Argyrophilic grain disease in individuals younger than 75 years: clinical variability in an under-recognized limbic tauopathy.

BACKGROUND AND PURPOSE: Argyrophilic grain disease (AGD) is a limbic-predominant 4R-tauopathy. AGD is thought to be an age-related disorder and is frequently detected as a concomitant pathology with other neurodegenerative conditions. There is a paucity of data on the clinical phenotype of pure AGD. In elderly patients, however, AGD pathology frequently associates with cognitive decline, personality changes, urine incontinence and cachexia. In this study, clinicopathological findings were analysed in individuals younger than 75. METHODS: Patients were identified retrospectively based on neuropathological examinations during 2006-2017 and selected when AGD was the primary and dominant pathological finding. Clinical data were obtained retrospectively through medical records. RESULTS: In all, 55 patients (2% of all examinations performed during that period) with AGD were identified. In seven cases (13%) AGD was the primary neuropathological diagnosis without significant concomitant pathologies. Two patients were female, median age at the time of death was 64 years (range 51-74) and the median duration of disease was 3 months (range 0.5-36). The most frequent symptoms were progressive cognitive decline, urinary incontinence, seizures and psychiatric symptoms. Brain magnetic resonance imaging revealed mild temporal atrophy. CONCLUSIONS: Argyrophilic grain disease is a rarely recognized limbic tauopathy in younger individuals. Widening the clinicopathological spectrum of tauopathies may allow identification of further patients who could benefit from tau-based therapeutic strategies.

OCT measurements on the foveal and perifoveal retinal thickness on diabetic patients after phacoemulsification and IOL implantation.

PURPOSE: The first aim (I.) was to measure the change of macular thickness in diabetic (DM group) and nondiabetic patients (NORMAL group), the values of operated eye (OP) were compared I.A.) to the fellow, non-operated eye (CONTROL) and I.B.) to preoperative baseline value of the operated eye (PRE). The second aim (II.) was to compare postoperative macular thickening of DM group to NORMAL group eyes. METHODS: Retinal thickness values of the foveal and perifoveal (fovea+3.0 mm, fovea+6.0 mm) sectors and the minimum values were measured in a prospective study using optical coherence tomography (OCT) in 18 eyes of 18 diabetic patients and 53 eyes of 53 nondiabetic patients who underwent uneventful cataract surgery. Data were collected on the preoperative day and on postoperative 1, 7, 30, and 60 days. Student's t-test was used for statistical analysis. RESULTS: No significant change of the thickness values could be measured one day after surgery. However, a significant increase could be detected on the postoperative 7, 30, and 60 days in the perifoveal 3.0 mm and 6.0 mm sectors either calculated alone or averaged together with the foveal values. The initial (preoperative) average value of 232.7+/-3.5 microm (mean+/-SEM, n=144) in the 6.0 mm perifoveal region increased to 246.0+/-8.2 microm (mean+/-SEM, n=120, P=0.032) one week, to 249.9+/-5.1 microm (n=112, P=0.017) one month, and to 249.5+/-6.2 microm (n=104, P=0.009) 2 months after surgery. DISCUSSION: Our results indicate that diabetes did not influence significantly the thickening of the macular regions after uncomplicated cataract surgery.

Change of foveal and perifoveal thickness measured by OCT after phacoemulsification and IOL implantation.

PURPOSE: Cataract surgery is known to elicit postoperative macular oedema owing to its traumatic effect. The aim of the study was to measure the foveal and perifoveal thickness of the retina after uneventful phacoemulsification and posterior chamber intraocular lens (PC-IOL) implantation. METHODS: Retinal thickness values of the foveal and perifoveal (fovea+3.0 mm, fovea+6.0 mm) sectors and the minimum values were measured in a prospective study using optical coherence tomography (OCT) in 71 eyes of 71 patients (34 men, 37 women, mean age: 68.8 years) who underwent cataract surgery. Phacoemulsification and 'in the bag' implantation of a foldable PC-IOL was performed under topical anaesthesia. Data were collected 1 day before the operation, and postoperatively at 1, 7, 30, and 60 days. Contralateral eyes of each patient served as controls. 'Student's t-test' was used for statistical analysis, and P<0.05 value was considered significant. RESULTS: No significant change of the thickness values could be measured 1 day after surgery. However, a significant increase could be detected on the postoperative 7, 30, and 60 days in the perifoveal 3.0 and 6.0 mm sectors either calculated alone or averaged together with the foveal values. The initial (preoperative) average value of 234.1+/-2.6 microm (mean+/-SEM, n=536) in the 6.0 mm perifoveal region increased to 242.5+/-2.6 microm (mean+/-SEM, n=488, P<0.01) 1 week, to 247.7+/-4.6 microm (n=352, P<0.01) 1 month, and to 246.0+/-5.9 microm (n=208, P<0.05) 2 months after surgery, which proved to be significant. However, the relative change of macular thickness was moderate, 3.5, 5.6, and 5.3% at the above periods, respectively. In a subgroup of patients (n=34) minimum 6 months after cataract surgery, the 6.0 mm perifoveal values were found to be elevated at (237.8+/-3.9 microm, mean+/-SEM, n=272) as compared to the control eyes (233.9+/-3.8 microm, mean+/-SEM, n=272). The difference was 1.7% and proved not to be significant (P>0.05). CONCLUSIONS: Mild subclinical perifoveal oedema following cataract surgery was detected using OCT at postoperative day 7 to 6 months. The minimal and the foveal values in itself are inadequate to describe the phenomenon. The macular thickness values of the perifoveal 3.0 and 6.0 mm sectors alone or averaged together with the foveal values provide more accurate description of the postoperative macular oedema.OCT enables the detection of minimal increases in perifoveal retinal thickness even 6 months after cataract surgery.

Phacoemulsification on previously vitrectomized eyes: results of a 10-year-period.

PURPOSE: To provide an overview of intraoperative and postoperative complications during phacoemulsification cataract surgery and to evaluate the visual results in patients having pars plana vitrectomy (PPV) with 10 years of follow-up. METHODS: Retrospective evaluation of intraoperative and postoperative complications and postoperative visual results in 134 consecutive patients who underwent phacoemulsification after PPV. Of 11,498 eyes treated with phacoemulsification, 143 (1.2%) had previous PPV during the 10-year period were included in this study. RESULTS: The phacoemulsification procedure seemed to be difficult where there was a deep or fluctuating anterior chamber (93%) and primary posterior capsule opacification (19%). The most frequent intraoperative complications were posterior capsule rupture (9%) and incomplete capsular rhexis (5%). Postoperative intraocular pressure elevation (7%), retinal detachment (6%), and posterior capsule opacification (8%) occurred most frequently during the mean follow-up period of 18.2 months (1.5-110 months). Best-corrected visual acuity (BCVA) increased two or more Snellen E lines in 55% of the cases or became better than or equal to 0.5 in 10% of the cases. CONCLUSIONS: Despite the well-known difficulties encountered in vitrectomized eyes such as zonular damage, increased mobility of the lens-iris diaphragm, and altered intraocular fluid dynamics, phacoemulsification proved to be a safe procedure in the hands of experienced surgeons.

Bayesian analyses of admixture in wild and domestic cats (Felis silvestris) using linked microsatellite loci.

Methods recently developed to infer population structure and admixture mostly use individual genotypes described by unlinked neutral markers. However, Hardy-Weinberg and linkage disequilibria among independent markers decline rapidly with admixture time, and the admixture signals could be lost in a few generations. In this study, we aimed to describe genetic admixture in 182 European wild and domestic cats (Felis silvestris), which hybridize sporadically in Italy and extensively in Hungary. Cats were genotyped at 27 microsatellites, including 21 linked loci mapping on five distinct feline linkage groups. Genotypes were analysed with structure 2.1, a Bayesian procedure designed to model admixture linkage disequilibrium, which promises to assess efficiently older admixture events using tightly linked markers. Results showed that domestic and wild cats sampled in Italy were split into two distinct clusters with average proportions of membership Q > 0.90, congruent with prior morphological identifications. In contrast, free-living cats sampled in Hungary were assigned partly to the domestic and the wild cat clusters, with Q < 0.50. Admixture analyses of individual genotypes identified, respectively, 5/61 (8%), and 16-20/65 (25-31%) hybrids among the Italian wildcats and Hungarian free-living cats. Similar results were obtained in the past using unlinked loci, although the new linked markers identified additional admixed wildcats in Italy. Linkage analyses confirm that hybridization is limited in Italian, but widespread in Hungarian wildcats, a population that is threatened by cross-breeding with free-ranging domestic cats. The total panel of 27 loci performed better than the linked loci alone in the identification of domestic and known hybrid cats, suggesting that a large number of linked plus unlinked markers can improve the results of admixture analyses. Inferred recombination events led to identify the population of origin of chromosomal segments, suggesting that admixture mapping experiments can be designed also in wild populations.

Effects of pollution from personal computers on perceived air quality, SBS symptoms and productivity in offices.

UNLABELLED: In groups of six, 30 female subjects were exposed for 4.8 h in a low-polluting office to each of two conditions--the presence or absence of 3-month-old personal computers (PCs). These PCs were placed behind a screen so that they were not visible to the subjects. Throughout the exposure the outdoor air supply was maintained at 10 l/s per person. Under each of the two conditions the subjects performed simulated office work using old low-polluting PCs. They also evaluated the air quality and reported Sick Building Syndrome (SBS) symptoms. The PCs were found to be strong indoor pollution sources, even after they had been in service for 3 months. The sensory pollution load of each PC was 3.4 olf, more than three times the pollution of a standard person. The presence of PCs increased the percentage of people dissatisfied with the perceived air quality from 13 to 41% and increased by 9% the time required for text processing. Chemical analyses were performed to determine the pollutants emitted by the PCs. The most significant chemicals detected included phenol, toluene, 2-ethylhexanol, formaldehyde, and styrene. The identified compounds were, however, insufficient in concentration and kind to explain the observed adverse effects. This suggests that chemicals other than those detected, so-called 'stealth chemicals', may contribute to the negative effects. PRACTICAL IMPLICATIONS: PCs are an important, but hitherto overlooked, source of pollution indoors. They can decrease the perceived air quality, increase SBS symptoms and decrease office productivity. The ventilation rate in an office with a 3-month-old PC would need to be increased several times to achieve the same perceived air quality as in a low-polluting office with the PC absent. Pollution from PCs has an important negative impact on the air quality, not only in offices but also in many other spaces, including homes. PCs may have played a role in previously published studies on SBS and perceived air quality, where PCs were overlooked as a possible pollution source in the indoor environment. The fact that the chemicals identified in the office air and in the chamber experiments were insufficient to explain the adverse effects observed during human exposures illustrates the inadequacy of the analytical chemical methods commonly used in indoor air quality investigations. For certain chemicals the human senses are much more sensitive than the chemical methods routinely used in indoor air quality investigations. The adverse effects of PC-generated air pollutants could be reduced by modifications in the manufacturing process, increased ventilation, localized PC exhaust, or personalized ventilation systems.

Genetic distinction of wildcat (Felis silvestris) populations in Europe, and hybridization with domestic cats in Hungary.

The genetic integrity and evolutionary persistence of declining wildcat populations are threatened by crossbreeding with widespread free-living domestic cats. Here we use allelic variation at 12 microsatellite loci to describe genetic variation in 336 cats sampled from nine European countries. Cats were identified as European wildcats (Felis silvestris silvestris), Sardinian wildcats (F. s. libyca) and domestic cats (F. s. catus), according to phenotypic traits, geographical locations and independently of any genetic information. Genetic variability was significantly partitioned among taxonomic groups (FST = 0.11; RST = 0.41; P < 0.001) and sampling locations (FST = 0.07; RST = 0.06; P < 0.001), suggesting that wild and domestic cats are subdivided into distinct gene pools in Europe. Multivariate and Bayesian clustering of individual genotypes also showed evidence of distinct cat groups, congruent with current taxonomy, and suggesting geographical population structuring. Admixture analyses identified cryptic hybrids among wildcats in Portugal, Italy and Bulgaria, and evidenced instances of extensive hybridization between wild and domestic cats sampled in Hungary. Cats in Hungary include a composite assemblage of variable phenotypes and genotypes, which, as previously documented in Scotland, might originate from long lasting hybridization and introgression. A number of historical, demographic and ecological conditions can lead to extensive crossbreeding between wild and domestic cats, thus threatening the genetic integrity of wildcat populations in Europe.

Electrical cataract and optic neuropathy.

We report on a 20 year old male, who developed mature cataract (Electrical Cataract) on both eyes after a severe electric shock from high voltage. Both of his hands and the left foot had to be amputated, because of the severe injury. Although electrical cataract due to high voltage or lightning is rare, they may still occur after industrial or home electric accidents. Even if extracapsular cataract extraction with intraocular lens implantation is successfully performed, the final visual acuity will depend on other ocular damage due to the electric current.

Results and complications of secondary intraocular lens implantation.

The results and the postoperative complications of 107 secondary intraocular lens implantations were reviewed over a two-year period. In 84.1% of the cases, postoperative visual acuity was the same or better than preoperative visual acuity with aphakic glasses or contract lenses. The most frequent postoperative complications were secondary glaucoma, six cases (5.6%); cystoid macular edema, five cases (4.6%); and bullous keratopathy, three cases (2.8%).

Effect of 3-methylcholanthrene on the protein composition of heterogeneous nuclear ribonucleoprotein particles in developing rat liver.

Nine-day-old rats were treated with a single 100 mg/kg body weight intraperitoneal dose of 3-methylcholanthrene (3-MC). 24, 48, 72 and 120 h later the protein composition of the heterogeneous nuclear ribonucleoprotein particles derived from the liver was analysed. The analysis was performed by urea-sodium dodecyl sulphate polyacrylamide gel electrophoresis. The protein composition of the hnRNP particles was compared to that derived from control animals of similar ages. The 35 000-Mr polypeptide which is normally absent found in the hnRNP particles of the young rats prior to the 11th day of life appears on the 10th day after 24 h 3-MC exposition. In addition, the minor polypeptide components of the particles were also found in higher quantity. The polypeptide pattern of the hnRNP particles derived from treated animals resembled those of the older animals but not those of controls of similar ages. Thus, the apparent accelerating effect of 3-MC on the liver development is also valid at the level of hnRNP particles.

Comparison of heterogeneous nuclear ribonucleoprotein particles of the developing rat liver.

hnRNP monoparticles were isolated from livers of newborn (1 day), 7, 10, 11, 14, 21, 28 days old and adult rats, and their sedimentation properties, buoyant densities and protein composition were compared. There were observable differences neither in sedimentation nor in buoyant density among the particles isolated from livers of rats of different ages. The hnRNP particles derived from every stage of ages sedimented in the 30-50S zone of sucrose gradient with a peak at about 38S. Glutaraldehyde fixed particles in CsCl gradients banded at 1.36 g/cm3 which corresponds to about 80% of protein in the particles. Urea/sodium dodecyl sulfate/polyacrylamide gel electrophoresis of hnRNP proteins demonstrated 19 polypeptides in the 35,000-140,000 Mr range. Three main polypeptides in hnRNP of every group of animals could be observed with apparent Mr of 39,000, 43,000 and 46,000. In the minor components only quantitative differences could be seen in the different age groups with the exception of a 35,000-Mr polypeptide which appeared on the 11th day after the birth.