Posterior Cortical Atrophy is a group of neurodegenerative disorders characterized by early, prominent and progressive impairment of visuospatial and visuoperceptual functions in the context of relatively preserved memory and insight in the early phases. Initial visual symptoms are vague, compelling patients to seek ophthalmologic consult. They present with simultagnosia and spatial disorientation, which are often missed by routine ophthalmologic and neurologic exams, causing delay in diagnosis. As the disease progresses, Posterior Cortical Atrophy ultimately leads to a more diffuse pattern of cognitive dysfunction. The underlying pathology is believed to be Alzheimer’s Disease and a greater level of amyloid plaques is correlated with earlier clinical symptoms of Posterior Cortical Atrophy. The clinical features of reported cases are heterogenous, leading to a classification of different variants and underlying pathologies. We report the serial clinical, cognitive and imaging data of a variant of Posterior Cortical Atrophy primarily affecting the dorsal stream.
Neuropsychological Tests

Objectives: To determine the retinal nerve fiber layer thickness (RNFL) in eyes with ethambutol-induced toxic optic neuropathy (ETON) at the time of diagnosis and to describe the visual outcomes at 1, 3, and 6 months after discontinuation of ethambutol Methods: This was a retrospective chart review of 8 patients (15 eyes) diagnosed with ETON that had RNFL thickness measurements using Cirrus® spectral-domain optical coherence tomography (OCT) at the time of diagnosis. Visual function was measured on initial visit and at 1, 3, and 6-month follow-up. Snellen visual acuity was converted to logMAR. Color vision was measured using Ishihara 14-plate test chart. Results: The mean duration from commencement of ethambutol intake to onset of visual symptoms was 16 weeks (range: 8-24). While, the mean duration from onset of visual symptoms to discontinuation of ethambutol was 4 weeks (range: 2-14). The mean global RNFL thickness at time of diagnosis was 101.2 ± 17.0 microns. Mean RNFL in the temporal, superior, nasal, and inferior sectors were as follows: 79.2 ± 15.4, 119.7 ± 27.9, 71.7 ± 9.2, and 136.7 ± 25.8 microns. Global and sectoral RNFL thicknesses were either normal or thick when compared to age-matched normal database. No eye displayed global or sectoral RNFL thinning. Mean baseline visual acuity and color vision were logMAR 1.2 and 5 plates, respectively. At 1, 3, and 6 months after discontinuation of ethambutol, mean visual acuity and color vision were 0.96 and 6, 0.63 and 11, and 0.44 and 13, respectively. Conclusion Patients with early ETON have normal or thick RNFL at time of diagnosis. They display good visual recovery 6 months following discontinuation of ethambutol.
Ethambutol ; Toxic Optic Neuropathy ; Tomography, Optical Coherence

We report a case of an otherwise healthy 23-month-old boy who presented with nystagmus, head shaking, and abnormal head posture suggestive of spasmus nutans. Neuro-ophthalmologic exam revealed bilateral, low-amplitude, high-frequency, horizontal, disconjugate nystagmus that was more prominent in one eye along with head shaking and a head tilt or face turn. The rest of the exam and the systemic physical examination were normal. Magnetic resonance imaging of the brain did not disclose optic pathway glioma, which has been reported to cause spasmus nutans-like disease. Electroretinogram (ERG) was also recommended to rule out occult retinopathies. However, it was not done due to unavailability of the appropriate corneal electrode for his age. Instead, close follow-up was advised to monitor spontaneous improvement or resolution, or until the child comes of age that he can undergo ERG. This case highlights the management approach and rationale of patients with presumed spasmus nutans. Recognition of the triad of spasmus nutans allows for quick diagnosis and more focused and efficient investigation.
pathologic nystagmus ; infantile spasms

OBJECTIVE: To describe the visual and clinical outcomes of 3 patients with sympathetic ophthalmia treated with a combination of systemic steroids and methotrexate. METHODS: This was a small, descriptive case series. RESULTS: We reported 3 cases of post-traumatic sympathetic ophthalmia treated with steroids and methotrexate. Two patients had inciting eyes with no light perception on presentation, while one had a best-corrected visual acuity (BCVA) of counting fingers. The initial BCVA of the sympathizing eyes ranged from 20/20 to 20/50. Control of ocular inflammation was achieved using methotrexate (12.5 to 15 mg weekly) in addition to oral steroids and topical therapy. The final BCVA of the sympathizing eyes ranged from 20/20 to 20/30, indicating that good visual outcomes were attainable with steroids and methotrexate as part of the maintenance regimen. None of the patients developed adverse side-effects from methotrexate. CONCLUSION: This small case series demonstrated the effectiveness and safety of methotrexate for control of intraocular inflammation in sympathetic ophthalmia.
Ophthalmia, Sympathetic ; Methotrexate ; Panuveitis ; Steroids

This paper provides the summaries on nine (9) important and clinically relevant publications in the field of uveitis. The first is on the standardization of uveitis nomenclature, more popularly known by its acronym - SUN, which was a result of an international workshop participated by uveitis experts in 2004. Five (5) papers were large, multicenter, clinical trials that demonstrated safety and efficacy of two (2) corticosteroids delivery devices (dexamethasone implant [Ozurdex] and fluocinolone acetonide implant [RetisertTM]) and one (1) immunomodulatory drug (adalimumab). The POINT trial compared various delivery approaches when using corticosteroids for the treatment of uveitic macular edema. The FAST trial compared two (2) durable and commonly-prescribed steroidsparing immunosuppressants, methotrexate and mycophenolate mofetil, for the treatment of non-infectious uveitis. Lastly, the SITE study, which was a large retrospective cohort study, determined the risks of overall and malignancy-related deaths among patients with inflammatory eye diseases receiving systemic immunosuppressants. Findings of these studies provide basis and rationale for the care and management of patients with uveitis and lay the groundwork for future research.
Uveitis ; Clinical Trial ; Review ; Adrenal Cortex Hormones

Neuroretinitis is an inflammatory optic neuropathy with a classic fundoscopic appearance of optic disc swelling and hard exudates on the macula in a star formation. It can be a manifestation of systemic, infectious or autoimmune disease. In nearly half of cases, the etiology is idiopathic. This review aims to summarize the clinical presentation, fundoscopic mimics, etiologies, investigation, and treatment of neuroretinitis. Cat-scratch disease, the most common cause of infectious neuroretinitis, and recurrent idiopathic neuroretinitis, which can cause ocular morbidity, are discussed in detail.
Retinitis ; Cat-Scratch Disease

Background and Objective: A parachiasmal lesion is defined as a mass or growth arising from structures around or near the chiasm. Ophthalmologic signs and symptoms may be observed in such condition, such as blurring of vision, visual field defects, and binocular double vision. The primary objective of this study was to describe the presenting ophthalmologic signs and symptoms of parachiasmal lesions among patients consulting at a single institution in the Philippines. Methods: This was a single-center, retrospective, cohort study. Medical records of patients with parachiasmal lesions seen in the Neuro-Ophthalmology clinic of a tertiary Philippine hospital from January 2014 to December 2019 were reviewed. Clinical profile, neuro-ophthalmologic presentation, diagnosis, management, and visual outcomes were summarized by descriptive statistics. Results: One hundred thirty-three (133) patient records satisfied the study criteria. Most common presenting symptoms were blurring of vision. headache, and loss of vision. Visual acuity at initial visit ranged from 20/20 to no light perception. A relative afferent pupillary defect was present in half of the study population. Almost half presented with normal-looking discs or disc pallor. Bitemporal hemianopia is the most common visual field defect pattern seen in both confrontation and automated visual field testing. Histopathology was significantly associated with visual outcome. Conclusion Parachiasmal lesion should be suspected in patients who complain of unilateral blurring of vision, and those who present with normal or pale optic discs. Pituitary adenoma is the most common radiologic and histopathologic diagnosis. Visual outcome after intervention has improved or remained stable in two-thirds of patients; visual recovery is multi-factorial, which is influenced by duration, surgery, and histopathology.
visual fields ; retrospective studies ; Philippines ; pituitary neoplasm ; visual acuity

High-quality clinical evidence, derived from well-designed and implemented clinical trials, serves to advance clinical care and to allow physicians to provide the most effective treatments to their patients. The field of ophthalmology, including the subspecialty of neuro-ophthalmology, abounds with such high-quality clinical trials that provide Level 1 clinical evidence. This review article summarizes the research design, key findings, and clinical relevance of select monumental clinical studies in neuro-ophthalmology with the primary goal of providing the readers with the rationale for current standard of care of various neuro-ophthalmic diseases. This includes the Optic Neuritis Treatment Trial, Ischemic Optic Neuropathy Decompression Trial, Idiopathic Intracranial Hypertension Treatment Trial, Rescue of Hereditary Optic Disease Outpatient Study, and Controlled High-Risk Avonex® Multiple Sclerosis Study
Optic Neuritis ; Optic Neuropathy, Ischemic ; Intracranial Hypertension

Purpose: This study compared the safety outcomes of two intracameral fluoroquinolone antibiotics, moxifloxacin and levofloxacin, as prophylaxis treatment in eyes that underwent uncomplicated cataract surgery. Methods: This is a prospective, double-masked, randomized, interventional, single-center clinical trial. Eyes with visually-significant cataracts underwent phacoemulsification and received preservative-free intracameral 0.5% moxifloxacin [58 eyes (M group)] or 0.5% levofloxacin [56 eyes (L group)] at the end of the surgery as antibiotic prophylaxis. The following safety parameters were evaluated postoperatively at Day 1, Week 1 and Month 1: central retinal thickness (CRT), macular volume (MV), central corneal thickness (CCT), and endothelial cell density (ECD). In-between group comparison was made at each of the 4 study visits using Student’s t-test. Results: Both M and L groups had similar baseline characteristics. There were no significant differences in CRT, MV, CCT and ECD between the 2 groups at each time point in the study. There were no significant differences in the mean changes in CRT, MV, CCT and ECD from baseline to final visit between the 2 groups. No study-related adverse events were observed during the study period. Conclusion Intracameral application of preservative-free 0.5% moxifloxacin and 0.5% levofloxacin appear to have similar safety outcomes when used as antibacterial prophylaxis among eyes undergoing cataract surgery. Based on the results, both fluoroquinolone agents are potentially suitable options for endophthalmitis chemoprophylaxis.
Levofloxacin ; Moxifloxacin ; Fluoroquinolones