An-Guor Wang

Leber's hereditary optic neuropathy: a multifactorial disease.

Lebers hereditary optic neuropathy (LHON) is a maternally transmitted disease characterized by acute or subacute visual loss predominantly affecting young men. The majority of LHON cases are caused by one of the three primary mitochondrial DNA (mtDNA) mutations: G3460A/ND1, G11778A/ND4, or T14484C/ND6. Although the primary etiological factor of LHON is a mtDNA mutation, the presence of a primary mtDNA mutation does not necessarily lead to visual loss. The pathogenesis of LHON remains unclear. The marked incomplete penetrance and gender bias indicate that additional genetic (nuclear or mitochondrial) and epigenetic factors may also be involved. Deficiency in respiratory chain function and reactive oxygen species (ROS) are believed to play a pivotal role in the pathophysiology of the disease.

Systemic lupus erythematosus-associated optic neuritis: clinical experience and literature review.

Purpose:  This study aimed to evaluate the visual outcome of optic neuritis in patients with systemic lupus erythematosus (SLE).

Pathogenesis and neuroprotective treatment in Purtscher's retinopathy

Purtschers retinopathy is characterized by sudden visual loss in severely traumatized patients and is associated with multiple areas of superficial retinal whitening located primarily in the posterior pole. Visual outcome in Purtschers retinopathy is variable, and there is no well-defined treatment. We report on a patient with immediate blurred vision in the right eye after a traffic accident. Ophthalmoscopy revealed multiple whitish patches scattered over the macular and peripapillary areas in the right eye. Fluorescein angiography showed multifocal retinal arteriolar occlusion in the early phase and staining of the involved retinal vessels and optic nerve head in the late phase. Indocyanine green angiography (ICG) showed rarefaction of choroidal vessels in the peripapillary area of the right eye at early phase. The late phase ICG study revealed multifocal hypofluorescent patches in the macular and peripapillary areas. Megadose steroid therapy was given with good visual response in the first 2 weeks, and the patients vision had recovered completely when followed-up 10 months later.

Heparin-surface-modified intraocular lens implantation in patients with glaucoma, diabetes, or uveitis

Abstract We investigated the clinical outcome in two groups of patients who had an extracapsular cataract extraction and implantation of a heparin‐surface‐modified intraocular lens (HSM IOL) (Group 1) or a conventional poly(methyl methacrylate) (PMMA) lens (Group 2). Nineteen patients in Group 1 had bilateral cataract extraction with implantation of an HSM IOL in one eye and a conventional lens in the fellow eye. All patients had glaucoma, diabetes, or uveitis. Over the long term, there was no statistically significant difference between groups in visual acuity, corneal edema, anterior chamber reaction, and amount of posterior synechia formation and IOL deposits. Yet short‐term clinical evaluation revealed significantly less reaction in eyes with the HSM IOL than in those with the PMMA lens. In patients with both lens types implanted, early postoperative anterior chamber reaction was less and IOL deposits fewer in the eye with the HSM IOL.

Preseptal and Orbital Cellulitis: A 10-Year Review of Hospitalized Patients

Background: Preseptal and orbital cellulitis range in severity from minor to potentially severe complications. The purpose of this study is to describe the clinical features of patients with preseptal or orbital cellulitis in one medical center in Taiwan, and to assess the effectiveness of treatments and the complications. Methods: Patients admitted between 1996 and 2005 to Taipei Veterans General Hospital under the diagnosis of preseptal or orbital cellulitis were retrospectively reviewed. The demographics, administrative history, past history, clinical presentations, treatments, and complications were analyzed. Results: In total, 94 patients fulfilling the diagnostic criteria for preseptal or orbital cellulitis were identified (67 had pre‐septal cellulitis, 27 had orbital cellulitis). While paranasal sinus disease was the most common predisposing cause in orbital cases, skin lesions in children and dacryocystitis in adults were the most common in preseptal cases. Microbiologic investigations showed variable results, but the most common pathogen isolated was Staphylococcus aureus. Cultures from eye swabs and local abscesses gave the highest positive yield. Blood cultures were taken in some patients, but the positive rate was extremely low. Treatments included intravenous antibiotics alone, or intravenous antibiotics combined with surgical drainage. Only one case had permanent ocular motility impairment after removal of the orbital foreign body. Conclusion: Despite the past history of potential morbidity and even mortality from orbital cellulitis, early diagnosis and prompt treatment with proper antibiotics and/or surgical intervention can achieve a good prognosis.

Leber's Hereditary Optic Neuropathy—The Spectrum of Mitochondrial DNA Mutations in Chinese Patients

PURPOSE To investigate the spectrum of mitochondrial DNA (mtDNA) mutations in Chinese patients with Lebers hereditary optic neuropathy (LHON), optic atrophy of unknown etiology, and optic neuropathy of known etiology. METHODS Twenty-seven patients from 25 LHON pedigrees, 22 patients with bilateral optic atrophy of unknown etiology, 21 patients with optic neuropathy of known etiology, and 25 normal healthy controls were included in this study. Twelve pairs of primers that covered the 21 reported mtDNA mutations were utilized. Single-strand conformation polymorphism analysis and DNA sequencing were used to detect base substitutions in mtDNA. RESULTS Twenty-three LHON pedigrees (92%) had the 11778 mtDNA primary mutation. Two pedigrees (8%) had the 14484 mutation. No 3460 mutations were detected in this group. Thirteen other sequence changes were found in these patients, but only the 4216 mutation had been reported previously. Thirteen pedigrees had multi-mutation patterns consisting of one primary mutation together with other sequence changes. No primary mutations were found in patients with optic atrophy of unknown etiology or in patients with optic neuropathy of known etiology. CONCLUSIONS High frequency of 11778 mtDNA mutation was found in Chinese patients with LHON. No specific multi-mutation pattern such as the European mtDNA haplogroup J was found.

Low Conversion Rate to Multiple Sclerosis in Idiopathic Optic Neuritis Patients in Taiwan

PurposeTo investigate the clinical characteristics of patients with idiopathic optic neuritis (ON) in Taiwan and to assess the conversion rate to multiple sclerosis (MS) in these patients.MethodsWe studied the medical records of a total of 109 patients with a clinical diagnosis of idiopathic ON treated in the Taipei Veterans General Hospital during the period from January 1986 to May 2003. Clinical characteristics, management, and disease courses were retrospectively reviewed. Our main focus was on the development of multiple sclerosis after an ON attack. Univariate and multivariate analyses were used to evaluate the risk indicators for MS conversion.ResultsThe patients (58 women, 51 men) had a mean age of 41.2 years at onset. ON was retrobulbar in 46.8% of the patients. Management with or without pulse therapy did not affect the final visual outcome. Female sex, retrobulbar type ON, recurrent cases, elevated cerebrospinal fluid (CSF) IgG index, and central nervous system (CNS) imaging abnormalities were identified as risk indicators for the development of MS (P < 0.05). The 2-year cumulative probability of developing MS was 5.92%, and the 5-year cumulative probability was 14.28%. The conversion rate to MS did not differ among treatment groups.ConclusionsIdiopathic ON patients in Taiwan have an older age at onset and a higher percentage of optic disc edema than reported in previous literature. The characteristic features of ON patients associated with a high risk of developing MS are female sex, retrobulbar type ON, CNS imaging abnormalities, elevated CSF IgG index, and recurrence. Idiopathic ON patients in Taiwan display a significantly lower conversion rate to MS. Jpn J Ophthalmol 2006;50:170–175

Leukaemic infiltration of the optic nerve as the initial manifestation of leukaemic relapse.

Leukaemic infiltration of the optic nerve as the initial manifestation of leukaemic relapse

Optic neuritis in herpes zoster ophthalmicus.

BACKGROUND Optic neuritis in herpes zoster ophthalmicus (HZO) has been reported rarely. We report two cases of HZO optic neuritis with detailed magnetic resonance imaging study and treatment responses. CASES One patient presented with anterior optic nerve involvement, and the second presented with retrobulbar optic neuritis. Contrast enhanced T(1)-weighted images were obtained in these 2 patients. Intravenous acyclovir and oral prednisolone were given simultaneously. OBSERVATIONS Magnetic resonance imaging revealed peripheral enhancement of the optic nerve sheath complex on T(1)-weighted scan. Both patients recovered their vision within 3 months following the start of treatment. CONCLUSIONS Magnetic resonance imaging is helpful for the diagnosis of HZO optic neuritis. Systemic acyclovir and steroid are effective in the treatment of HZO optic neuritis.

Paradoxical worsening with superior ophthalmic vein thrombosis after gamma knife radiosurgery for dural arteriovenous fistula of cavernous sinus: a case report suggesting the mechanism of the phenomenon.

Paradoxical worsening with superior ophthalmic vein thrombosis after gamma knife radiosurgery for dural arteriovenous fistula of cavernous sinus: a case report suggesting the mechanism of the phenomenon