Inflammatory activity following motor progression due to critical CNS demyelinating lesions.

BACKGROUND: New inflammatory activity is of unclear frequency and clinical significance in progressive multiple sclerosis (MS); it is uncertain in patient cohorts with motor progression due to critical demyelinating lesions. OBJECTIVES: The aim of this study is to determine the likelihood of central nervous system (CNS) inflammatory activity, assessed by new clinical relapses or active magnetic resonance imaging (MRI) lesions, following onset of motor progression due to critical demyelinating lesions. METHODS: Patients with progressive upper motor neuron impairment for ⩾1 year attributable to critical demyelinating lesions with single CNS lesion (progressive solitary sclerosis (PSS)), 2 to 5 total CNS demyelinating lesions (progressive "pauci-sclerosis" (PPS)), or >5 CNS demyelinating lesions and progressive exclusively unilateral monoparesis or hemiparesis (PUHMS) were identified. Clinical data were reviewed for acute MS relapses, and subsequent MRI was reviewed for active T1-gadolinium-enhancing or T2-demyelinating lesions. RESULTS: None of the 91 patients (22 PSS, 40 PPS, 29 PUHMS) identified experienced clinical relapses over a median clinical follow-up of 93 months (range: 12-518 months). Nine patients (10%) developed active lesions over median 84 months radiologic follow-up (range: 12-518 months). Active lesions occurred in 24% PUHMS, 5% PSS, and 3% PPS cohorts. CONCLUSION: New inflammatory activity, defined by active lesions and clinical relapses following motor progression in patients with critical demyelinating lesions, is low. Disease-modifying therapies that reduce demyelinating relapses and active MRI lesions are of uncertain benefit in these cohorts.

Rapid and robust generation of functional oligodendrocyte progenitor cells from epiblast stem cells.

Myelin-related disorders such as multiple sclerosis and leukodystrophies, for which restoration of oligodendrocyte function would be an effective treatment, are poised to benefit greatly from stem cell biology. Progress in myelin repair has been constrained by difficulties in generating pure populations of oligodendrocyte progenitor cells (OPCs) in sufficient quantities. Pluripotent stem cells theoretically provide an unlimited source of OPCs, but current differentiation strategies are poorly reproducible and generate heterogenous populations of cells. Here we provide a platform for the directed differentiation of pluripotent mouse epiblast stem cells (EpiSCs) through defined developmental transitions into a pure population of highly expandable OPCs in 10 d. These OPCs robustly differentiate into myelinating oligodendrocytes in vitro and in vivo. Our results demonstrate that mouse pluripotent stem cells provide a pure population of myelinogenic oligodendrocytes and offer a tractable platform for defining the molecular regulation of oligodendrocyte development and drug screening.

Evaluation of a new device to treat negative dysphotopsia.

PURPOSE: To evaluate the efficacy and safety of Negative Dysphotopsia (ND) Ring implantation for treating and preventing ND. SETTING: Multicentric tertiary eye hospitals. DESIGN: Prospective interventional cohort study. METHODS: 22 patients with ND were enrolled. Eyes with other ocular structural pathologies (corneal, macular, optic nerve head, retinal, neuro-ophthalmological) were excluded. In 15 eyes, the ring was implanted to treat ND (therapeutic group), and in 7 eyes, it was implanted during cataract surgery of the fellow eye as a prophylactic measure (prophylactic group) to prevent the occurrence of ND. Preoperative evaluation included video recording of the patients' complaints aside complete eye examination. Postoperatively, patients were interviewed to confirm resolution of complaints related to ND. The intraoperative difficulties and postoperative adverse events were recorded. A minimum follow-up of 1 year was completed for all eyes. RESULTS: In the therapeutic group, 14 of 15 eyes (93.3%) patients reported complete resolution of ND on the first postoperative day while 1 patient reported persistence of a smaller and lighter temporal shadow. No ND was reported by any patient in any of the 7 eyes treated prophylactically. No significant adverse intraoperative event was recorded; however, vitreous upthrust was noted in 2 of 22 eyes (9.0%). No permanent drop in visual acuity was recorded in the therapeutic group. CONCLUSIONS: ND Ring implantation was an easy, safe, and effective approach for both treating and preventing ND.

MR Imaging Features of Critical Spinal Demyelinating Lesions Associated with Progressive Motor Impairment.

BACKGROUND AND PURPOSE: Progressive MS is typically heralded by a myelopathic pattern of asymmetric progressive motor weakness. Focal individual "critical" demyelinating spinal cord lesions anatomically associated with progressive motor impairment may be a compelling explanation for this clinical presentation as described in progressive solitary sclerosis (single CNS demyelinating lesion), progressive demyelination with highly restricted MR imaging lesion burden (2-5 total CNS demyelinating lesions; progressive paucisclerotic MS), and progressive, exclusively unilateral hemi- or monoparetic MS (>5 CNS demyelinating progressive unilateral hemi- or monoparetic MS [PUHMS] lesions). Critical demyelinating lesions appear strikingly similar across these cohorts, and we describe their specific spinal cord MR imaging characteristics. MATERIALS AND METHODS: We performed a retrospective, observational MR imaging study comparing spinal cord critical demyelinating lesions anatomically associated with progressive motor impairment with any additional "noncritical" (not anatomically associated with progressive motor impairment) spinal cord demyelinating lesions. All spinal cord MR images (302 cervical and 91 thoracic) were reviewed by an experienced neuroradiologist with final radiologic assessment on the most recent MR imaging. Anatomic association with clinical progressive motor impairment was confirmed independently by MS subspecialists. RESULTS: Ninety-one individuals (PUHMS, 37 [41%], progressive paucisclerosis 35 [38%], progressive solitary sclerosis 19 [21%]) with 91 critical and 98 noncritical spinal cord MR imaging demyelinating lesions were evaluated. MR imaging characteristics that favored critical spinal cord demyelinating lesions over noncritical lesions included moderate-to-severe, focal, lesion-associated spinal cord atrophy: 41/91 (45%) versus 0/98 (0%) (OR, 161.91; 9.43 to >999.9); lateral column axial location (OR, 10.43; 3.88-28.07); central region (OR, 3.23; 1.78-5.88); ventral column (OR, 2.98; 1.55-5.72); and larger lesion size of the axial width (OR, 2.01;1.49-2.72), transverse axial size (OR, 1.66; 1.36-2.01), or lesion area (OR, 1.14; 1.08-1.2). Multiple regression analysis revealed focal atrophy and lateral axial location as having the strongest association with critical demyelinating lesions. CONCLUSIONS: Focal, lesion-associated atrophy, lateral column axial location, and larger lesion size are spinal cord MR imaging characteristics of critical demyelinating lesions. The presence of critical demyelinating lesions should be sought as these features may be associated with the development of progressive motor impairment in MS.

Adult-onset Coats disease.

Coats disease is an idiopathic retinal vasculopathy characterized by telangiectasia and aneurysm of retinal vessels along with intra and subretinal exudation and fluid. While Coats disease is classically described in young male population, there is an adult variant of Coats disease presenting in adulthood. Adult onset Coats disease have a similar presentation but a slower progression, localised lipid deposition, both peripheral and juxta-macular involvement. In this review article, we have attempted to describe in detail the characteristic clinical features, pathogenesis, investigation modalities and treatment in adult-onset Coats disease.

Anthropometry of deep-set eyes with respect to difficulty in docking during femtosecond laser procedures.

Purpose: To identify the facial anthropometric parameters that predict the difficulty during femtosecond (FS) laser. Methods: This was a: single-center observational study was conducted on participants between the ages 18 and 30 years who were planned for FS-LASIK (femtosecond laser-assisted laser in situ keratomileusis) or SMILE (small incision lenticule extraction) at Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India. The front and side-facing images of the participants were analyzed using Image J software to measure different anthropometric parameters. The nasal bridge index, facial convexity, and other parameters were measured. The difficulty faced by the surgeon during docking was recorded for each subject. The data were analyzed on Stata 14. Results: A total of 97 subjects were included. The mean age was 24 (±7) years. Twenty-three (23.71%) subjects were females while the rest were males. Difficulty in docking was seen in 1 (4.34%) female and 14 (19%) males. The mean nasal bridge index was 92.58 (±4.01) in subjects with deep-set eyes and 89.72 (±4.30) in normal subjects. The mean total facial convexity was 129.28 (±4.24) in deep-set eyes, and 140.23 (±4.74) in normal subjects. Conclusion: Total facial convexity appeared as the most important feature, with the value being less than 133° in most subjects with unfavorable facial anthropometry.

Cerebrospinal fluid evaluation in patients with progressive motor impairment due to critical central nervous system demyelinating lesions.

BACKGROUND: Elevated intrathecal immunoglobulin G (IgG; oligoclonal bands (OCBs)) or IgG in people with progressive motor impairment due to "critical" demyelinating lesions are of uncertain significance. OBJECTIVE: Compare clinical/radiological features of people with "critical" demyelinating lesion-induced progressive motor impairment with/without elevated intrathecal IgG synthesis. METHODS: A total of 133 people with progressive motor impairment attributable to "critical" demyelinating lesions (corticospinal tract location, consistent with the progressive motor deficit) were compared regarding clinical and radiological presentation with and without ≥2 unique cerebrospinal fluid (CSF) OCB and/or IgG index ≥0.85. RESULTS: Ninety-eight (74%) had CSF-elevated OCB and/or IgG index, higher with increased magnetic resonance imaging-lesion burden. No differences were found with/without CSF abnormalities in sex (46 of 98 female (47%) vs. 22 of 35 (63%), p = 0.11), onset-age (median 49 vs. 50 years, p = 0.5), progression from onset (62 of 98 (63%) vs. 25 of 35 (71%)), progression post-relapse (36 of 98 (37%) vs. 10 of 35 (29%), p = 0.4), and duration between demyelinating disease onset and CSF examination (30 (0-359) vs. 48 (0-323) months p = 0.7). "Critical" lesions were radiologically similar, most commonly cervical spine located (72 of 98 (74%) vs. 19 of 35 (54%), p = 0.18) both with/without CSF abnormalities. CONCLUSIONS: People with "critical" demyelinating lesion-induced progressive motor impairment typically have elevated intrathecal IgG (OCB and/or IgG) and similar clinical and radiological presentation regardless of CSF findings, therefore representing valid presentations of progressive demyelinating disease.

Pediatric idiopathic macular hole - A case report and review of literature.

Macular hole in the pediatric age group is usually post-traumatic unlike, the adult counterpart. Herein we describe a case of idiopathic macular hole occurring in a 9-year-old male who presented to us with complaints of diminution of vision in OD (oculus dextrus) for 2 months. The child underwent 25-gauge pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling. On follow-up his best-corrected visual acuity improved with type 1 closure of macular hole.

Role of ultrasonography in childhood eye diseases in a tertiary care setting: indications and scope.

We sought to evaluate the role and diagnostic potential of ocular B-scan ultrasonography in childhood eye disease in an observational cross-sectional study; 1091 patients with a total of 1445 eyes examined were studied. Cataract was the single most common indication for ultrasound followed by corneal pathology, ocular trauma, posterior segment pathology, primary congenital glaucoma, leukocoria, orbital pathology and other disease. Ultrasonography resulted in a change in diagnosis in 198 cases (18%). We conclude that B-scan ultrasonography plays an important adjunctive role in the management of childhood eye disease.

Oral hygiene practices among paramedical staff of a private dental institution in India

Introduction: Health promotion in dentistry is targeted at the two most common oral diseases - dental caries and periodontal disease. The maintenance of good oral hygiene is considered to be a central issue in oral health promotion. Objective: To assess the oral hygiene practices among paramedical staff of a private dental institution in India. Material and methods: A cross sectional questionnaire survey was undertaken among 100 paramedical staff of a private dental institution in Mangalore, India. Data was collected by using self designed questionnaire. Results: About 49% of the subjects brushed thrice with soft toothbrush and 55% changed their tooth brush once in 3 months. About 77% of them cleaned their tongue either by tongue cleaner, toothbrush or finger and 82% of the subjects used tooth paste. About 56% of the subjects had never used dental floss. Majority of the subjects (56%) visited the dentist only when required. Conclusion: Oral hygiene practices among the paramedical staff were poor.