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

OBJECTIVE

This study described the ophthalmic symptoms and signs in patients with nasopharyngeal carcinoma (NPCA).

This was a retrospective, cross-sectional, descriptive study involving patients with histologically-confirmed NPCA seen in two subspecialty eye clinics in a single referral hospital from January 2014 to December 2018. Chart review obtained data on symptoms and ophthalmic findings of patients with NPCA on the first visit. Descriptive statistics was used to analyze the data.

There were 36 patients in the study. There were 27 males (75%) and mean age was 47 years (Range: 13 - 83). Delay to consult was marked, with 28 patients (78%) presenting later than three months; 19 (53%) had invasion to distant sites on presentation. Almost all of the patients (35/36 or 97%) had either diplopia or blurring of vision, with nasal symptoms as the most common extra-ophthalmic accompanying symptom. Multiple cranial nerve palsies, particularly optic nerve plus at least one ocular motor nerve, was a prominent feature. The combination of nasal symptoms with ophthalmoparesis was noted in 24 patients (67%) and was identified as a red flag for NPCA.

Blurred vision and diplopia were the most common ocular complaints of patients with NPCA who were evaluated at the ophthalmology department of a tertiary hospital. Blurred vision is frequently from optic nerve involvement while diplopia is due to ophthalmoparesis secondary to multiple ocular motor cranial nerves involvement. Male patients in their 40s who present with combination of optic neuropathy or ocular motor palsies should be probed for presence of otologic or nasal symptoms as well as neck masses as these are the common presentation of NPCA in the ophthalmology clinics.

We report a 39-year-old male who had generalized tonic-clonic seizure with loss of awareness. Investigations led to a diagnosis of a left temporal lobe tumor. He underwent resection of the mass with consequent loss of brain tissue in the temporal lobe and was found to have a complete right homonymous hemianopia in the immediate postoperative period. Macular ganglion cell analysis on optical coherence tomography (OCT) showed homonymous thinning affecting the inferonasal sector in the right eye and inferotemporal sector in the left eye. This case demonstrates transsynaptic retrograde degeneration through the interruption of the inferior optic radiation, and its corresponding effect on the structure and function of the affected retinal field. Temporal lobe lesions may cause not only a homonymous visual f ield defect contralateral to the side of the lesion but also result to homonymous sectoral thinning of the macular ganglion cell complexes in both eyes located ipsilateral to the side of the lesion.
Human ; retinal ganglion cells ; hemianopsia ; temporal lobe

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

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

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

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

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