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Spinocerebellar ataxias (SCA) comprise a rare, genetic subgroup within the degenerative ataxias and are dominantly inherited, with up to 48 recognized genetic subtypes. While an updated review on the management of degenerative ataxia is published recently, an evidence-based review focussed on the management of SCA is lacking. Here, we reviewed the pharmacological and non-pharmacological management of SCA by conducting a systematic review on Medline Ovid and Scopus. Of 29,284 studies identified, 47 studies (pharmacological: n = 25; non-pharmacological: n = 22) that predominantly involved SCA patients were included. Twenty studies had a high risk of bias based on the Cochrane’s Collaboration risk of bias tool. As per the European Federation of Neurological Societies 2004 guideline for therapeutic intervention, the remaining 27 studies were of Class I (n = 4) and Class II (n = 23) evidence. Only two therapies had Level A recommendations for the management of ataxia symptoms: riluzole and immediate in-patient neurorehabilitation. Ten therapies had Level B recommendations for managing ataxia symptoms and require further investigations with better study design. These include high dose valproate acid, branched-chain amino acid, intravenous trehalose; restorative rehabilitation using cycling regimen and videogame; and cerebellar stimulations using transcranial direct current stimulation and transcranial magnetic stimulation. Lithium and coaching on psychological adjustment received Level B recommendation for depressive symptoms and quality of life, respectively. Heterogeneous study designs, different genotypes, and non-standardized clinical measures alongside short duration and small sample sizes may hamper meaningful clinical translation. Therefore, rating of recommendations only serve as points of reference.

Glaucoma services are overwhelmed and struggling to accommodate current demand. Reducing the need for hospital based services would improve our ability to see those most at risk of vision loss, which could both reduce demand and improve patient outcomes. Digital technologies that provide opportunities for home monitoring of glaucoma progression have potential to contribute to solve these challenges and, potentially, improve glaucoma care. This article will review the literatures of well-established technologies that support home monitoring for glaucoma, specifically home tonometry (with rebound tonometry) and perimetry with Moorfields Motion Displacement Test and Melbourne Rapid Field.

To cross-culturally translate and adapt the National Eye Institute Visual Functioning Questionnaire (NEIVFQ-25) into Malay or Bahasa Malaysia, and to analyze its psychometric properties in a cohort of Malaysian patients with visual impairment from various causes. Cross-sectional validation study. The NEI-VFQ 25 was translated and cross-culturally adapted into the Malay version. Total of 324 visually impaired patients caused by cataracts, glaucoma, age-related macular degeneration (ARMD) or diabetic macular edema (DME), and a control group were included. Psychometric analysis was performed including test-retest and internal consistency reliability, convergent validity, discriminant validity, and factor analysis. Clinical validity was measured by correlation of clinical measurements with subdomain scores and known-groups comparison. Participants' average age was 60.4 years (SD 15.4) and 47.2% were male. The internal consistency was high in most subdomains, with Cronbach alpha ranging from 0.66-0.89. The test-retest reliability was high (intraclass correlation coefficient 0.92). Even when just one eye had impaired vision, participants scored much lower on the Malay NEIVFQ 25, highlighting how well the questionnaire reflects the real impact of visual impairment. Moderate correlations were detected between visual acuity and the 'General vision', 'Near Activities', 'Distance Activities', 'Social Functioning', 'Mental Health', 'Role Difficulties', 'Dependency' and 'Driving' subdomains suggesting that these subdomains were associated with central vision. Factor analysis demonstrated that 4 factors can be extracted from 12 subdomains. This study revealed that the Malay version of the NEIVFQ-25 is a valid, reliable, and reproducible instrument to measure the vision-related quality of life in patients with visual impairment. Quality of life was adversely affected even with only one eye having poor vision.

AimTo estimate the minimally important difference (MID) in change in National Eye Institute Visual Function Questionnaire-25 (VFQ-25) composite score using methods aligned with patient perception.MethodsRetrospective analysis of prospectively collected data from adults with primary angle closure or primary angle closure glaucoma enrolled in the Effectiveness, in Angle-closure Glaucoma, of Lens Extraction study. We included data from 335 participants with patient reported visual function (VFQ-25) and health status measured by the EQ-5D-3L over 36 months. We used the recommended anchor-based methods (receiver operating characteristic (ROC), predictive modelling and mean change) to determine the MID of the VFQ-25. EQ-5D-3L anchor change was defined as none (<0.065); minimal (0.065≤EQ-5D-3L change ≤0.075 points) and greater change (>0.075 points).ResultsMean baseline VFQ-25 score was 87.6 (SD 11.8). Estimated MIDs in the change in VFQ-25 scores (95% CI) were 10.5 (1.9 to 19.2); 3.9 (−2.3 to 10.1); 5.8 (1.9 to 7.2) and 8.1 (1.7 to 14.8) for the ‘within-patient’, ‘between-patient’ change, ROC and predictive modelling anchor methods respectively. Excluding estimates from the methodologically weaker ‘within-patient’ method, the MID of a change in VFQ-25 composite score is 5.8 (median value).ConclusionsEstimates of the MID using multiple methods assist in the interpretation of the VFQ scores. In the context of early glaucoma related visual disability, a change score of around six points on the VFQ-25 is likely to be important to patients. Further confirmatory research is required. Studies comparing changes in patient-reported outcome measure scores with a global measure of patients’ perceived change are required.

We report a case of successful argon laser synechiolysis as a non-invasive alternative for peripheral anterior synechiae release after trabeculectomy in a young patient with steroid-induced ocular hypertension. Steroid-induced ocular hypertension is a known complication of vernal keratoconjunctivitis due to prolonged treatment with steroids. In refractive conditions, augmented trabeculectomy becomes the surgery of choice in these patients. In this article, we report successful treatment of iris tissue plugging the internal ostium with an argon laser and reinstatement of aqueous flow.

This case report aims to describe a case of unilateral central retinal artery occlusion (CRAO) with cilioretinal artery sparing, which was complicated by neovascular glaucoma (NVG). A 75-year-old Indian woman with underlying normal tension glaucoma presented with the sudden onset of painless generalized blurring of the right eye's vision for a week. Her right eye vision was hand motion with the presence of a right relative afferent pupillary defect. Fundus examination revealed retinal whitening over the macula sparing the papillomacular bundle with generalized retinal arteriolar attenuation, which was suggestive of right CRAO with cilioretinal artery sparing. Systemic examination revealed high blood pressure (175/75 mmHg) without ocular bruit or audible murmur on auscultation. Optical coherence tomography of the macula showed inner retinal thickening over the temporal macula. Ultrasound carotid Doppler and computed tomography angiography of the carotid showed more than 75% stenosis over the right distal internal carotid artery.Unfortunately, she developed rubeosis iridis over her right eye two weeks after her presentation, which required pan-retinal photocoagulation. She subsequently progressed to NVG, requiring maximum anti-glaucoma medications to stabilize intraocular pressure. In conclusion, CRAO is a sight-threatening medical emergency. Thorough investigations are required to determine the underlying cause so that early intervention can be done to reduce the risk of a similar attack in the fellow eye and the risk of a cerebrovascular event or cardiac ischemia, which could be life-threatening. The presence of a cilioretinal artery does not prevent ocular neovascularization in CRAO. Hence, patients should also be closely monitored after the initial diagnosis to prevent devastating complications such as NVG.

Epithelial conjunctival malignancies are one of the most prevalent ocular surface tumors. Primary basal cell carcinoma (BCC) of the conjunctiva is extremely rare. We report the case of a 67-year-old Indian gentleman who presented with a fleshy conjunctival lesion for one year on his right eye. Examination revealed a lightly pigmented conjunctival mass adjacent to the limbus. The surface was irregular and non-ulcerated with few feeder vessels. The working diagnosis was ocular surface squamous neoplasia (OSSN). A wide excisional biopsy using the no-touch technique and double-freeze-thaw cryotherapy to the conjunctival margins was performed. The bare scleral area was covered with an amniotic membrane. Histopathological examination revealed a basaloid cell neoplasm favoring BCC, and a tumor-free margin was achieved. Three cycles of topical mitomycin-C 0.02% were prescribed as adjunct chemotherapy postoperatively. There was no evidence of recurrence three months after treatment. The primary BCC of the conjunctiva is unusual and can resemble OSSN. Therefore, it should be considered in the differential diagnosis of patients presenting with atypical features of OSSN.

We report a unique case of paraneoplastic syndrome (PS) associated with primary lung cancer. A 57-year-old woman experienced headaches and bilateral visual loss one month after the onset of isolated right abducens palsy. Examination revealed bilateral poor visual acuity (VA), papilledema, and persistent right abducens palsy. Neuroimaging was normal. Lumbar puncture revealed high cerebrospinal fluid (CSF) opening pressure and protein levels. She was started on acetazolamide and pulse methylprednisolone followed by oral corticosteroids. Her abducens nerve palsy resolved, but her VA deteriorated. Anti-Hu and anti-CV2 were positive. A positron emission tomography (PET) scan revealed primary lung cancer, and she died six months after her initial presentation. This case demonstrated that PS poses a diagnostic challenge and may be associated with poor prognosis.

We report a case of bilateral open globe injury that resulted from a durian fruit falling on a 62-year-old woman's unprotected face during durian picking in her orchard. On presentation, the bilateral vision was light perception. The right eye sustained a curvilinear corneal laceration with expelled intraocular content. Meanwhile, the left eye sustained a corneoscleral laceration with expelled uvea and retina. Additionally, the right upper lid margin was lacerated. Emergency wound exploration, primary toilet, and suturing were performed on bilateral eyes. Preoperatively, she received intramuscular anti-tetanus toxoid and intravenous ciprofloxacin. Intravitreal ceftazidime and vancomycin were given intraoperatively as endophthalmitis prophylaxis. Postoperatively, the vision remained as light perception. There were no signs of endophthalmitis in both eyes. Although traumatic globe injury due to durian is uncommon, individuals should wear protective gear while in a durian orchard to avoid such unprecedented accidents. Prompt yet scrupulous action should be taken to save the globe and further possible complications.

Iridodialysis occurs less commonly due to ocular surgeries as compared to trauma. Several approaches to iridodialysis repair have been described in the literature. In this report, we describe a novel technique to treat iridodialysis that occurred due to complicated extracapsular cataract extraction (ECCE). This technique utilizes pre-existing ECCE wounds by making use of common materials found in the usual operation theaters and is relatively easy to master. Improved cosmetic appearance and visual acuity was the final outcome as demonstrated in our patient.