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2. In‐depth analysis of data from the RAS‐ALS study reveals new insights in rasagiline treatment for amyotrophic lateral sclerosis.

Author

Schuster, Joachim, Dreyhaupt, Jens, Mönkemöller, Karla, Dupuis, Luc, Dieterlé, Stéphane, Weishaupt, Jochen H., Kassubek, Jan, Petri, Susanne, Meyer, Thomas, Grosskreutz, Julian, Schrank, Berthold, Boentert, Matthias, Emmer, Alexander, Hermann, Andreas, Zeller, Daniel, Prudlo, Johannes, Winkler, Andrea S., Grehl, Torsten, Heneka, Michael T., and Johannesen, Siw

Subjects
AMYOTROPHIC lateral sclerosis, SINGLE nucleotide polymorphisms, DATA analysis, RANDOMIZED controlled trials, STATISTICAL power analysis
Abstract

Background and purpose: In 2016, we concluded a randomized controlled trial testing 1 mg rasagiline per day add‐on to standard therapy in 252 amyotrophic lateral sclerosis (ALS) patients. This article aims at better characterizing ALS patients who could possibly benefit from rasagiline by reporting new subgroup analysis and genetic data. Methods: We performed further exploratory in‐depth analyses of the study population and investigated the relevance of single nucleotide polymorphisms (SNPs) related to the dopaminergic system. Results: Placebo‐treated patients with very slow disease progression (loss of Amyotrophic Lateral Sclerosis Functional Rating Scale–Revised [ALSFRS‐R] per month before randomization of ≤0.328 points) showed a per se survival probability after 24 months of 0.85 (95% confidence interval = 0.65–0.94). The large group of intermediate to fast progressing ALS patients showed a prolonged survival in the rasagiline group compared to placebo after 6 and 12 months (p = 0.02, p = 0.04), and a reduced decline of ALSFRS‐R after 18 months (p = 0.049). SNP genotypes in the MAOB gene and DRD2 gene did not show clear associations with rasagiline treatment effects. Conclusions: These results underline the need to consider individual disease progression at baseline in future ALS studies. Very slow disease progressors compromise the statistical power of studies with treatment durations of 12–18 months using clinical endpoints. Analysis of MAOB and DRD2 SNPs revealed no clear relationship to any outcome parameter. More insights are expected from future studies elucidating whether patients with DRD2CC genotype (Rs2283265) show a pronounced benefit from treatment with rasagiline, pointing to the opportunities precision medicine could open up for ALS patients in the future. [ABSTRACT FROM AUTHOR]

Published
2024
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4. Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial

Author

Funke, Andreas, Kettemann, Dagmar, Meyer, Robert, Meyer, Thomas, Grehl, Torsten, Gruhn, Kai, Schwenkreis, Peter, Stude, Philipp, Heneka, Michael T, Kurzwelly, Delia, Hermann, Andreas, Storch, Alexander, Richter, Nicole, Frank, Tobias, Göricke, Bettina, Hein, Katharina, Emmer, Alexander, Hanisch, Frank, Hanke, Dagmar, Kraya, Torsten, Posa, Andreas, Romanakova, Martina, Schilling, Susanne, Abdulla, Susanne, Böselt, Sebastian, Janssen, Claas, Lange, Imken, Kobeleva, Xenia, Körner, Sonja, Kollewe, Katja, Osmanovic, Alma, Petri, Susanne, Scharn, Nicole, Rath, Klaus J, Dahms, Christiane, Grosskreutz, Julian, Gunkel, Anne, Heiling, Bianka, Ringer, Thomas, Smesny, Uta, Baumeister, Sarah, Berthele, Achim, Bublitz, Sarah, Winkler, Andrea S, Akova-Öztürk, Esra, Boentert, Matthias, Stubbe-Dräger, Bianca, Rahmann, Alexandra, Young, Charlotte, Young, Peter, Baldaranov, Dobri, Bogdahn, Ulrich, Wollebæk Johannesen, Siw, Khomenko, Andrei, Schulte-Mattler, Wilhelm, Stadler, Christina, Husung, Susanne, Prudlo, Johannes, Tesar, Simone, Dargah-Zaden, Nigar, Last, Christina, Langer, Eva, Ludolph, Albert C, Weishaupt, Jochen H, Weiland, Ulrike, Lauenstein, Ann-Sophie, Lensch, Eckard, Mc Farlane, Carolyn, Fischer-Brasse, Heike, Orbán, Klara, Schrank, Bertold, Schürger, Sonja, Klebe, Stephan, Kraft, Peter, Musacchio, Thomas, Seiler, Carola, Zeller, Daniel, Schuster, Joachim, Dorst, Johannes, Dupuis, Luc, Dreyhaupt, Jens, Kassubek, Jan, and Schrank, Berthold

Published
2018
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7. Real-world data on eculizumab treatment in NMOSD: high efficacy and potential challenges (P13-5.017)

Author

Ayzenberg, Ilya, primary, Asseyer, Susanna, additional, Lindenblatt, Gero, additional, Fischer, Katinka, additional, Pul, Refik, additional, Özalp, Sinem-Hilal, additional, Lohmann, Lisa, additional, Giglhuber, Katrin, additional, Häussler, Vivien, additional, Karenfort, Michael, additional, Hellwig, Kerstin, additional, Paul, Friedemann, additional, Bellmann-Strobl, Judith, additional, Otto, Carolin, additional, Ruprecht, Klemens, additional, Ziemssen, Tjalf, additional, Emmer, Alexander, additional, Rothhammer, Veit, additional, Nickel, Florian, additional, Angstwurm, Klemens, additional, Linker, Ralf, additional, Warnke, Clemens, additional, Jarius, Sven, additional, Korporal-Kuhnke, Mirjam, additional, Wildemann, Brigitte, additional, Wolff, Stephanie, additional, Seipelt, Maria, additional, Yalachkov, Yavor, additional, Retzlaff, Nele, additional, Zettl, Uwe, additional, Rommer, Paulus, additional, Kowarik, Markus, additional, Wickel, Jonathan, additional, Geis, Christian, additional, Hümmert, Martin W., additional, Trebst, Corinna, additional, Senel, Makbule, additional, Klotz, Luisa, additional, Gold, Ralf, additional, Kleinschnitz, Christoph, additional, Meuth, Sven, additional, Aktas, Orhan, additional, Berthele, Achim, additional, and Ringelstein, Marius, additional

Published
2023
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8. Clinical and genetic features of amyotrophic lateral sclerosis patients with C9orf72 mutations

Author

Wiesenfarth, Maximilian, primary, Günther, Kornelia, additional, Müller, Kathrin, additional, Witzel, Simon, additional, Weiland, Ulrike, additional, Mayer, Kristina, additional, Herrmann, Christine, additional, Brenner, David, additional, Schuster, Joachim, additional, Freischmidt, Axel, additional, Lulé, Dorothée, additional, Meyer, Thomas, additional, Regensburger, Martin, additional, Grehl, Torsten, additional, Emmer, Alexander, additional, Petri, Susanne, additional, Großkreutz, Julian, additional, Rödiger, Annekathrin, additional, Steinbach, Robert, additional, Klopstock, Thomas, additional, Reilich, Peter, additional, Schöberl, Florian, additional, Wolf, Joachim, additional, Hagenacker, Tim, additional, Weyen, Ute, additional, Zeller, Daniel, additional, Ludolph, Albert C, additional, and Dorst, Johannes, additional

Published
2023
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9. Spectrum and frequency of genetic variants in sporadic amyotrophic lateral sclerosis

Author

Ruf, Wolfgang P, primary, Boros, Matej, additional, Freischmidt, Axel, additional, Brenner, David, additional, Grozdanov, Veselin, additional, de Meirelles, Joao, additional, Meyer, Thomas, additional, Grehl, Torsten, additional, Petri, Susanne, additional, Grosskreutz, Julian, additional, Weyen, Ute, additional, Guenther, Rene, additional, Regensburger, Martin, additional, Hagenacker, Tim, additional, Koch, Jan C, additional, Emmer, Alexander, additional, Roediger, Annekathrin, additional, Steinbach, Robert, additional, Wolf, Joachim, additional, Weishaupt, Jochen H, additional, Lingor, Paul, additional, Deschauer, Marcus, additional, Cordts, Isabell, additional, Klopstock, Thomas, additional, Reilich, Peter, additional, Schoeberl, Florian, additional, Schrank, Berthold, additional, Zeller, Daniel, additional, Hermann, Andreas, additional, Knehr, Antje, additional, Günther, Kornelia, additional, Dorst, Johannes, additional, Schuster, Joachim, additional, Siebert, Reiner, additional, Ludolph, Albert C, additional, and Müller, Kathrin, additional

Published
2023
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10. Epstein-Barr Virus-Induced Genes and Endogenous Retroviruses in Immortalized B Cells from Patients with Multiple Sclerosis

Author

Wieland, Lisa, primary, Schwarz, Tommy, additional, Engel, Kristina, additional, Volkmer, Ines, additional, Krüger, Anna, additional, Tarabuko, Alexander, additional, Junghans, Jutta, additional, Kornhuber, Malte E., additional, Hoffmann, Frank, additional, Staege, Martin S., additional, and Emmer, Alexander, additional

Published
2022
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13. GFPT1-Associated Congenital Myasthenic Syndrome Mimicking a Glycogen Storage Disease – Diagnostic Pitfalls in Myopathology Solved by Next-Generation-Sequencing

Author

Mensch, Alexander, primary, Cordts, Isabell, additional, Scholle, Leila, additional, Joshi, Pushpa Raj, additional, Kleeberg, Kathleen, additional, Emmer, Alexander, additional, Beck-Woedl, Stefanie, additional, Park, Joohyun, additional, Haack, Tobias B., additional, Stoltenburg-Didinger, Gisela, additional, Zierz, Stephan, additional, and Deschauer, Marcus, additional

Published
2022
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16. Surfactant Protein-G in Wildtype and 3xTg-AD Mice: Localization in the Forebrain, Age-Dependent Hippocampal Dot-like Deposits and Brain Content

Author

Meinicke, Anton, primary, Härtig, Wolfgang, additional, Winter, Karsten, additional, Puchta, Joana, additional, Mages, Bianca, additional, Michalski, Dominik, additional, Emmer, Alexander, additional, Otto, Markus, additional, Hoffmann, Karl-Titus, additional, Reimann, Willi, additional, Krause, Matthias, additional, and Schob, Stefan, additional

Published
2022
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17. Surfactant protein C is associated with perineuronal nets and shows age-dependent changes of brain content and hippocampal deposits in wildtype and 3xTg mice

Author

Schob, Stefan, primary, Puchta, Joana, additional, Winter, Karsten, additional, Michalski, Dominik, additional, Mages, Bianca, additional, Martens, Henrik, additional, Emmer, Alexander, additional, Hoffmann, Karl-Titus, additional, Gaunitz, Frank, additional, Meinicke, Anton, additional, Krause, Matthias, additional, and Härtig, Wolfgang, additional

Published
2021
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18. Overexpression of Endogenous Retroviruses and Malignancy Markers in Neuroblastoma Cell Lines by Medium-Induced Microenvironmental Changes

Author

Wieland, Lisa, Engel, Kristina, Volkmer, Ines, Krüger, Anna, Posern, Guido, Kornhuber, Malte E., Staege, Martin S., and Emmer, Alexander

Subjects
neuroblastoma, Oncology, endogenous retrovirus, viruses, CD200, biomarker, CD133, Original Research, human endogenous retrovirus
Abstract

Neuroblastoma (NB) is the commonest solid tumor outside the central nervous system in infancy and childhood with a unique biological heterogeneity. In patients with advanced, metastasizing neuroblastoma, treatment failure and poor prognosis is often marked by resistance to chemo- or immunotherapy. Thus, identification of robust biomarkers seems essential for understanding tumor progression and developing effective therapy. Here, we have studied the expression of human endogenous retroviruses (HERV) as potential targets in NB cell lines during stem-cell medium-induced microenvironmental change. Quantitative PCR revealed that relative expression of the HERV-K family and HERV-W1 ENV were increased in all three NB cell lines after incubation in stem-cell medium. Virus transcriptome analyses revealed the transcriptional activation of three endogenous retrovirus elements: HERV-R ENV (ERV3-1), HERV-E1 and HERV-Fc2 ENV (ERVFC1-1). Known malignancy markers in NB, e.g. proto-oncogenic MYC or MYCN were expressed highly heterogeneously in the three investigated NB cell lines with up-regulation of MYC and MYCN upon medium-induced microenvironmental change. In addition, SiMa cells exclusively showed a phenotype switching from loosely-adherent monolayers to low proliferating grape-like cellular aggregates, which was accompanied by an enhanced CD133 expression. Interestingly, the overexpression of HERV was associated with a significant elevation of immune checkpoint molecule CD200 in both quantitative PCR and RNA-seq analysis suggesting tumor escape mechanism in NB cell lines after incubation in serum-free stem cell medium.

Published
2021

19. Surfactant protein C, a modulator of paravascular brain waste clearance, is associated with perineuronal nets and shows age-dependent changes of brain content and characteristic deposits in wildtype and 3xTg mice

Author

Schob, Stefan, primary, Puchta, Joana, additional, Winter, Karsten, additional, Michalski, Dominik, additional, Mages, Bianca, additional, Martens, Henrik, additional, Emmer, Alexander, additional, Hoffmann, Karl-Titus, additional, Gaunitz, Frank, additional, Meinicke, Anton, additional, Krause, Matthias, additional, and Härtig, Wolfgang, additional

Published
2021
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20. Identification of Differentially Expressed Human Endogenous Retrovirus Families in Human Leukemia and Lymphoma Cell Lines and Stem Cells

Author

Engel, Kristina, Wieland, Lisa, Krüger, Anna, Volkmer, Ines, Cynis, Holger, Emmer, Alexander, Staege, Martin Sebastian, and Publica

Subjects
hematopoietic stem cells (HSCs), Embryonic stem cells (ESC), viruses, leukemia, RNA sequencing, lymphoma, Leukämie, human endogenous retroviruses (HERVs), differential RNA sequencing, Hematopoietic stem celld (HSC), Oncology, Human endogenous retroviruses (HERV), embryonic stem cells (ESCs), embryonic structures, gene expression, LL-100 panel, Original Research
Abstract

Endogenous retroviruses (ERVs) are becoming more and more relevant in cancer research and might be potential targets. The oncogenic potential of human ERVs (HERVs) has been recognized and includes immunosuppression, cell fusion, antigenicity of viral proteins, and regulation of neighboring genes. To decipher the role of HERVs in human cancers, we used a bioinformatics approach and analyzed RNA sequencing data from the LL-100 panel, covering 22 entities of hematopoietic neoplasias including T cell, B cell and myeloid malignancies. We compared HERV expression in this panel with hematopoietic stem cells (HSCs), embryonic stem cells (ESCs) and normal blood cells. RNA sequencing data were mapped against a comprehensive synthetic viral metagenome with 116 HERV sequences from 14 different HERV families. Of these, 13 HERV families and elements were differently expressed in malignant hematopoietic cells and stem cells. We found transcriptional upregulation of HERVE family in acute megakaryocytic and erythroid leukemia and of HERVFc family in multiple myeloma/plasma cell leukemia (PCL). The HERVFc member HERVFc-1 was found transcriptionally active in the multiple myeloma cell line OPM-2 and also in the Hodgkin lymphoma cell line L-428. The expression of HERVFc-1 in L-428 cells was validated by qRT-PCR. We also confirm transcriptional downregulation of ERV3 in acute megakaryocytic and erythroid leukemia, and HERVK in acute monocytic and myelocytic leukemia and a depression of HERVF in all malignant entities. Most of the higher expressed HERV families could be detected in stem cells including HERVK (HML-2), HERV-like, HERVV, HERVT, ERV9, HERVW, HERVF, HERVMER, ERV3, HERVH and HERVPABLB.

Published
2021

24. Complex Repetitive Discharges: A Sign of Motor Axonal Reinnervation?

Author

Posa, Andreas, Niśkiewicz, Izabela, Emmer, Alexander, Hanisch, Frank, and Kornhuber, Malte E.

Subjects
body regions, electromyography (EMG), myo-axonal ephaptic re-excitation, pathological spontaneous activity (PSA), musculoskeletal system, complex repetitive discharge (CRD), lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry, axonal sprouting, Article, lcsh:RC321-571
Abstract

Complex repetitive discharges (CRDs) are poorly understood phenomena in needle electromyography (EMG) recordings. The data presented here suggest that CRDs may mainly be a sign of motor unit reinnervation. EMG &ldquo, video&rdquo, data of 108 CRDs from neurogenic (ND, n = 39) and myogenic (MD, n = 14) disorders were retrospectively analyzed for cycle duration, potential-free time intervals, spike components (SC), maximum amplitudes, blockade, and increased jitter. CRD-SC in ND disorders (9.3 &plusmn, 7.8) outnumbered those in MD disorders (6.3 &plusmn, 6.2). The CRD cycle duration was correlated with SC and silent periods (p each &lt, 0.000001). Blockade was observed in 36% and increased jitter in 27% of the CRDs. A higher number of CRD-SC in ND vs. MD fits the known differences in motor unit dimensions. Blockade and increased jitter are known features of diseased neuromuscular junctions, such as during reinnervation. The SC patterns of single CRD cycles resemble reinnervation potentials. Thus, CRDs may result from myo-axonal re-excitation in sprouting motor units. The purpose of this investigation was to better understand the circumstances under which CRDs may occur and eventually to contribute to the understanding of their pathogenesis.

Published
2020

25. Formation of HERV-K and HERV-Fc1 Envelope Family Members is Suppressed on Transcriptional and Translational Level

Author

Gröger, Victoria, Wieland, Lisa, Naumann, Marcel, Meinecke, Ann-Christin, Meinhardt, Beate, Rossner, Steffen, Ihling, Christian, Emmer, Alexander, Staege, Martin S., Cynis, Holger, Gröger, Victoria, Wieland, Lisa, Naumann, Marcel, Meinecke, Ann-Christin, Meinhardt, Beate, Rossner, Steffen, Ihling, Christian, Emmer, Alexander, Staege, Martin S., and Cynis, Holger

Abstract

The human genome comprises 8% sequences of retroviral origin, so-called human endogenous retroviruses (HERVs). Most of these proviral sequences are defective, but some possess open reading frames. They can lead to the formation of viral transcripts, when activated by intrinsic and extrinsic factors. HERVs are thought to play a pathological role in inflammatory diseases and cancer. Since the consequences of activated proviral sequences in the human body are largely unexplored, selected envelope proteins of human endogenous retroviruses associated with inflammatory diseases, namely HERV-K18, HERV-K113, and HERV-Fc1, were investigated in the present study. A formation of glycosylated envelope proteins was demonstrated in different mammalian cell lines. Nevertheless, protein maturation seemed to be incomplete as no transport to the plasma membrane was observed. Instead, the proteins remained in the ER where they induced the expression of genes involved in unfolded protein response, such as HSPA5 and sXBP1. Furthermore, low expression levels of native envelope proteins were increased by codon optimization. Cell-free expression systems showed that both the transcriptional and translational level is affected. By generating different codon-optimized variants of HERV-K113 envelope, the influence of single rare t-RNA pools in certain cell lines was demonstrated. The mRNA secondary structure also appears to play an important role in the translation of the tested viral envelope proteins. In summary, the formation of certain HERV proteins is basically possible. However, their complete maturation and thus full biologic activity seems to depend on additional factors that might be disease-specific and await elucidation in the future.

Published
2020

33. WebHERV: A Web Server for the Computational Investigation of Gene Expression Associated With Endogenous Retrovirus-Like Sequences

Author

Kruse, Konstantin, Nettling, Martin, Wappler, Nadine, Emmer, Alexander, Kornhuber, Malte E., Staege, Martin Sebastian, and Gro��e, Ivo

Subjects
Microbiology (medical), web server, viruses, HERVs, CYP4Z1, Microbiology, endogenous retroviruses, embryonic structures, DRUMS, BLAST, Hodgkin lymphoma, database, Original Research
Abstract

More than eight percent of the human genome consists of human endogenous retroviruses (HERVs). Typically, the expression of HERVs is repressed, but varying activities of HERVs have been observed in diseases ranging from cancer to neuro-degeneration. Such activities can include the transcription of HERV-derived open reading frames, which can be translated into proteins. However, as a consequence of mutations that disrupt open reading frames, most HERV-like sequences have lost their protein-coding capacity. Nevertheless, these loci can still influence the expression of adjacent genes and, hence, mediate biological effects. Here, we present WebHERV (http://calypso.informatik.uni-halle.de/WebHERV/), a web server that enables the computational prediction of active HERV-like sequences in the human genome based on a comparison of genome coordinates of expressed sequences uploaded by the user and genome coordinates of HERV-like sequences stored in the specialized key-value store DRUMS. Using WebHERV, we predicted putative candidates of active HERV-like sequences in Hodgkin lymphoma (HL) cell lines, validated one of them by a modified SMART (switching mechanism at 5��� end of RNA template) technique, and identified a new alternative transcription start site for cytochrome P450, family 4, subfamily Z, polypeptide 1 (CYP4Z1).

Published
2018
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37. Cooccurrences of Putative Endogenous Retrovirus-Associated Diseases

Author

Brütting, Christine, Emmer, Alexander, Kornhuber, Malte E., and Staege, Martin S.

Subjects
Article Subject
Abstract

At least 8% of the human genome is composed of endogenous retrovirus (ERV) sequences. ERVs play a role in placental morphogenesis and can sometimes protect the host against exogenous viruses. On the other hand, ERV reactivation has been found to be associated with different diseases, for example, multiple sclerosis (MS), schizophrenia, type 1 diabetes mellitus (T1D), or amyotrophic lateral sclerosis (ALS). Little is known about the cooccurrence of these diseases. If all these diseases are caused by ERV, antiretroviral therapy should perhaps also show some effects in the other diseases. Here, we summarize literature demonstrating that some ERV-associated diseases seem to appear together more often than expected, for example, MS and ALS, MS and T1D, MS and schizophrenia, or ALS and T1D. In contrast, some ERV-associated diseases seem to appear together less frequently than expected, for example, schizophrenia and T1D. Besides, some reports demonstrate amelioration of MS, ALS, or schizophrenia under antiretroviral therapy in human immunodeficiency virus-infected patients. If such results could be confirmed in larger studies, alternative therapy strategies for ERV-associated diseases like MS and schizophrenia might be possible.

Published
2017
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38. Effect of High-Caloric Nutrition on Survival in Amyotrophic Lateral Sclerosis.

Author

Ludolph, Albert C., Dorst, Johannes, Dreyhaupt, Jens, Weishaupt, Jochen H., Kassubek, Jan, Weiland, Ulrike, Meyer, Thomas, Petri, Susanne, Hermann, Andreas, Emmer, Alexander, Grosskreutz, Julian, Grehl, Torsten, Zeller, Daniel, Boentert, Matthias, Schrank, Bertold, Prudlo, Johannes, Winkler, Andrea S., Gorbulev, Stanislav, Roselli, Francesco, and Schuster, Joachim

Subjects
AMYOTROPHIC lateral sclerosis, MOTOR neuron diseases, NUTRITION, WEIGHT loss, DIET, RANDOMIZED controlled trials, SURVIVAL analysis (Biometry), BLIND experiment, RILUZOLE, NEUROPROTECTIVE agents, RESEARCH funding, COMBINED modality therapy, STATISTICAL sampling
Abstract

Objective: Weight loss has been identified as a negative prognostic factor in amyotrophic lateral sclerosis, but there is no evidence regarding whether a high-caloric diet increases survival. Therefore, we sought to evaluate the efficacy of a high-caloric fatty diet (HCFD) for increasing survival.Methods: A 1:1 randomized, placebo-controlled, parallel-group, double-blinded trial (LIPCAL-ALS study) was conducted between February 2015 and September 2018. Patients were followed up at 3, 6, 9, 12, 15, and 18 months after randomization. The study was performed at 12 sites of the clinical and scientific network of German motor neuron disease centers (ALS/MND-NET). Eligible patients were randomly assigned (1:1) to receive either HCFD (405kcal/day, 100% fat) or placebo in addition to riluzole (100mg/day). The primary endpoint was survival time, defined as time to death or time to study cutoff date.Results: Two hundred one patients (80 female, 121 male, age = 62.4 ± 10.8 years) were included. The confirmatory analysis of the primary outcome survival showed a survival probability of 0.39 (95% confidence interval [CI] = 0.27-0.51) in the placebo group and 0.37 (95% CI = 0.25-0.49) in the HCFD group, both after 28 months (point in time of the last event). The hazard ratio was 0.97, 1-sided 97.5% CI = -∞ to 1.44, p = 0.44.Interpretation: The results provide no evidence for a life-prolonging effect of HCFD for the whole amyotrophic lateral sclerosis population. However, post hoc analysis revealed a significant survival benefit for the subgroup of fast-progressing patients. ANN NEUROL 2020;87:206-216. [ABSTRACT FROM AUTHOR]

Published
2020
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39. Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial

Author

Ludolph, Albert C, primary, Schuster, Joachim, additional, Dorst, Johannes, additional, Dupuis, Luc, additional, Dreyhaupt, Jens, additional, Weishaupt, Jochen H, additional, Kassubek, Jan, additional, Weiland, Ulrike, additional, Petri, Susanne, additional, Meyer, Thomas, additional, Grosskreutz, Julian, additional, Schrank, Berthold, additional, Boentert, Matthias, additional, Emmer, Alexander, additional, Hermann, Andreas, additional, Zeller, Daniel, additional, Prudlo, Johannes, additional, Winkler, Andrea S, additional, Grehl, Torsten, additional, Heneka, Michael T, additional, Wollebæk Johannesen, Siw, additional, Göricke, Bettina, additional, Funke, Andreas, additional, Kettemann, Dagmar, additional, Meyer, Robert, additional, Gruhn, Kai, additional, Schwenkreis, Peter, additional, Stude, Philipp, additional, Heneka, MichaelT, additional, Kurzwelly, Delia, additional, Storch, Alexander, additional, Richter, Nicole, additional, Frank, Tobias, additional, Hein, Katharina, additional, Hanisch, Frank, additional, Hanke, Dagmar, additional, Kraya, Torsten, additional, Posa, Andreas, additional, Romanakova, Martina, additional, Schilling, Susanne, additional, Abdulla, Susanne, additional, Böselt, Sebastian, additional, Janssen, Claas, additional, Lange, Imken, additional, Kobeleva, Xenia, additional, Körner, Sonja, additional, Kollewe, Katja, additional, Osmanovic, Alma, additional, Scharn, Nicole, additional, Rath, Klaus J, additional, Dahms, Christiane, additional, Gunkel, Anne, additional, Heiling, Bianka, additional, Ringer, Thomas, additional, Smesny, Uta, additional, Baumeister, Sarah, additional, Berthele, Achim, additional, Bublitz, Sarah, additional, Akova-Öztürk, Esra, additional, Stubbe-Dräger, Bianca, additional, Rahmann, Alexandra, additional, Young, Charlotte, additional, Young, Peter, additional, Baldaranov, Dobri, additional, Bogdahn, Ulrich, additional, Khomenko, Andrei, additional, Schulte-Mattler, Wilhelm, additional, Stadler, Christina, additional, Husung, Susanne, additional, Tesar, Simone, additional, Dargah-Zaden, Nigar, additional, Last, Christina, additional, Langer, Eva, additional, Ludolph, Albert C, additional, Lauenstein, Ann-Sophie, additional, Lensch, Eckard, additional, Mc Farlane, Carolyn, additional, Fischer-Brasse, Heike, additional, Orbán, Klara, additional, Schrank, Bertold, additional, Schürger, Sonja, additional, Klebe, Stephan, additional, Kraft, Peter, additional, Musacchio, Thomas, additional, and Seiler, Carola, additional

Published
2018
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40. Localization, Occurrence, and CSF Changes of SP-G, a New Surface Active Protein with Assumable Immunoregulatory Functions in the CNS

Author

Krause, Matthias, primary, Peukert, Nicole, additional, Härtig, Wolfgang, additional, Emmer, Alexander, additional, Mahr, Cynthia Vanessa, additional, Richter, Cindy, additional, Dieckow, Julia, additional, Puchta, Joana, additional, Pirlich, Mandy, additional, Hoffmann, Karl-Titus, additional, Nestler, Ulf, additional, and Schob, Stefan, additional

Published
2018
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48. Apparent diffusion coefficient (ADC) does not correlate with different serological parameters in myositis and myopathy.

Author

Ziemann, Oliver, Meyer, Hans-Jonas, Surov, Alexey, Kornhuber, Malte, Emmer, Alexander, Quäschling, Ulf, and Schob, Stefan

Subjects
MAGNETIC resonance imaging, DIFFUSION coefficients, DERMATOMYOSITIS, POLYMYOSITIS, ELECTROMYOGRAPHY, DIAGNOSIS of muscle diseases, MUSCLE diseases, MYOSITIS, SERODIAGNOSIS, RETROSPECTIVE studies
Abstract

Background Magnetic resonance imaging (MRI) is widely used in several muscle disorders. Diffusion-weighted imaging (DWI) is an imaging modality, which can reflect microstructural tissue composition. The apparent diffusion coefficient (ADC) is used to quantify the random motion of water molecules in tissue. Purpose To investigate ADC values in patients with myositis and non-inflammatory myopathy and to analyze possible associations between ADC and laboratory parameters in these patients. Material and Methods Overall, 17 patients with several myositis entities, eight patients with non-inflammatory myopathies, and nine patients without muscle disorder as a control group were included in the study (mean age = 55.3 ± 14.3 years). The diagnosis was confirmed by histopathology in every case. DWI was obtained in a 1.5-T scanner using two b-values: 0 and 1000 s/mm2. In all patients, the blood sample was acquired within three days to the MRI. The following serological parameters were estimated: C-reactive protein, lactate dehydrogenase, alanine aminotransferase, aspartate aminotransferase, creatine kinase, and myoglobine. Results The estimated mean ADC value for the myositis group was 1.89 ± 0.37 × 10-3 mm2/s and for the non-inflammatory myopathy group was 1.79 ± 0.33 × 10-3 mm2/s, respectively. The mean ADC values (1.15 ± 0.37 × 10-3 mm2/s) were significantly higher to unaffected muscles (vs. myositis P = 0.0002 and vs. myopathy P = 0.0021). There were no significant correlations between serological parameters and ADC values. Conclusion Affected muscles showed statistically significantly higher ADC values than normal muscles. No linear correlations between ADC and serological parameters were identified. [ABSTRACT FROM AUTHOR]

Published
2018
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