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Stiff-person syndrome (SPS) is a rare and complex neurologic disorder characterized by progressive muscle stiffness, painful spasms, and gait difficulties. In this report, we describe a case of SPS who presented with a relapse while on maintenance immunosuppressive treatment. In addition, we review the literature of 16 previously reported cases of SPS from India, highlighting the diverse clinical features, comorbidities, treatment response, and relapse. The occurrence of paraneoplastic SPS emphasizes the need for early recognition and diagnosis. [ABSTRACT FROM AUTHOR]

Stiff-person syndrome (SPS) is a rare and complex neurologic disorder characterized by progressive muscle stiffness, painful spasms, and gait difficulties. In this report, we describe a case of SPS who presented with a relapse while on maintenance immunosuppressive treatment. In addition, we review the literature of 16 previously reported cases of SPS from India, highlighting the diverse clinical features, comorbidities, treatment response, and relapse. The occurrence of paraneoplastic SPS emphasizes the need for early recognition and diagnosis.

The article analyses the anniversary of a regional capital as a tool of identity politics. From the point of view of an identity approach, an event of this kind can act as a catalyst for reflection, have a positive impact on the involvement of residents in solving city problems, and integrate the community. The anniversary is considered as a symbolic practice of purposefully constructing the identity discourse that ensures integration of the urban/regional community and solves the problems of positioning the territory. The key content aspects of the jubilee discourse have been identified: image, infrastructure, and identity. If the first two aspects are aimed at the utilitarian economic effect, reinforcing the popularity of the territory and the real changes in the urban landscape, the identity perspective captures the meanings associated with understanding oneself as a community. Using the example of the 300th anniversary of Perm, the rhetoric of key actors in identity politics has been analysed from the perspective of these aspects. The author concludes that in the case of Perm, the anniversary was considered mainly as a tool for solving image problems. The agenda of constructing identity occupied a subordinate place in relation to the promotion issue. The anniversary was linked to the possibility of understanding urban and regional identity. However, that trend did not translate into a broad public discussion among intellectuals.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a relapsing–remitting neurological disorder that falls within the stiff person syndrome–spectrum disorders. We report a 16-year-old girl with PERM associated with an anti-glutamic acid decarboxylase (GAD) antibody. She had an aggressive initial presentation mimicking fulminant septic shock, followed by truncal and lower limb rigidity, stimulus-sensitive spasm, cognitive impairment, brainstem signs (hyperekplexia, nystagmus), and dysautonomia (urinary retention, constipation, facial flushing, blood pressure fluctuation). Cerebrospinal fluid, electroencephalography, and magnetic resonance imaging of the brain and spine showed features suggestive autoimmune encephalitis and myelitis. The serum anti-GAD antibody was positive, and the diagnosis of PERM was made. She had fluctuating clinical response despite intravenous immunoglobulin, steroids, plasmapheresis, and symptomatic medications. Eventually, in the fourth month since admission, she showed gradual and persistent clinical improvement after introducing rituximab. She was discharged after 6 months of hospitalization, and no relapse was observed in the first 3 years of follow-up. PERM is a rare and underrecognized condition in children. Contrary to previous reports, our case describes an aggressive and life-threatening presentation for PERM. Vague symptoms and the lack of gold diagnostic tests hinder a timely diagnosis. Our study also highlights the need for developing standardized diagnostic criteria and consensus in managing PERM. [ABSTRACT FROM AUTHOR]

Abstract Background Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological condition with paraneoplastic etiology in about 20% of cases, usually presenting before or shortly after the oncological diagnosis is established. PERM associated with anti-glycine receptor antibodies is not previously reported in a patient with bladder cancer. Case presentation A 72-years-old Caucasian male was admitted with acute onset of dysarthria, dysphagia and trismus three years after initial surgical treatment for bladder cancer. The condition was initially diagnosed as tetanus and treated accordingly, but the diagnosis was reconsidered because of progression despite adequate treatment. Diagnostic workup on readmission revealed lung and paraaortal metastases from bladder cancer and anti-glycine receptor (anti-GlyR) antibodies both in the cerebrospinal fluid and in serum, which supplemented with the clinical presentation led to the diagnosis of PERM, presumably related to bladder cancer. The patient showed improvement and stabilization after treatment with intravenous immunoglobulin and chemotherapy against metastatic bladder cancer. Conclusion To the best of our knowledge, this is the first reported case of anti-GlyR antibody positive PERM related to urothelial carcinoma. The symptoms mimicked tetanus, and responded to chemotherapy and immunotherapy.

AbstractPrimary healthcare (PHC) has become a concern in most resource constraint economies particularly in the global south where meeting this important economic need has become a burden. Given this, blockchain technology (BT), a promising transformation in contemporary service delivery, has become an alternative for service-oriented institutions to meet their desired goals. In primary healthcare, BT has been utilised to lessen the burden on medical supply chain and data management. Nonetheless, the technology seems to be a preserve of the developed economies. In the global south, not only has the complexities of the technology hindered the implementation of the technology but the understanding of its affordances has also been nascent. This study extends Task-technology fit model with the perceived e-readiness model. Drawing on the best-worst method, this paper investigates PHC managers’ decision to embrace BT for PHC delivery in Ghana. The study finds that, in order of relevance, task-technology, infrastructure and individual characteristics are the main drivers of BT adoption and implementation. The study proposes the implementation of various strategies relevant to ensuring a sustainable BT for the management of PHC in resource constraint economies.

Background: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological condition with paraneoplastic etiology in about 20% of cases, usually presenting before or shortly after the oncological diagnosis is established. PERM associated with anti-glycine receptor antibodies is not previously reported in a patient with bladder cancer. Case presentation: A 72-years-old Caucasian male was admitted with acute onset of dysarthria, dysphagia and trismus three years after initial surgical treatment for bladder cancer. The condition was initially diagnosed as tetanus and treated accordingly, but the diagnosis was reconsidered because of progression despite adequate treatment. Diagnostic workup on readmission revealed lung and paraaortal metastases from bladder cancer and anti-glycine receptor (anti-GlyR) antibodies both in the cerebrospinal fluid and in serum, which supplemented with the clinical presentation led to the diagnosis of PERM, presumably related to bladder cancer. The patient showed improvement and stabilization after treatment with intravenous immunoglobulin and chemotherapy against metastatic bladder cancer. Conclusion: To the best of our knowledge, this is the first reported case of anti-GlyR antibody positive PERM related to urothelial carcinoma. The symptoms mimicked tetanus, and responded to chemotherapy and immunotherapy. [ABSTRACT FROM AUTHOR]

Primary healthcare (PHC) has become a concern in most resource constraint economies particularly in the global south where meeting this important economic need has become a burden. Given this, blockchain technology (BT), a promising transformation in contemporary service delivery, has become an alternative for service-oriented institutions to meet their desired goals. In primary healthcare, BT has been utilised to lessen the burden on medical supply chain and data management. Nonetheless, the technology seems to be a preserve of the developed economies. In the global south, not only has the complexities of the technology hindered the implementation of the technology but the understanding of its affordances has also been nascent. This study extends Task-technology fit model with the perceived e-readiness model. Drawing on the best-worst method, this paper investigates PHC managers' decision to embrace BT for PHC delivery in Ghana. The study finds that, in order of relevance, task-technology, infrastructure and individual characteristics are the main drivers of BT adoption and implementation. The study proposes the implementation of various strategies relevant to ensuring a sustainable BT for the management of PHC in resource constraint economies. [ABSTRACT FROM AUTHOR]

This article is a reply to a letter discussing the relationship between infections and Stiff-Person Spectrum Disorders (SPS). The authors of the reply appreciate the valuable information provided by the letter and acknowledge the case of SPS with cerebellar features following SARS-CoV-2 infection. They note that the primary inquiry is the nature of the link between SARS-CoV-2 infection and the development of neurological disorders. While specific triggers for SPS disorders are still unknown, there is evidence supporting an association between SARS-CoV-2 infection and GAD autoimmunity in Type 1 Diabetes Mellitus. The authors suggest that further research is needed to understand post-COVID-19 autoimmune disorders, including SPS. [Extracted from the article]

This paper provides an overview of the study of reindeer husbandry and vocabulary associated with it on the material of the Uralic languages. The article is based on the methods of etymological and comparative analysis. The source base of the research was the dictionary materials and personal observations of the author. The purpose of this work is to analyze the reindeer herding vocabulary in the context of the linguistic contacts of the Ural peoples. It is noted that the systematic study of various aspects of reindeer husbandry based on the material of different ethnic groups began in the second half of the 20th century. The most important for the analysis are the materials of the Samoyed languages, in particular the Nenets, whose influence extended to adjacent linguistic continua. There is a section on reindeer husbandry in the context of interethnic and interlingual contacts, since the latter is often the result of economic and interethnic contacts. In a number of cases, the Nenets were the source of distribution of certain methods of using reindeer. An attempt was made to comprehensively analyze the Northern Russian dialect borrowed data, possible etymons that are recorded in the Komi language, the Baltic-Finnish, Ob-Ugric languages, as well as in the Samoyed languages. The interaction of the Samoyed, Perm and Ob-Ugric continuums is noted on the data of the vocabulary of reindeer husbandry.

The article is devoted to the identification of groups of factors that artificially overstated or, on the contrary, underestimated the results of the punitive policy of the state to strengthen labor discipline during the Great Patriotic War in the industrial sector of the Soviet economy. The study is carried out on the basis of an analysis of both the changed legislative framework in the studied area and the office documentation of the Committees of the Communist Party in the Molotov region. Studying the real practice of applying laws in certain large enterprises of the Molotov region, the author concludes that there is a discrepancy between the number of actual labor crimes and the number of workers convicted of them.

BackgroundProgressive encephalomyelitis with rigidity and myoclonus (PERM) is a subtype of stiff-person syndrome, a rare cerebrospinal disease that causes brainstem symptoms, myoclonus, muscle rigidity, and hyperekplexia.Case presentationA 71-year-old man experienced left-sided stiff face, and was subsequently admitted to our hospital because of the appearance of left-dominant lower limb myoclonus. Muscle rigidity followed 3 days later. Magnetic resonance imaging revealed no abnormality. An electrophysiological examination showed a toughness of the antagonistic muscle following evocation of the Achilles tendon reflex, and a tonic phenomenon affecting the left facial muscles during the blink reflex. The patient's serum was positive for anti-glycine receptor (anti-GlyR) antibody, suggesting PERM. The patient was administered steroids, immunoglobulin therapy, and immunosuppressive drugs. He gradually improved after these therapies and became able to walk using a walker.ConclusionsWe conclude that this was a rare case of anti-GlyR antibody-positive PERM with unilateral brainstem symptoms, myoclonus, and muscle rigidity.

In our patient, the diagnosis of autoimmune GFAP astrocytopathy was confirmed by the presence of lymphocytic meningoencephalitis and GFAP antibodies in both his serum and CSF. Keywords: autoimmune encephalitis; GFAP; PERM; antibody EN autoimmune encephalitis GFAP PERM antibody 827 829 3 05/18/23 20230501 NES 230501 Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a rare inflammatory brain disease that more commonly presents with meningoencephalitis and myelitis. [Extracted from the article]

Brainstem syndrome, PERM, SARS-CoV-2, stiff-person syndrome, COVID-19 1.5 T gadolinium-enhancement brain magnetic resonance imaging (MRI) was unremarkable at 2 and 4 weeks after symptoms onset. Keywords: PERM; brainstem syndrome; stiff-person syndrome; SARS-CoV-2; COVID-19 EN PERM brainstem syndrome stiff-person syndrome SARS-CoV-2 COVID-19 824 826 3 05/18/23 20230501 NES 230501 A spectrum of neurological manifestations has been described as possible complication of SARS-CoV-2 disease, either through direct or indirect mechanisms. [Extracted from the article]

Although it was Kalkowsky (1908) who coined the term "stromatolite", Freiesleben (1809) and perhaps Hausmann (1805) had described stromatolitic structures a century earlier. They had found them at the top of the Zechstein Limestone of the Upper Permian Zechstein Group around the Harz Mountains. Similar stromatolites have also been found in the Zechstein Limestone of Thuringia where they form the frame-builders of large reefs. They have been known since the middle of the nineteenth century, but were originally misidentified as fossil sponges, stromatoporoids or calcareous algae. [ABSTRACT FROM AUTHOR]

Time indications for the correlation and calibration of the Rotliegend of Germany are integrated and presented in a new way. The correlations are based on the visual balance of bio- and ecostratigraphic indications and radio-isotopic age determinations (RIA), whereby confidence limits, equivalent to those of the RIA, are applied also for other time indications (conchostracans, aquatic tetrapods, fishes, insects, macroflora, and ecostratigraphic indications). In contrast, terrestrial tetrapods and their tracks, chemo- and sequence stratigraphy, and magnetic susceptibility did not contribute to correlations in the Rotliegend in Central Europe. The Illawarra Reversal of the Earth' magnetic field (≈ 265 Ma) is fundamental for correlation and calibration. In general, however, the Regional Stratigraphic Scale is calibrated using U-Pb CA-ID-TIMS-RIA on zircon, Ar-Ar- and Rb-Sr-based RIA and, in addition, in the Early and Late Rotliegend using orbital-climatically controlled eccentricity cycles (≈ 100 ka, ≈ 400 ka). The boundary ages of the subgroups and of most formations are rounded to 0.5 Ma to avoid overinterpretation. Because of some inconsistent time indications alternative interpretations are also offered. The hitherto differing ages for the global Carboniferous-Permian boundary are the result of different dating methods: The ≈ 296 Ma in the Stratigraphic Tables of Germany 2002 and 2016 A (STG 2002; STG 2016 A) is an integrative age based on RIA from Central Europe, whereas the ≈ 299 Ma in the Global Stratigraphic Scales 2004 ff. and the STG 2016 B is based on a CA-ID-TIMS RIA on zircon from the southern Ural area. The age of ≈ 299 Ma is plausible from the perspective of the current Rotliegend RIA because the Rb-Sr age of 290.7 ± 0.9 Ma for the Donnersberg Formation (Lippolt & Hess 1989) has been replaced by the age of 294.5 ± 2.2 Ma, as recalculated in the present work. The updated time scale developed here will be integrated in the Stratigraphische Tabelle von Deutschland Kompakt 2022 (STDK 2022) and into the Stratigraphic Table of Germany Compact 2022 (STGC 2022). The age of the base of the Rotliegend in the Saar-Nahe Basin (SN, base Remigiusberg Formation and Glan Subgroup) is ≈ 299 Ma (U-Pb CA-ID-TIMS, zircon) resp. ≈ 300 Ma (Ar-Ar); the balanced compromise age of both is ≈ 299.5 Ma. In the Thüringer Wald (TW), the Rotliegend begins with the Ilmenau Formation (Subkommission Perm-Trias 2011) at ≈ 299.5 Ma (U-Pb CA-ID-TIMS). On the Flechtingen Block, at the southern margin of the Central European Basin, the oldest volcanic rock of the Altmark Subgroup is dated at 302 ± 3 Ma (U-Pb SHRIMP data). Accordingly, the Rotliegend in the STG 2002 and STG 2016 A starts at ≈ 302 Ma. However, the mean age calculated in the present work for the SHRIMP data of Breitkreuz & Kennedy (1999) is 298.6 ± 1.9 Ma, so that the base of the Altmark volcanic succession is here re-dated as ≈ 300.5 Ma. Thus, the ages of the present lithostratigraphic lower limits of the Rotliegend in northern Germany (CEB), in the TW and in the SN are, with ≈ 300.5 Ma to ≈ 299.5 Ma, only weakly diachronous if the nominal (face) values, which are both in the latest Carboniferous, are compared without the confidence intervals. For the first time, the Rotliegend of Germany is geochronologically subdivided into Early (≈ 300.5-295.5/294 Ma), Middle (≈ 295.5/294-266 Ma) and Late Rotliegend (≈ 266-257.5 Ma). The very long lasting Middle Rotliegend includes numerous and also extremely long stratigraphic gaps as a result of the amalgamation and the associated immense uplift of Pangaea in the area of Central and Western Europe. A very large stratigraphic gap in the Central European Basin (CEB) of ≈ 15 Ma (≈ 281-266 Ma, here called the Pangaea Gap) is supported by conchostracans, the Illawarra Reversal and the different palaeomagnetic properties of the sediments below and above the gap, which are discussed here for the first time. The Middle Rotliegend (duration of ≈ 29.5/28 Ma) is approx. twice as long as the Early (≈ 5.5/7 Ma) and Late Rotliegend (≈ 8.5 Ma) combined. The latter, however, contain the thickest successions in the Saar-Nahe Basin and the Central European Basin, respectively, which are free of gaps in the basin centre, with 'gaps' being defined in this context as time spans of ≤ 100 ka and ≤ 400 ka, respectively. In the CEB, the Elbe Subgroup is cyclostratigraphically calibrated to 5.6 Ma. A gapless area in the Saar-Nahe Basin is the Glan Subgroup, which covers only 4 Ma and 3 Ma, respectively; correspondingly, its subunits G1 to G10 (Glan 1 to Glan 10), introduced here, last on average 0.4 Ma and 0.3 Ma, respectively. This results in a maximum accumulation rate of 870 Bubnoff (Bub, m/Ma, mm/ka) and of 1150 Bub, respectively, which can be explained by a maximum of tectonic activity along the Hunsrück Southern Border Fault. For the lower Nahe Subgroup (N1 to N5, Nahe 1 to Nahe 5), the maximum accumulation rates are ≈ 450-200 Bub and for the upper Nahe Subgroup (N6-N8), at an estimated duration of 12 Ma ≈ 125 Bub and ≈ 50 Bub at a duration of 30 Ma, respectively. In the Central European Basin the maximum accumulation rates are much lower compared to the Glan Subgroup, with 200 Bub in the Elbe Subgroup and ≈ 360 Bub in the Havel Subgroup (Fig. 27), although here the subsidence rate may have been higher than the accumulation rate, as the basin was ≈ 130 m below sea level at the end of Folge ro4 (Top of the Hannover Formation; this paper). We recommend (1) Bubnoff (Bub) as unit of measure of the accumulation rate and (2) the stratigraphic terminology of the Subkommission Perm-Trias (2011) and the STG 2016 A. We here propose that numerous tuffs of the Glan Subgroup could originate from volcanic centres of the TW, whereas so far only source areas south of the SN were discussed: On the one hand, the tuff-rich time of the upper Jeckenbach Subformation - Odernheim Formation (= Glan units 7 and 8 = G7/G8) seems to correlate with the volcanogenic Oberhof time in the TW and, on the other hand, the tuff-poor time of the Wahnwegen Formation - middle Jeckenbach Subformation (G2/G6) seems to correlate with the upper Ilmenau, Manebach, Goldlauter and lowest Oberhof formations, which are devoid of volcanic rocks in the TW. Should causality exist here, an additional and also precise criterion for correlation would be established. At present there are two variants: On the one hand, volcanism in the Oberhof Formation, according to RIA (U-Pb CA-ID-TIMS on zircon), begins ≈ 1.8 Ma earlier and ends ≈ 0.8 Ma earlier than in the Donnersberg Formation (Ar-Ar, Rb- Sr recalculated). On the other hand, the conchostracan Lioestheria pseudotenella in the TW, if considered equivalent to L. cf. pseudotenella in the SN, the ecostratigraphic and lithofacial indications and the aquatic tetrapod D. pulcherrimus, point to for a similar age of these magmatites. The Sobernheim Lens (SN, Nahe units N4/N5) with its fossils so important for correlations is here assigned to the Jakobsweiler Member (N4, upper Donnersberg Formation), to the Quartzite-Conglomerate (N5, in literature often Wadern) and to the lowermost Standenbühl Formation (N4/N5). The youngest time indication Co13, the conchostracan Lioestheria arroyoensis sp. nov. correlates the upper Standenbühl Formation (N8, Martens 2020, where N8 may be extremely long), roughly with the Arroyo Formation in Texas (≈ early Kungurian). The here favoured age of the Tambach Formation of ≈ 294-292 Ma is significantly higher than the ≈ 286-283 Ma of STG 2016 A and the ≈ 284-281 Ma of STG 2016 B. It is based on RIA R25 (Table 9; lower Rotterode Formation, 295.8 ± 0.4 Ma; Lützner et al. 2021), which is the youngest CA-ID-TIMS RIA in the TW, and the duration estimate of the Rotterode - Tambach gap. In the SN, the youngest RIA R29 (Rb/Sr) is from the Donnersberg Formation (294.5 ± 2.2 Ma, this paper). From here on until ≈ 265 Ma (Illawarra Reversal), the numerical ages of all Rotliegend units and their connection to the Global Stratigraphic Scale (GSS) are uncertain and therefore correlations between Central Europe and the global reference section in the southern Ural area, partly biostratigraphically via North America, vary greatly. In this contect, the „series“ Wolfcampian and Leonardian (standard Permian units of the USA) played an important role. However, these lithostratigraphic units are at least unsuitabel as a reference system for large parts of North America, because they represent very variable time spans from region to region, similar with the "Lower Rotliegend" ("Autun") and "Upper Rotliegend" ("Saxon") of Central and Western Europe and therefore their assignement to the GSS is also very variable. This is due, among other things, to the fact that the Wolfcampian is often still understood as "lower Lower Permian", although considerable parts of it are now placed in the Carboniferous, because as a result of the introduction of the Global Stratigraphic Section and Point (GSSP) for the base of the Asselian Stage (= global base of the Permian in the southern Ural area), the conventional regional Carboniferous-Permian boundaries shifted to an higher stratigraphic level and thus to a younger age in the USA, China and the Donbass (Fig. 14). If the 57 sedimentary cycles in the Ottweiler and Glan subgroups (Königer & Stollhofen 2001: mean duration 245 ka each) in the SN are orbital-climatically induced, these cycles can only be 100 ka cycles (short eccentricity). This is also supported by the 6 cycles in units G7 and G8 that have been described in detail in the literature, which are consistent with the duration of the Glan Subgroup of 3 Ma and (preferred) 4 Ma, respectively. The total duration to be expected from 57 cycles à ≈ 100 ka is ≈ 5.7 Ma in contrast to the duration determined here according to RIA of ≤ 8 Ma. One reason for the difference could be that in the Ottweiler und Glan subgroups there are cycles that have not been recorded so far. The time span of ≤ 8 Ma is also an outcome of the new estimate for the duration of the Cantabrian Gap (early Stephanian) in the Saar-Nahe Basin of ≈ 3.6 Ma that is clearly longer than proposed in the STG 2002 and STG 2016. [ABSTRACT FROM AUTHOR]

The cored drill Bad Vilbel Kurpark 2005 for the first time produced a considerable profile in the Rotliegend of Bad Vilbel (N Frankfurt), allowing for a more precise diagnosis of the lithofacies and the sedimentary environment. However, no detailed determination is achieved on whether this facies must be assigned to the Duedelsheim Formation or to the Altenstadt Formation of the Wetterau Basin - or to both formations, as hitherto. Furthermore, it is of importance that the drill has penetrated 20 m of pre-Permian, Variscan clay and silt slates as well as litharenites ("greywackes") in a palaeogeographic position in between the outcrops of the Rhenohercynian in the N and the Mid-German Crystalline Zone in the S. These pre-Permian rocks can be assigned to the complex of the Giessen Nappe. [ABSTRACT FROM AUTHOR]

The airport terminals in Perm, Saratov and Kemerovo created by Asadov Architectural bureau are presented. General concepts of complexes, architectural and interior solutions for passenger terminals, as well as elaboration of design codes of administrative and technical buildings are given. Particular attention is paid to passenger comfort and safety.

The airport terminals in Perm, Saratov and Kemerovo created by Asadov Architectural bureau are presented. General concepts of complexes, architectural and interior solutions for passenger terminals, as well as elaboration of design codes of administrative and technical buildings are given. Particular attention is paid to passenger comfort and safety.

Background: Stiff person syndrome (SPS) is an autoimmune condition involving antibodies against several components of the inhibitory synapse in the spinal cord, with glutamic acid decarboxylase antibodies being the predominant immune marker. SPS affects approximately 1 patient per million population per year. The effect of intravenous immunoglobulin (IVIG) has been established, but studies on the long‐term efficacy of regular IVIG are limited. Objectives: To review clinical details and long‐term treatment response using a patient‐reported questionnaire in SPS and related syndromes. Methods: Patients were identified from a tertiary neuroimmunology clinic based on classical clinical symptoms, autoimmune profiles, and neurophysiological changes (Dalakas criteria). They were followed up after treatment to assess the response to IVIG. Results: A total of 23 patients fulfilled the selection criteria. Patients' demographic profiles and clinical presentations were akin to that reported in literature. There was significant improvement in the functional ability (assessed by the modified Rankin scale [mRS]) and quality of life (QoL) following treatment with IVIG within 4 to 10 weeks (pre‐mRS vs. post‐mRS, P < 0.0001; pre‐QoL vs. post‐QoL, P = 0.0003) and sustained after 5 years of treatment (pre‐mRS vs. present mRS, P = 0.0003; pre‐QoL vs. present QoL, P = 0.0002). Conclusions: This article describes one of the largest single‐center experiences of 23 patients with SPS and related syndromes and is the first to establish the long‐term efficacy of regular IVIG using a patient‐reported scoring system (Birmingham Response to Immunomodulatory Therapy [BRIT]). Consistent improvement in QoL and functional scores were seen over nearly 5 years after regular use of IVIG. It is recommended to use BRIT scores to assess the initial response as well as to monitor continued improvement to immunomodulation in SPS. [ABSTRACT FROM AUTHOR]

Efficient collection systems and information about the characteristics and quality of collected secondary plastic waste flows are of fundamental importance for the development of circular economies. In order to assess the effectiveness of the implementation of separate collection systems for plastic packaging, especially polyethylene terephthalate (PET) bottles, characteristic of the collected PET bottles in street mesh containers were studied in the city of Perm, Russia. The share of extraneous fractions was assessed and differentiation was carried out by volume, type of product, label presence, shape, content of solid and liquid impurities and colour. These results indicate that PET composition in different seasons is very similar, despite the assumption that the consumption of PET bottles in the spring and autumn seasons varies. In the mesh containers, up to 34% of the items were foreign objects, considering that only PET bottles should be collected. In each dimensional flow of PET bottles, the proportion of transparent bottles prevailed; it ranged from 31% to 70%. Based on the results of the experiment, almost all PET bottle categories had a standard shape, except packaging for food products and household chemicals, in which 26–27% of PET bottles had a non-standard shape. The results about charactersitic of source-separated PET bottles are fundamental for goal-oriented design and implementation of collection, recycling technologies, secondary separation facilities, the economics of recycling intitatives and reverse vending machines for collecting materials. [ABSTRACT FROM AUTHOR]

Abstract Background Progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome is a rare neurological condition. Its clinical characteristics include axial and limb muscle rigidity, myoclonus, painful spasms and hyperekplexia. Diagnosis of this disease can be very challenging and optimal long-term treatment is unclear. Case presentation We report a case of a 62 year old patient admitted for repetitive myoclonus and rigidity in the lower limbs progressing since 10 years, associated with a fluctuating encephalopathy requiring stays in Intensive Care Unit. Multiple diagnostics and treatment were proposed, unsuccessfully, before the diagnosis of PERM syndrome was established. In association with the clinical presentation, a strong positive result for GAD (glutamic acid decarboxylase) antibodies lead to the diagnosis of PERM syndrome. Conclusions PERM syndrome is a rare disease and its diagnosis is not easy. Once the diagnosis is established, the correct treatment should follow and could be lifesaving, regardless of a delayed diagnosis. Maintenance of long-term oral corticotherapy is suggested to prevent relapses.

Austroalpine Saualpe basement in Carinthia (Austria, Eastern Alps) display a polymetamorphic microstructural and mineral-chemical evolution. SEM automated mineralogy methods and analysis by EPMA were applied for garnet mineralchemical characterisation and monazite dating. Two garnet generations with monophase and polyphase porphyroblasts and two monazite age populations have been revealed in low-Ca and high-Al-metapelites. Garnet 1 in both Kliening and Preims complexes has decreasing Mn, low Ca and significantly increasing Mg from cores to rims. Geothermobarometry of garnet 1 assemblages signals a crystallisation along a M1 prograde metamorphic P-T path culminating at 600-650 °C/7 kbar, followed by decompression. In the Preims Complex a subsequent crystallisation of a ~265 Ma monazite 1 population with high-Y and high-Gd indicates a Permian minimum age for the M1 metamorphic event. Monazite 1 crystallisation coincides with the intrusion of Permian and Early Triassic pegmatites. In the Kliening Complex where pegmatites are lacking, no Permian monazite is observed. Resorption of garnet 1 rims and corona structures of apatite and allanite around Permian monazite in the Preims Complex signal a retrogressive stage. An M2 metamorphic event with garnet 2 postdates the monazite corona structures. Garnet 2 displays always low Mn at high Ca and Mg. The XCa is increasing and then decreasing, while XMg is decreasing and then increasing toward the rims. The garnet 2 assemblages record a prograde P-T path from 530 °C/8 kbar to 700 °C/10-13 kbar in the Kliening Complex, and from 620 °C/10-12 kbar to 700 °C/14-15 kbar in the Preims Complex. Monazite 2 populations with ages between 95 and 81 Ma and with lower Y and Gd contents crystallised at decreasing pressure subsequent to garnet 2 and indicate a Cretaceous (Eo-Alpine) age for the metamorphic event M2. In the histogram view, the monazite age pattern in the Preims Complex and the overlying Saualpe Eclogite Complex are quite similar with Permian and Cretaceous age maxima. These patterns differ from other Austroalpine basement areas with unimodal Carboniferous or bimodal Carboniferous and Permian monazite age populations. [ABSTRACT FROM AUTHOR]

The dialogue of major reform in immigration laws along with increase in rejection of H-1B petitioners since the start of the Trump administration may make employers find H-1B renewal becoming more difficult and costly for low-end positions and less-educated employees. Using the Program Electronic Review Management (PERM) application data, this article examines the impact of the recent threat of immigration reform on the share of entry-level positions and petitioners with no college degree in Green Card applications. We found evidence that entry-level positions and applicants without a college degree became more heavily represented among new PERM applications after 2016, even when a trend and occupations were accounted for. The findings are consistent with the hypothesis that employers face stronger incentive to retain workers and sponsor their PERM applications since H-1B renewal will likely become more difficult and costly for low-end positions and less-educated workers. [ABSTRACT FROM AUTHOR]

Aims: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a life‐threatening condition often associated with highly raised serum antibodies to glycine receptors (GlyRs); these bind to the surface of large neurons and interneurons in rodent brain and spinal cord sections and, in vitro, inhibit function and reduce surface expression of the GlyRs. The effects in vivo have not been reported. Methods: Purified plasma IgG from a GlyR antibody‐positive patient with PERM, and a healthy control (HC), was injected daily into the peritoneal cavity of mice for 12 days; lipopolysaccharide (LPS) to open the blood–brain barrier, was injected on days 3 and 8. Based on preliminary data, behavioural tests were only performed 48 h post‐LPS on days 5–7 and 10–12. Results: The GlyR IgG injected mice showed impaired ability on the rotarod from days 5 to 10 but this normalized by day 12. There were no other behavioural differences but, at termination (d13), the GlyR IgG‐injected mice had IgG deposits on the neurons that express GlyRs in the brainstem and spinal cord. The IgG was not only on the surface but also inside these large GlyR expressing neurons, which continued to express surface GlyR. Conclusions: Despite the partial clinical phenotype, not uncommon in passive transfer studies, the results suggest that the antibodies had accessed the GlyRs in relevant brain regions, led to antibody‐mediated internalization and increased GlyR synthesis, compatible with the temporary loss of function. [ABSTRACT FROM AUTHOR]

This is a proposal to encode the Old Permic script in the international character encoding standard Unicode. Old Permic was published in Unicode Standard version 7.0 in June 2014. The script was devised in the 14c to write the Komi and Komi-Permyak languages. It was used for Komi until the 17c. It was also used cryptographically from the 15c to write Russian.

The Vikings’ rise in Northern Russia is a chapter in European history that is yet to be written. Political, linguistic and mental barriers have meant that this period of Northern Russia’s great expansion was sadly neglected by historians. But after 1990, with the opening of Russia’s archives for western researches, we can finally begin collecting the small pieces for a jigsaw puzzle of the Vikings’ journey in the area of the Kola Peninsula. Recent archaeological finds help us to support the theory that the Viking voyages in the White Sea and the Arctic Ocean have had greater significance and extent than previously thought. This article will shed new light on Bjarmaland, the Bjarmians and why the Vikings for many centuries pilgrimage to the area where Arkhangelsk is today. Were they in the area at all? And how there still are traces in our modern society of this era. Furthermore, the trade connections between Vikings, Bjarmians and Russians will be put into a larger perspective and the Vikings’ trade routes in both Eastern and Western Europe will be described.

September 2016 marks 95 years since the foundation of Dermatology and Venereology Department in Perm State Medical University. This article provides information on its organization, formation and development, the brightest and the most outstanding representatives of the department, main directions of scientific, educational and therapeutic activities.

Stiff-person syndrome (SPS), first described in 1956 by Moersch and Woltman, is a progressive autoimmune disorder with core features of chronic fluctuating progressive truncal and limb rigidity and painful muscle spasms leading to gait difficulties, falls and an appearance that resembles tin soldiers. The syndrome is a rare, highly disabling disorder of the central nervous and frequently results in significant disability. Understanding of the etiology, clinical spectrum, diagnostic workup and therapeutic modalities for this painful and disabling disorder has vastly evolved over the past few years with more confidence in classifying and treating the patients. The purpose of this review is to increase the awareness, early detection, and treatment of this disabling disease. PubMed was searched, all date inclusive, using the following phrases: stiff person syndrome,anti-Glutamic acid decarboxylase (Anti-GAD) antibody syndrome, Progressive encephalomyelitis with rigidity and myoclonus (PERM), and Paraneoplastic Stiff Person syndrome. No filters or restrictions were used. A total of 888 articles were identified. The results were narrowed to 190 citations after excluding non-English and duplicate reports. Clinical presentation, laboratory testing, treatment, and prognosis were categorized and summarized. In this article we will discuss the epidemiology, presentation and classification. Explain the pathophysiology of SPS and the autoimmune mechanisms involved. Discuss the diagnostic approach and treatments available, as well as, the prognosis and outcome. • Introducing the understanding of the pathophysiology of Stiff person syndrome. • Illustrating the classification of Stiff person syndrome. • Explaining the diagnostic steps for Stiff person syndrome. • Explaining the main features differentiating Stiff person syndrome from other similar disorders. • Introducing a step-wise therapeutic approach to Stiff person syndrome. [ABSTRACT FROM AUTHOR]

In a 184-year-old stand of Scots pine Рinus sylvestris L. of the 1st class of bonitet on two plots on an area of 2.2 ha with a stand height of 32.1 and 33.5 m, average diameters of 39.8 and 42.1 cm, and a fullness of 0.91 and 0.95, candidates for plus trees (hereinafter referred to as candidates) with an excess of 30 % in diameter were studied. All live (735 pieces), and fall away trees for 14 years were plotted on the plan with an accuracy of ± 10–35 cm. To determine the age, we took cores from 40 trees with diameters of 30–61 cm. On one of the two sites, the feeding area of 326 trees aged ≈ 170 years and the maximum feed area was 79.9, the minimum was 5.1, the average value was (25.8 ± 0.58) m2. The average area of their nutrition was 19.7 % larger in the sample of 18 candidates, than in the sample of ordinary trees, but the difference is not significant. Polygons represented the area that the tree inherited at the age of 170 years and it was used as a factor determining the size of the tree in 184 years. It was found that it affected the diameter of trees with a force of 7.7 % and to the height of the trunk with a strength of 0.0–2.4 %. The age of the trees ranged from 173 to 195 years, candidates were both from 174 years to 193 years. Probably, either they had rapid growth due to genetics, or there are exceptionally favorable places in the biotope, where the pine settled later and not only caught up with its neighbors, but also reached an outstanding size. Perhaps such places may be due to the influence of lithology. It was concluded that for selection purposes, trees of maximum age with the largest feeding area should be rejected from candidates.

Background: Atypical Parkinsonian syndromes with prominent frontal lobe involvement can occur in the 4R-taupathies progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Secondary forms of movement disorders may occur in the context of autoimmune encephalitis with antineuronal antibodies, such as anti-glycine receptor (anti-GlyR) antibodies, which are typically associated with Stiff-Person spectrum syndrome, or progressive encephalomyelitis with rigidity and myoclonus. Overlaps between neurodegenerative and immunological mechanisms have been recently suggested in anti-IgLON5 disease. In this case study, the authors describe a patient with a Parkinsonian syndrome with frontal lobe involvement and anti-GlyR antibodies. Case presentation: The patient presented was a 63-year-old female. Her symptoms had begun with insomnia at the age of 60, after which, since the age of 61, increasing personality changes developed, leading to a diagnosis of depression with delusional symptoms. Severe cognitive deficits emerged, along with a left-side accentuated Parkinsonian syndrome with postural instability. The personality changes involved frontal systems. Magnetic resonance imaging (MRI) showed low-grade mesencephalon atrophy. [18F]fluorodeoxyglucose positron emission tomography (FDG PET) depicted a moderate hypometabolism bilateral frontal and of the midbrain, while [123I]FPCIT single-photon emission computed tomography (SPECT) revealed severely reduced dopamine transporter availability in both striata, indicating pronounced nigrostriatal degeneration. In addition, anti-GlyR antibodies were repeatedly found in the serum of the patient (max. titer of 1:640, reference: Conclusion: The patient presented here was suffering from a complex Parkinsonian syndrome with frontal lobe involvement. Because of the high anti-GlyR antibody titers, the presence of an autoimmune cause of the disorder was discussed. However, since no typical signs of autoimmune anti-GlyR antibody syndrome (e.g., hyperexcitability, anti-GlyR antibodies in CSF, or other inflammatory CSF changes) were detected, the possibility that the anti-GlyR antibodies might have been an unrelated bystander should be considered. Alternatively, the anti-GlyR antibodies might have developed secondarily to neurodegeneration (most likely a 4-repeat tauopathy, PSP or CBD) without exerting overt clinical effects, as in cases of anti-IgLON5 encephalopathy. In this case, such antibodies might also potentially modify the clinical course of classical movement disorders. Further research on the role of antineuronal antibodies in Parkinsonian syndromes is needed.

Objectives: To evaluate the stability of RNA and microRNA (miRNA) in PAXgene-fixed paraffinembedded tissue blocks after 7 years' storage. Methods: RNA and miRNA were extracted from PAXgene-fixed paraffin-embedded (PFPE) blocks in 2009 then stored at -80°C. Seven years later, RNA and miRNA were again extracted from the same blocks. RNA and miRNA integrity in the 2009 and 2016 extractions were compared using RNA integrity number (RIN), paraffinembedded RNA metric (PERM), reverse transcription polymerase chain reaction (RT-PCR) for different amplicon lengths, and quantitative RT-PCR (qRT-PCR) for three mRNA and three miRNA targets. Results: In PFPE blocks, mRNA was poorer in 2016 extractions compared to the 2009 extractions in all blocks and all assays applied, with transcripts degrading at different rates in the same blocks. For miRNA, qRT-PCR showed no statistically significant differences between 2009 and 2016 extractions. Conclusions: mRNA in PFPE tissue blocks degrades at room temperature storage over 7 years. [ABSTRACT FROM AUTHOR]

Immune mediated movement disorders include movement disorders in the context of autoimmune encephalitis such as anti-NMDAR encephalitis, post-infectious autoimmune movement disorders such as Sydenham chorea, paraneoplastic autoimmune movement disorders such as opsoclonus myoclonus ataxia syndrome, and infection triggered conditions such as paediatric acute neuropsychiatric syndrome. This review focuses on the approach to treatment of immune mediated movement disorders, which requires an understanding of the immunopathogenesis, whether the disease is destructive or ‘altering’, and the natural history of disease. Factors that can influence outcome include the severity of disease, the delay before starting therapy, use of multimodal therapy and whether the course is monophasic or relapsing. Although the four main conditions listed above have different pathophysiological processes, there are general themes that broadly apply including: early diagnosis and treatment is better, minimise the severity of disease, escalate treatment if the patient is not responding to initial treatments, and minimise relapse. [ABSTRACT FROM AUTHOR]

Carboniferous sedimentary and volcanic rocks occur in several small occurrences in the Black Forest (Southwestern Germany). Early Carboniferous flyschoid greywackes, andesitic to dacitic volcanics, and nonmarine molasse-type clastics are restricted to the Badenweiler-Lenzkirch Zone of the southern Black Forest and have been heavily faulted and partially folded. They have been dated by invertebrate and plant fossils from the sediments and few radiometric data from the volcanics. Late Carboniferous nonmarine deposits with tuff layers, minor coal measures and plant fossils are scattered through the central and northern Black Forest in several small occurrences, mostly representing the latest Carboniferous initial sedimentary fill of different extensional basins that further evolved into Permian Rotliegend internal molasse basins. Only the early Late Carboniferous Berghaupten Formation has some different structural position, since it has been folded and thrusted by gneiss in late Carboniferous time, just before the extensional Carboniferous to Permian basins initiated. [ABSTRACT FROM AUTHOR]