bilateral involved. In total 33 affected eyes, ocular or periocular pain was noted in 14 eyes (69.7%). Optic disc swelling was noted in 5 eyes (15.2%). A total of 6 cases had recurrent episodes, and two of them were diagnosed with multiple sclerosis thereafter. At 6-month follow up, 24 eyes (72.7%) had good visual recovery (better than 20/40). Only 2 eyes (6%) had severe visual loss (<20/1000). Optic disc pale was detected in 72.7% of the eyes during follow up. CONCLUSION: Visual recovery was observed in most eyes with acute optic neuritis, although disc pale detectable. Patients with recurrent optic neuritis had worse visual outcome. There was a low association of optic neuritis with multiple sclerosis in our patients.

A Comparison of Optic Neuritis in Asian and in Western Countries.

Acute demyelinating optic neuritis is a common optic neuropathy in young adults. There is usually satisfactory visual recovery. However, some patients convert to multiple sclerosis (MS) with potential sequelae of neurological disability. The Optic Neuritis Treatment Trial in the United States was conducted prospectively for 15 years and provided valuable data about clinical course, efficacy of steroid treatment, and risk of conversion to MS. Compared to the many studies in Western countries, research concerning optic neuritis in Asia has so far not been extensive. However, cumulative evidence shows that various features of this disorder differ between patients of Caucasian and Oriental descent. In this article we review up-to-date studies on optic neuritis in Asia and compare the results with prior literature. Prospective and multi-centre studies are currently underway in these regions to increase our understanding of optic neuritis in Asia.

The effect of interferon beta-1a on optic neuritis relapse in patients with multiple sclerosis.

BACKGROUND: To evaluate the clinical effect of interferon beta-1a on optic neuritis (ON) relapse in patients with multiple sclerosis (MS) in Taiwan. METHODS: Data were collected from 23 MS patients with ON at National Taiwan University Hospital between January 1, 1993 and February 1, 2007. Twenty-three MS patients with ON received interferon beta-1a (Rebif) 44 microg via subcutaneous injection three times weekly. All patients received corticosteroids pulse therapy followed by oral prednisolone for acute ON. The annual relapse rate (ARR) of ON in these MS patients before and after the use of interferon beta-1a (Rebif) was the main clinical parameter of outcome in this study. RESULTS: The ARR of ON was lower in the posttreatment period than in the pretreatment period (P = 0.0068). Thirteen patients (56.5%) had improved final visual acuity (>2 lines), and the other ten patients (43.5%) had stable final visual outcome (-2 lines < X < 2 lines). In addition, no recurrence of ON was noted in 15 patients (65.2%) during the posttreatment period. CONCLUSIONS: The use of interferon beta-1a 44 microg via subcutaneous injection three times weekly did not increase the ON attacks in MS patients receiving this treatment. In addition, beneficial effects were found with the use of interferon beta-1a on these patients.

Anterior ischemic optic neuropathy associated with pegylated interferon therapy for chronic hepatitis C.

PURPOSE: To report a case of anterior ischemic optic neuropathy (AION) associated with the combined therapy of pegylated interferon alpha (PEG-IFNalpha) and ribavirin. METHODS: Case report. RESULTS: A 57-year-old male with chronic hepatitis C, treated with PEG-IFNalpha -2b and ribavirin for 6 months, noted sudden onset of painless blurred vision in his right eye. The initial visual acuity decreased to 20/60 in the right eye. Fundoscopy of right eye revealed optic disc edema with disc hemorrhage. Visual field examination disclosed inferior altitudinal defect. Relative afferent pupillary defect with poor color vision function was also noted in the right eye. Visual evoked potential demonstrated decreased amplitude in the right eye. A marked resolution of disc edema and improvement of visual acuity to 20/20 were noted at 6 weeks follow-up. CONCLUSIONS: AION may occur in combined treatment of PEG-IFNalpha -2b and ribavirin for chronic hepatitis C. Patients who are candidates for this treatment should be informed about its possible occurrence.

Uveitis-glaucoma-hyphema syndrome caused by posterior chamber intraocular lens--a rare complication in pediatric cataract surgery.

We report a case of postoperative uveitis-glaucome-hyphema (UGH) syndrome following pediatric cataract surgery due to posterior chamber intraocular lens (PC-IOL). Slit-lamp examination revealed the optic of PC-IOL migrated into anterior chamber. The PC-IOL explantation was performed and ocular inflammation subsided.

Optic neuritis among National Health Insurance enrollees in Taiwan, 2000-2004.

BACKGROUND: The objective was to examine the incidence of optic neuritis in Taiwan. A 5-year longitudinal, nationwide, population-based study was conducted. The participants were a random sample of 191,761 subjects, or about 1% of the enrollees in the National Health Insurance program of Taiwan. METHODS: We identified subjects who had at least 1 service claim for ambulatory or inpatient care with a principal diagnosis of optic neuritis between 2000 and 2004. We calculated and statistically analyzed the rates of optic neuritis. The main outcome measures were the incidence rates of optic neuritis. RESULTS: From 2000 to 2004, the total cumulative incidence was 1.33 per 1,000 (1.00 vs. 1.67 for male vs. female subjects; p < 0.001, chi(2) test). The cumulative incidence rates for the age groups of 0-19, 20-39, 40-59 and >or=60 years were 0.33, 1.05, 2.16 and 2.81 cases per 1,000, respectively. Among the subjects aged 40-59 years, the cumulative incidence was higher in women than in men (p < 0.001, chi(2) test). The incidence density of developing multiple sclerosis after optic neuritis was 0.41% per person-year, and the cumulative incidence of multiple sclerosis after a new diagnosis of optic neuritis was 0.78%. CONCLUSIONS: We believe our study is the first nationwide investigation of the incidence rates of optic neuritis in Taiwan. In our investigation, the cumulative incidence peaked among the subjects aged 40-59 years, especially in women. In Taiwan, patients with optic neuritis have a notably low rate of conversion to multiple sclerosis.

Optic neuritis secondary to Angiostrongylus cantonensis infection.

PURPOSE: To describe a case of optic neuritis secondary to Angiostrongylus cantonensis infection. METHOD: Case report of a patient with eosinophilic meningitis secondary to A. cantonensis infection. The patient developed a sudden decrease in visual acuity with a visual field defect and color vision loss in his left eye. Both visual evoked potential and clinical manifestations suggested the diagnosis of optic neuritis (os). RESULT: After two weeks of larvicidal drugs and steroid treatment, the patient's visual acuity and color vision had dramatically improved. CONCLUSION: Optic neuritis may occur in patients with A. cantonensis meningitis. Treatment with a combination of steroid and larvicidal agents may be beneficial to these patients.