The French Society of Neuropathology was created in 1989, succeeding the French Club of Neuropathology set up in 1965. The Society organizes two scientific meetings per year

French Society of Neuropathology, The

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Open Access-Zeitschriften an der WWU Münster (Westfälische Wilhelms-Universität)

Free Neuropathology 1:34 (2020)  doi: https://doi.org/10.17879/freeneuropathology-2020-3134 page 1 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.    62nd Meeting of the French Society of Neuropathology Meeting Abstracts  December 4th, 2020     The French Society of Neuropathology was created in 1989, succeeding the French Club of Neuropathology set up in 1965. The Society organizes two scientific meetings per year. Submitted: 11 December 2020 · Accepted: 14 December 2020 · Published: 15 December 2020 Meeting Summary   page 2 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Plu I, Evrard B, Duchesne M, Regnault B, Pérot P, Chrétien D, Eloit M, Seilhean D (2020) Etiological diagnosis of a plasma cell encephalitis by next generation sequencing. Free Neuropathol 1, 34: 2  Etiological diagnosis of a plasma cell encephalitis by next generation sequencing Isabelle Plu1, Bruno Evrard2, Mathilde Duchesne3, Béatrice Regnault4, Philippe Pérot4, Delphine Chrétien4, Marc Eloit4, Danielle Seilhean1 1 Department of Neuropathology, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France 2 Intensive Care Department, University Hospital Limoges, France 3 Department of Pathology, University Hospital Limoges, France 4 Pathogen discovery unit, Department of Virology, Pasteur Institute, Paris  A 59-year-old man, working as a forester, developed a fever and temporal headache associated with cranial nerves deficits. Etiological investigations including multiplex PCR and the search for autoantibodies remained negative. His condition quickly worsened leading to coma and death after 2 months.  Neuropathology revealed lymphoplasmacytic infiltrates in the leptomeninges, cranial nerve roots and around the vessels in cerebral gray and white matter. Perivascular cuffs were associated with astrocytic gliosis and mi-croglial activation. Immunochemistry against known viruses was negative. The New Generation DNA Sequencing (NGS) without a priori has led to the identification of a type 1 Lyssavirus of the European bat (EBLV1), the reser-voir of which is an insectivorous bat. Only two cases of transmission to human have been reported to date. This case of zoonosis illustrates the interspecies transmission of an unusual virus leading to a potential emerging disease. NGS coupled with neuropathology provide a valuable tool for identifying new causes of encephalitis.   Meeting Abstract [Short communications]   page 3 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Teyssou E, Muratet F, del Mar Amador M, Gyorgy B, Guegan J, Marie Y, Meininger V, Salachas F, Millecamps S, Seilhean D (2020) A novel mutation in SOD1 causing unusual neuropathological findings. Free Neuropathol 1, 34: 3  A novel mutation in SOD1 causing unusual neuropathological findings Elisa Teyssou1*, François Muratet1*, Maria del Mar Amador1,2, Beata Gyorgy1, Justine Guegan1, Yannick Marie1, Vincent Meininger2,3, François Salachas1,2, Stéphanie Millecamps1, Danielle Seilhean1,4 1 INSERM U1127, CNRS UMR7225, Sorbonne University-UMRS1127, Brain Institute ICM, Paris, France 2 Department of Neurology, Reference Center for ALS Ile de France, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France 3 Peupliers Hospital, Ramsay Générale de Santé, Paris, France 4 Department of Neuropathology, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France * Equal contribution  A 63-year-old man with no personal or family history developed amyotrophic lateral sclerosis (ALS) at age 63, fatal in 17 months. In the anterior horns of the spinal cord, neuronal loss was moderate. Cystatin C immunohisto-chemistry (IHC) showed numerous Bunina bodies. IHC of ubiquitin, p62 or TDP43, did not show skein-like inclu-sions. Swollen neurons in clusters, were labelled with anti- ubiquitin, SOD-1 and phosphorylated neurofilaments. They were different from inclusions usually observed in SOD-1 mutations. Whole exome sequencing analysis identified a novel SOD1 mutation c.164C>T, p.Thr55Ileu, confirmed by Sanger sequencing. No other rare variant was identified in any other ALS-related genes. Association of neuropathology and whole exome sequencing can provide a useful tool for the identification of unknown forms of the disease, better understanding of the physiopathology and lead to new therapeutic targets.   Meeting Abstract [Short communications]    page 4 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Siegert E, Dittmayer C, Schneider U, Preuße C, Goebel HH, Stenzel W (2020) Myositis in scleroderma – Capillary pathology is fundamental. Free Neuropathol 1, 34: 4  Myositis in scleroderma – Capillary pathology is fundamental Elise Siegert, Carsten Dittmayer, Udo Schneider, Corinna Preuße, Hans-Hilmar Goebel, Werner Stenzel Department of Neuropathology, Charité - Universitätsmedizin, corporate member of Freie Universität Berlin, Hum-boldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany   Systemic sclerosis is a chronic disease of connective tissues characterized by fibosis, vasculopathy and autoim-munity. Affected patients show signs of the skin, internal organs and sometimes overlap myositis. The vasculopa-thy is considered obliterative but the pathogenic basis is not known to date. We are presenting light-microscopic and ultrastructural as well as clinical data of 18 patients suffering form scle-roderma and myositis. We have applied a new electron microscopical technique which we call large scale elec-tron microscopy allowing a ‘pan and zoom’ approach similar to ‘google earth’ viewing. This analysis allows to study >1000 capillaries of patients and controls, highlighting reduplications of basement. We show that this type of ultrastructural changes is membranes, endothelial activation and proliferation of per-icytes specific for a subtype of scleromyositis and discus possible pathogenic mechanisms.   Meeting Abstract [Short communications]    page 5 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Boluda S, Wallon D, Rovelet-Lecrux A, Campion D, Nicolas G, Duyckaerts C (2020) Neuropathological variability of four tauopathy cases with MAPT microduplication. Free Neuropathol 1, 34: 5  Neuropathological variability of four tauopathy cases with MAPT microduplication Susana Boluda1,2, David Wallon3,4,5, Anne Rovelet-Lecrux3,4,6, Dominique Campion3,4,6, Gaël Nicolas3,4,6, Charles Duyckaerts1,2 1 Department of Neuropathology, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France 2 INSERM U1127, CNRS UMR7225, Sorbonne University-UMRS1127, Brain Institute ICM, Paris, France 3 INSERM U1245, University of Normandy, UNIROUEN, Rouen, France 4 CNR-MAJ, Normandy Center for Genomic and Personalized Medicine, Rouen, France 5 Department of Neurology, University Hospital, Rouen, France 6 Department of Genetics, University Hospital, Rouen, France  We report the neuropathology of four MAPT duplication carriers, a rare chromosomal rearrangement involving the 17q21.31 chromosomal region that causes an early onset dementia (EOD) clinically mimicking Alzheimer disease or an atypical extrapyramidal syndrome, as recently described. They were three males and one female with ages ranging between 37 and 57 years. They were all tauopathies with a variability in the morphology and distribution of the aggregates. The cases either mimicked Pick disease with neuronal globular aggregates similar to Pick bodies and pathology involving predominantly the cortical and limbic regions and subcortical nuclei or they resembled progressive supranuclear palsy with tufted astrocytes with major involvement of the brain stem and subcortical nuclei. The tau isoform expression also varied from either only 3R, only 4R or a mixed 3R/4R expression. In conclusion, MAPT duplication may lead to a tauopathy spectrum with a range of 3R and 4R expression.   Meeting Abstract [Short communications]    page 6 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Seilhean D, Mokhtari K, Plu I, Boluda-Casas S, Mathon B, Cao A, Hervé D, Mégarbane B, Bielle F, Levavasseur E, Malet I, Marot S, El Hachimi H, Marty S, Prigent A, Duyckaerts C, Potier MC, Haïk S, Delatour B, Marcelin AG (2020) Multiple cerebral angiopathy in SARS-COV-2 infection. Free Neuropathol 1, 34: 6  Multiple cerebral angiopathy in SARS-COV-2 infection Danielle Seilhean1,2, Karima Mokhtari1, Isabelle Plu1, Susana Boluda-Casas1,2, Bertrand Mathon3, Albert Cao4, Dominique Hervé5, Bruno Mégarbane6, Franck Bielle1,2, Etienne Levavasseur2, Isabelle Malet7, Stéphane Marot7, Hamid El Hachimi2, Serge Marty2, Annick Prigent2, Charles Duyckaerts1,2, Marie-Claude Potier2, Stéphane Haïk1,2, Benoît Delatour2, Anne-Geneviève Marcelin7 1 Department of Neuropathology, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France 2 INSERM U1127, CNRS UMR7225, Sorbonne University-UMRS1127, Brain Institute ICM, Paris, France 3 Department of Neurosurgery, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France 4 Department of Neurology, Neuro-ICU, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France 5 Department of Neurology, Lariboisière Hospital, AP-HP Nord- Paris University, Paris, France 6 Department of Intensive Care, Lariboisière Hospital, AP-HP Nord- Paris University, Paris, France 7 Department of Virology, INSERM UMRS 1136, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France  SARS-CoV-2 is the cause of a pandemic characterized by its severity in elderly subjects or those presenting met-abolic or vascular risk factors. Brain damage, although relatively rare, is often fatal. From four cases (one autopsy and three brain biopsies) we analyzed the involvement of small cerebral arteries associated with white matter lesions, without signs of vasculitis or encephalitis. Viral RNA has not been detected in the brain. SARS-CoV2 spike protein (S) has been detected in the Golgi apparatus of endothelial cells, colocalized with a host protease. Our observations suggest the possibility of hematogenous neuroinvasion. The interaction of a small amount of pro-tein S with endogenous proteases is thought to be able to disrupt the permeability of brain endothelial cells causing vascular damage. This result could provide therapeutic avenues to prevent or cure severe brain forms in patients at risk.   Meeting Abstract [Short communications]    page 7 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Bourhis A, Peyre M, Bielle F (2020) A rare tumor of the peripheral nerve mimicking a schwannoma. Free Neuro-pathol 1, 34: 7  A rare tumor of the peripheral nerve mimicking a schwannoma Amélie Bourhis1, Matthieu Peyre2,3, Franck Bielle2,4 1 Department of Pathology, University Hospital, Brest, France 2 INSERM U1127, CNRS UMR7225, Sorbonne University-UMRS1127, Brain Institute ICM, Paris, France 3 Department of Neurosurgery, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France 4 Department of Neuropathology, Pitie Salpetriere Hospital, AP-HP Sorbonne University, Paris, France  A 38 year-old man presented with pain of the left forearm for five years. He had a history of old HIV infection and several past opportunistic infections. The pain was paroxysmal, favored by pronation and was increasing for two years. MRI evidenced a tumor nodule on the path of the posterior interosseous nerve mimicking a schwan-noma. Intracapsular tumor resection stopped the pain. Microscopic examination showed a tumor proliferation of fascicled eosinophilic fusiform cells. Immunostaining showed the expression of the muscular markers smooth muscle actin and desmin, and the absence of expression of SOX10. In situ hybridization for EBER RNA of EBV was positive in tumor cells. We diagnosed an EBV-associated leiomyoma of the immunocompromised, which was located in the peripheral nerve, a localization not previously reported. We discuss the tumorigenesis of this rare neoplasm.   Meeting Abstract [Short communications]    page 8 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Eloit M (2020) Virus spillover from animal reservoirs and vectors to human detection using agnostic tools. Free Neuropathol 1, 34: 8  Virus spillover from animal reservoirs and vectors to human detection using agnostic tools Marc Eloit Head of Pathogen Discovery Laboratory, Pasteur Institute, Paris, France OIE Collaborating Centre for Detection and Identification in Humans of Emerging Animal Pathogens, Pasteur Insti-tute, Paris, France Professor of Virology, Veterinary School of Maisons Alfort, France  Viruses from wildlife including arboviruses vectored by mosquitoes have caused dramatic outbreaks over the last 25 years. On the one hand, new sequencing capacities have deeply affected the means by which new viruses are discovered, leading to the identification of ever more viruses in animal reservoirs and arthropods. On the other hand, it is worth noting that the continually growing knowledge regarding these viruses does not per se serve to identify potential human or animal threats. Our laboratory aims to fill this gap by identifying, in selected ecosys-tems, whether as yet unknown, unexpected or neglected mosquito or wildlife viruses are responsible for fre-quent but sub-clinical or mild infection in humans highly exposed to wild life/arthropods, and thus represent good candidates for global spreading. It combines high throughput screening of viruses in animal/arthropods linked to antibody screening in healthy exposed human populations and virus search in patients presenting with severe diseases of unknown etiology. I will show our methodology and some results   Meeting Abstract [Conferences]    page 9 of 9     Copyright: © 2020 The author(s). This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited, a link to the Creative Commons license is provided, and any changes are indicated. The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.  Ludes B (2020) Migration of Siberian populations of the past: contribution of a multi-genetic markers approach. Free Neuropathol 1, 34: 9  Migration of Siberian populations of the past: contribution of a multi-genetic markers approach Bertrand Ludes University of Paris-CNRS, FRE 2029 Head of the Forensic Institute of Paris (Institut Médico-Légal de Paris)  Molecular markers of nuclear DNA (autosomes and Y chromosome) and mitochondrial DNA from human samples help clarifying the migrations of the first peoples of the Eurasian steppes. We analyzed samples dating from the middle of the 2nd millennium BC to 4th century AD and originating from the Krasnoyarsk region (South Siberia). We confirmed that, during the Bronze and Iron Ages, southern Siberia was a region dominated by European peoples, suggesting an eastward migration of the Kurgan peoples across the Russo-Kazak steppe. The results further showed that at that time, the inhabitants of southern Siberia must have had fair eyes, skin and hair.    Meeting Abstract [Conferences]   

62nd Meeting of the French Society of Neuropathology - Meeting Abstracts , December 4th. 2020

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62nd Meeting of the French Society of Neuropathology - Meeting Abstracts , December 4th. 2020

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Open Access-Zeitschriften an der WWU Münster (Westfälische Wilhelms-Universität)