Non-fluent Primary Progressive Aphasia and Its Association to Motor Neuron Disease.

Frontotemporal dementia (FTD) is among the most common forms of dementia, with an average symptom onset in the fifth decade of life. Neuropathologic changes in FTD demonstrate degeneration in the frontal and/or temporal lobes, which is defined as frontotemporal lobar degeneration (FTLD). FTD is categorized into a subset of variants by symptomatic presentation and corresponding clinical workup. Primary progressive aphasia (PPA) is among these variants of FTD and is distinguished by its primary clinical presentation of language impairment with correlating neuropathology in the aforementioned areas of the brain. More specifically, the classification of PPA is further subdivided into three clinical variants, which has allowed for appropriate diagnostic and prognostic considerations within this patient population. Among these variants in PPA are the semantic (svPPA), non-fluent (navPPA), and logopenic (lvPPA) forms. Motor neuron disease (MND) is a progressive and irreversible process of neuronal degeneration that can lead to an upper motor neuron, a lower motor neuron, or a combination of these two symptomologies. FTD and its association with MND is a well-established spectrum, although more rarely among the PPA variant of FTD. Comparatively, there is a significant body of clinical knowledge on the association between the behavioral variant of FTD (bvFTD) and MND. This is the case of a 69-year-old female with navPPA who later presented with clinical symptoms of MND. Although the two clinical diagnoses, PPA and MND, are irreversible and progressive, this case serves to elucidate diagnostic and prognostic considerations in this rare patient population.

Cerebral Venous Thromboses in a Patient With No Reported Risk Factors: A Case Report.

Stroke is a neurologic condition caused either by brain ischemia or brain hemorrhage, where most cases are a result of ischemic brain injury. Stroke more commonly affects the arterial blood supply of the brain, but in rare cases, it is evoked by the occlusion of the venous sinuses that drain blood from the brain. This phenomenon is known as cerebral venous sinus thrombosis (CVST), also referred to as cerebral sinovenous thrombosis. The pathogenesis of CVST is not completely understood, although common risk factors associated with the condition include obesity, hypercoagulable states, oral contraceptive use, intracranial infections, trauma, and, more recently, coronavirus disease 2019 (COVID-19). Immediate medical intervention is required because CVST can result in increased intracranial pressure and diffuse cerebral edema, which can bring about fatal complications that can lead to early death. However, CVST is challenging to diagnose, as its clinical presentation is highly variable. It can range from headaches to signs of elevated intracranial pressure, including nausea, vomiting, and vision problems. In this case report, the patient is a 25-year-old previously healthy African American female who presented with a weeklong headache and acute onset of delirium an hour prior to arrival at the hospital. The patient had prior emergency department (ED) visits from different facilities where head imaging was performed and showed negative results allowing her to return home. The patient was then brought by a friend to our ED due to altered mental status and agitation. Initial computed tomography of the head did not reveal acute abnormalities; however, magnetic resonance angiography and magnetic resonance venography revealed evidence of venous sinus thrombosis and lack of flow requiring urgent attention. The patient was then referred to endovascular neurology, but despite medical intervention, the patient's medical status deteriorated, and she was declared brain dead. Although rare, this case report emphasizes the atypical presentation and the severity of CVST where a young individual with no significant past medical history presented with neurological symptoms that rapidly progressed to complications that caused her early death.

Clinical implication of recent randomized control trial in primary angle-closure disease management.

Blindness due to primary angle-closure glaucoma (PACG) can be reduced significantly if the ongoing angle-closure process is arrested at an early stage. Various treatments such as laser peripheral iridotomy (LPI), iridoplasty, and clear lens extraction (CLE) have been advocated as first-line therapy for primary angle-closure (PAC), PACG, and high-risk cases of primary angle-closure suspect (PACS). EAGLE study, propagated the effectiveness of CLE over LPI for the management of primary angle closure and have sparked controversy regarding the role of LPI as a first line procedure. Randomized controlled trials (RCT), systematic reviews, and meta-analyses of RCTs done on the same question provide us with a solid base for creating guidelines/modules for our day-to-day clinical practice. A systematic review was conducted, searching several databases, including PubMed, Cochrane Library, EMBASE, and ClinicalTrials.gov, for the last 16 years (January 2005-December 2021) for RCTs with data published related to primary angle-closure disease (PACD). The search strategy included the following terms: "Primary Angle Closure disease," "Primary Angle Closure Glaucoma," "Primary Angle Closure," "Primary Angle Closure Suspect," "clear lens extraction," "laser iridotomy," "laser peripheral iridotomy," "argon laser peripheral iridoplasty," "selective laser trabeculoplasty," "trabeculectomy," "randomized control trial," and "meta-analysis of randomized control trial." In this review, we will discuss recently published RCTs (within the last 16 years) for the management of PACD and their clinical implications in day-to-day practice.

Retinal nerve fiber layer thickness in amblyopia.

Purpose: To report retinal nerve fiber layer thickness (RNFLT) in eyes with amblyopia compared with contralateral healthy eyes. Methods: In this cross-sectional study, we included patients with anisometropic amblyopia, strabismic amblyopia, and mixed amblyopia. All subjects underwent complete ophthalmic examination, including RNFLT measurement with time-domain OCT (Stratus OCT) and scanning laser polarimeter (GDX VCC). A paired "t" test was used to compare average and quadrant-wise RNFL thickness between the amblyopic and contralateral normal eyes. In addition, an analysis of variance test was used to compare various RNFL thickness parameters between the three groups. Results: A total of 33 eyes of 33 subjects with anisometropic amblyopia, 20 eyes of 20 subjects with strabismic amblyopia, and 38 eyes of 38 subjects with mixed amblyopia were included. In the anisometropic amblyopia group, the average RNFLT in the amblyopic eye was 98.2 µm and 99.8 µm in the fellow normal eye (P = 0.5), the total foveal thickness was 152.82 µm (26.78) in the anisometropic eye and 150.42 µm (23.84) in the fellow eye (P = 0.38). The difference between amblyopic and contralateral normal eye for RNFL and macular parameters was statistically insignificant in all three groups. The RNFL thickness in four quadrants was similar in the amblyopic and non-amblyopic eye between all three groups and statistically non-significant. Conclusion: Our study showed that RNFL thickness was similar in amblyopic and non-amblyopic eyes between all three amblyopia groups.

A deep dive into the latest European Glaucoma Society and Asia-Pacific Glaucoma Society guidelines and their relevance to India.

Glaucoma is the second leading cause of blindness in India. Despite advances in diagnosing and managing glaucoma, there is a lack of India-specific clinical guidelines on glaucoma. Ophthalmologists often refer to the European Glaucoma Society (EGS) and Asia-Pacific Glaucoma Society (APGS) guidelines. A group of glaucoma experts was convened to review the recently released EGS guideline (fifth edition) and the APGS guideline and explore their relevance to the Indian context. This review provides the salient features of EGS and APGS guidelines and their utility in Indian scenario. Glaucoma diagnosis should be based on visual acuity and refractive errors, slit-lamp examination, gonioscopy, tonometry, visual field (VF) testing, and clinical assessment of optic nerve head, retinal nerve fiber layer (RNFL), and macula. The intraocular pressure target must be individualized to the eye and revised at every visit. Prostaglandin analogues are the most effective medications and are recommended as the first choice in open-angle glaucoma (OAG). In patients with cataract and primary angle-closure glaucoma (PACG), phacoemulsification alone or combined phacoemulsification and glaucoma surgery are recommended. Trabeculectomy augmented with antifibrotic agents is recommended as the initial surgical treatment for OAG. Laser peripheral iridotomy and surgery in combination with medical treatment should be considered in high-risk individuals aged <50 years. In patients with phakic and PACG, phacoemulsification alone or combined phacoemulsification and glaucoma surgery are recommended. Visual acuity, VF testing, clinical assessment of the optic disc and RNFL, and tonometry are strongly recommended for monitoring glaucoma progression.

Optic disc morphology in primary open-angle glaucoma versus primary angle-closure glaucoma in South India.

Purpose: The aim of this study was to investigate the optic disc morphology in primary angle-closure glaucoma (PACG) versus primary open-angle glaucoma (POAG) in South Indians. Methods: A total of 60 patients (60 eyes) with PACG and 52 patients (52 eyes) with POAG were included in a cross-sectional observational study. The glaucoma diagnosis was based on a glaucomatous appearance of the optic disc correlating with visual field defects. The glaucoma was graded as early, moderate, or severe, depending upon perimetric loss. All patients underwent an ophthalmic evaluation, including visual field examination and planimetric analysis of 30° stereoscopic color optic disc photographs. Results: The POAG and PACG groups did not differ significantly in a disc or rim area, rim width, and frequencies of disc hemorrhages or rim notches. However, early POAG group (n = 15) had a significantly deeper cup depth (P = 0.01), larger beta zone (P = 0.01), and a higher frequency of localized retinal nerve fiber layer (RNFL) defects (P = 0.02) than early PACG (n = 20). Conclusion: In the early stage of the disease, POAG compared to PACG may be characterized by deeper disc cupping, a larger beta zone of peripapillary atrophy, and a higher frequency of localized RNFL defects. Such differences in early glaucoma may suggest differences in pathophysiology in POAG and PACG.

Chemical disinfectants in ophthalmic practice.

The instrumentation used in ophthalmic clinics can be a source of epidemics in health care set up. Contact tonometry with Schiotz or Applanation tonometer is associated with nosocomial epidemic keratoconjunctivitis outbreaks. Recently identified SARS-CoV-2 (COVID -19) spreads mainly via the respiratory route and fomites and can transmit through other body fluids, including tear film. Various ophthalmic instruments can become a common source of spreading cross infections. Chemical disinfection is one of the most common methods employed to decontaminate instruments and environmental surfaces and prevent transmission of infectious pathogens to patients through medical and surgical instruments. Various chemical disinfectants are available with a varied spectrum to work on a different group of organisms. In this article, we briefly cover commonly used chemical disinfectants in ophthalmic practice like Alcohol (Ethyl Alcohol, Isopropyl Alcohol), Chlorine-based solution (mainly Sodium Hypochlorite), Glutaraldehyde, Hydrogen Peroxide, Formaldehyde, Iodophors, and Quaternary Ammonium Compounds.

Cost-effectiveness and cost utility of community screening for glaucoma in urban India.

OBJECTIVES: Population-based screening for glaucoma has been demonstrated to be cost-effective if targeted at high-risk groups such as older adults and those with a family history of glaucoma, and through use of a technician for conducting initial assessment rather than a medical specialist. This study attempts to investigate the cost-effectiveness of a hypothetical community screening and subsequent treatment programme for glaucoma in comparison with current practice (i.e. with no screening programme but with some opportunistic case finding) in the urban areas of India. STUDY DESIGN: A hypothetical screening programme for both primary open-angle glaucoma and angle-closure disease was built for a population aged between 40 and 69 years in the urban areas of India. METHODS: Screening and treatment costs were obtained from an administrator of a tertiary eye hospital in India. The probabilities for the screening pathway were derived from published literature and expert opinion. The glaucoma prevalence rates for urban areas were adapted from the Chennai Glaucoma Study findings. A decision-analytical model using TreeAge Pro 2015 was built to model events, costs and treatment pathways. One-way sensitivity analyses were conducted. RESULTS: The introduction of a community screening programme for glaucoma is likely to be cost-effective, the estimated incremental cost-effectiveness ratio (ICER) values being 10,668.68 when compared with no screening programme and would treat an additional 4443 cases and prevent 1790 person-years of blindness over a 10-year period in the urban areas of India. Sensitivity analyses revealed that glaucoma prevalence rates across various age groups, screening uptake rate, follow-up compliance after screening, treatment costs and utility values of health states associated with medical and surgical treatment of glaucoma had an impact on the ICER values of the screening programme. CONCLUSIONS: In comparison with current practice (i.e. without a screening programme but with some opportunistic case finding), the introduction of a community screening programme for glaucoma for the 40-69 years age group is likely to be relatively cost-effective if implemented in the urban areas of India.

Pulmonary leucostasis in a patient with chronic lymphocytic leukaemia.

A 71-year-old woman with no chronic medical problems presented to the emergency room with a 24-month history of chronic non-productive cough, malaise, increasing fatigue and weight loss. On physical examination, she had significant cervical and axillary lymphadenopathy with no organomegaly. She was diagnosed with chronic lymphocytic leukaemia (CLL) due to her lymphocyte count of 1789.4×10(9)/L on admission. A few days after hospitalisation, she developed respiratory failure requiring intubation. A chest X-ray showed interstitial markings. The abnormally high number of mature lymphocytes could have caused leucostasis in the lungs of this patient with CLL.

Evaluation of blotchy pigments in the anterior chamber angle as a sign of angle closure.

BACKGROUND: Blotchy pigments in the anterior chamber (AC) angle are considered diagnostic of primary angle closure (PAC). But there are no reports either on the prevalence of blotchy pigments in AC angles or the validity of this sign. AIMS: To determine the prevalence of blotchy pigments in AC angles and to evaluate their relationship with glaucomatous optic neuropathy (GON) in eyes with occludable angles. SETTING AND DESIGN: Cross-sectional, comparative study. MATERIALS AND METHODS: Gonioscopy was performed in 1001 eyes of 526 subjects (245 eyes of 148 consecutive, occludable angle subjects and 756 eyes of 378 non-consecutive, open angle subjects), above 35 years of age. Quadrant-wise location of blotchy pigments was documented. STATISTICAL ANALYSIS: Odds of blotchy pigments in occludable angles against that in open angles were evaluated. Relationship of GON with blotchy pigments in occludable angle eyes was evaluated using a multivariate model. RESULTS: Prevalence of blotchy pigments in occludable angles was 28.6% (95% CI, 22.9-34.3) and in open angles was 4.7% (95% CI, 3.2-6.3). Blotchy pigments were more frequently seen in inferior (16%) and superior quadrants (15%) of occludable angles, and inferior quadrant of open angles (4%). Odds of superior quadrant blotchy pigments in occludable angles were 33 times that in open angles. GON was seen in 107 occludable angle eyes. Blotchy pigments were not significantly associated with GON (odds ratio = 0.5; P = 0.1). CONCLUSIONS: Blotchy pigments were seen in 28.6% of occludable angle eyes and 4.7% of open angles eyes. Presence of blotchy pigments in the superior quadrant is more common in occludable angles. Presence of GON in occludable angle eyes was not associated with blotchy pigments.

Development and implementation of a comprehensive heparin-induced thrombocytopenia recognition and management protocol.

PURPOSE: A quality initiative to improve the management of heparin-induced thrombocytopenia (HIT) at an academic medical center, including the development of guidelines on the use of direct thrombin inhibitors (DTIs), is described. SUMMARY: In keeping with the Joint Commission's National Patient Safety Goal (NPSG) for anticoagulant therapy (goal 03.05.01), a multidisciplinary working group conducted a needs assessment to identify areas for improvement in the center's HIT management practices, particularly the use of DTI therapy (an issue not specifically addressed by NPSG 03.05.01). The resulting action steps included (1) the implementation of a detailed protocol for the recognition and management of HIT, as well as guidelines on the use of the DTIs argatroban and lepirudin, (2) more efficient use and optimized documentation of initial and confirmatory tests in the electronic medical record (EMR), and (3) the education of pharmacists, nurses, and physicians on the use of the HIT protocol, with initial and ongoing case-based competency testing of pharmacy staff. Early postimplementation experience indicated that the protocol and associated activities have resulted in improved DTI prescribing and dosing, HIT documentation, and patient education practices while expanding pharmacists' involvement in ensuring optimal, cost-effective management of patients with HIT. CONCLUSION: In one institution, an HIT working group extended the scope of NPSG 03.05.01 to include the parenteral DTIs. The implementation of the HIT protocol has resulted in greater compliance with appropriate DTI dosing and improved EMR documentation of HIT.

There is insufficient evidence to recommend lens extraction as a treatment for primary open-angle glaucoma: an evidence-based perspective.

Cataract extraction in primary open-angle glaucoma has not been thought to provide a clinically useful or predictable decrease in IOP. This concept has now been challenged, with the opposite belief being promulgated: namely, that lens exchange should be considered as treatment for glaucoma. This revelation could bring a significant change in the glaucoma treatment paradigm. There are no randomised controlled trials to guide the role of lens extraction in primary open-angle glaucoma. The available evidence suggests at most a modest reduction in IOP from cataract extraction - greater in the presence of pseudoexfoliation - which is likely to be of marginal benefit, and only in milder forms of open-angle glaucoma. There is currently no evidence of any quality to suggest that lens extraction routinely represents a clinically useful treatment for primary open-angle glaucoma.

Effect of optic disc size and disease severity on the diagnostic capability of glaucoma imaging technologies in an Indian population.

PURPOSE: To evaluate the influence of optic disc size and disease severity on the diagnostic validity of optical coherence tomography (Stratus OCT), scanning laser polarimetry [GDx variable corneal compensator (VCC)], and confocal scanning laser ophthalmoscopy [Heidelberg retina tomograph II (HRT II)] in Indian eyes with glaucoma. METHODS: Ninety-five normal and 125 glaucoma patients underwent imaging with Stratus OCT, GDx VCC, and HRT II. One eye of each person was randomly selected for analysis. Using disc area determined on HRT II, discs were classified as small (<2 mm), moderate (2 to 3 mm), and large (>3 mm). The parameter with the best sensitivity for each device, at a fixed specificity, was compared for different disc sizes. Logistic marginal regression was used to study the effect of disc size and disease severity (mean deviation on standard automated perimetry) on the diagnostic performance of these imaging devices. RESULTS: At a fixed specificity of 84.2%, the sensitivity of HRT II was significantly different for varying disc sizes (P=0.0004). The sensitivities for dissimilar disc sizes were not significantly different for the GDx VCC (P=0.928) or Stratus OCT (P=0.381). Logistic marginal regression also showed that sensitivity of HRT II increased with increasing disc size, whereas sensitivity of OCT and GDx were independent of the disc size. The sensitivity of all 3 technologies increased with increasing disease severity (decreasing mean deviation). CONCLUSIONS: Optic disc size affects the diagnostic capability of HRT II but not that of GDx VCC or Stratus OCT. The sensitivity of all 3 imaging technologies increased with increasing disease severity.

Role of anterior segment optical coherence tomogram in Descemet's membrane detachment.

Descemet's membrane detachment (DD) is a rare but serious complication of intraocular surgery. In rare cases where corneal edema is severe and we may not be able to visualize DD on slit-lamp examination, anterior segment optical coherence tomogram (AS-OCT) would be helpful. We describe two patients with DD, highlighting the role of AS-OCT in early diagnosis and management of patients with DD. One of the patients had DD with rolled in edge, which could only be visualized with AS-OCT. In such a situation, AS-OCT can identify the edge of detachment and show the exact position of the rolled edge, which can allow us to plan the surgical strategy to unroll the DD.

Unusual presentation in Axenfeld-Rieger syndrome.

We report an unusual presentation of a case of Axenfeld-Rieger (A-R) syndrome. A 14-year-old male presented with gradual dimness of vision for 1 year and redness of left eye for 3 days. The patient had megalocornea with Haab's striae in the right eye and posterior embryotoxon in both the eyes. In the left eye, there was a white cord-like structure traversing the anterior chamber with adhesions to iris tissue along its course. On two antiglaucoma medications, his intraocular pressure (IOP) was 22 mm Hg in the right eye and 18 mm Hg in the left eye. Gonioscopy revealed a cord-like structure originating at the level of Schwalbe's line. He underwent right eye trabeculectomy with mitomycin-C. This case highlights a rare presentation of a strange cord-like structure, a rare presentation of A-R syndrome.

Enter the reverend: introduction to and application of Bayes' theorem in clinical ophthalmology.

BACKGROUND: Ophthalmic practice utilizes numerous diagnostic tests, some of which are used to screen for disease. Interpretation of test results and many clinical management issues are actually problems in inverse probability that can be solved using Bayes' theorem. DESIGN: Use two-by-two tables to understand Bayes' theorem and apply it to clinical examples. SAMPLES: Specific examples of the utility of Bayes' theorem in diagnosis and management. METHODS: Two-by-two tables are used to introduce concepts and understand the theorem. The application in interpretation of diagnostic tests is explained. Clinical examples demonstrate its potential use in making management decisions. MAIN OUTCOME MEASURE: Positive predictive value and conditional probability. RESULTS: The theorem demonstrates the futility of testing when prior probability of disease is low. Application to untreated ocular hypertension demonstrates that the estimate of glaucomatous optic neuropathy is similar to that obtained from the Ocular Hypertension Treatment Study. Similar calculations are used to predict the risk of acute angle closure in a primary angle closure suspect, the risk of pupillary block in a diabetic undergoing cataract surgery, and the probability that an observed decrease in intraocular pressure is due to the medication that has been started. The examples demonstrate how data required for management can at times be easily obtained from available information. CONCLUSIONS: Knowledge of Bayes' theorem helps in interpreting test results and supports the clinical teaching that testing for conditions with a low prevalence has a poor predictive value. In some clinical situations Bayes' theorem can be used to calculate vital data required for patient management.

Dilated episcleral veins with secondary open angle glaucoma.

We report a case of dilated episcleral vein with secondary open angle glaucoma. A 65-year-old female presented with redness of both the eyes without any prior systemic history. Her ocular examination revealed dilated episcleral veins and a high intraocular pressure (IOP) of 38 mm Hg in the right eye. Systemic examination was negative for carotid cavernous fistula, low-grade dural arteriovenous fistula, dysthyroid ophthalmopathy, and primary pulmonary hypertension. During follow-up, her IOP remained in high thirties despite maximum medications. She underwent right eye trabeculectomy with partial thickness sclerectomy with sclerotomy. In the beginning, the sclerotomy incision was not deepened into the suprachoroidal space. She developed choroidal effusion during surgery and the sclerotomy was deepened into suprachoroidal space and straw color fluid was drained. Postoperatively, she developed choroidal effusion, which resolved with conservative management. This case highlights the importance of sclerotomy in such cases of high episcleral venous pressure.