{"id":"https://openalex.org/W2432991705","doi":null,"title":"[Cavitary orthochromatic leukodystrophy in the adult. Oligodendroglial proliferation and inclusions].","display_name":"[Cavitary orthochromatic leukodystrophy in the adult. Oligodendroglial proliferation and inclusions].","publication_year":1984,"publication_date":"1984-01-01","ids":{"openalex":"https://openalex.org/W2432991705","mag":"2432991705","pmid":"https://pubmed.ncbi.nlm.nih.gov/6093215"},"language":"fr","primary_location":{"id":"pmid:6093215","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/6093215","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Revue neurologique","raw_type":"Case Reports"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5084333187","display_name":"Gautier Jc","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"J C Gautier","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5107484481","display_name":"Fran\u00e7oise Gray","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"F Gray","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111665805","display_name":"Adnan A. Awada","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"A Awada","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5011239100","display_name":"R Escourolle","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"R Escourolle","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":16,"citation_normalized_percentile":{"value":0.0,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":94},"biblio":{"volume":"140","issue":"8-9","first_page":"493","last_page":"501"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.10130000114440918,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.10130000114440918,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T12331","display_name":"Hereditary Neurological Disorders","score":0.09520000219345093,"subfield":{"id":"https://openalex.org/subfields/2804","display_name":"Cellular and Molecular Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.06840000301599503,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/adult-onset-leukodystrophy","display_name":"adult-onset leukodystrophy","score":0.7459999918937683},{"id":"https://openalex.org/keywords/oligodendrocyte-proliferation","display_name":"oligodendrocyte proliferation","score":0.8169999718666077},{"id":"https://openalex.org/keywords/demyelination","display_name":"demyelination","score":0.7459999918937683},{"id":"https://openalex.org/keywords/generalized-tonic-clonic-seizures","display_name":"generalized tonic-clonic seizures","score":0.6169999837875366},{"id":"https://openalex.org/keywords/spastic-paraparesis","display_name":"spastic paraparesis","score":0.9909999966621399},{"id":"https://openalex.org/keywords/intellectual-disability","display_name":"intellectual disability","score":0.8029999732971191},{"id":"https://openalex.org/keywords/akinetic-mutism","display_name":"akinetic mutism","score":0.9950000047683716},{"id":"https://openalex.org/keywords/oligodendroglial-inclusions","display_name":"oligodendroglial inclusions","score":0.7459999918937683},{"id":"https://openalex.org/keywords/brain-biopsy","display_name":"brain biopsy","score":0.43299999833106995}],"concepts":[{"id":"https://openalex.org/C2776100374","wikidata":"https://www.wikidata.org/wiki/Q1821559","display_name":"Leukodystrophy","level":3,"score":0.7815163135528564},{"id":"https://openalex.org/C2781192897","wikidata":"https://www.wikidata.org/wiki/Q822050","display_name":"White matter","level":3,"score":0.7527575492858887},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.6725043654441833},{"id":"https://openalex.org/C2778609137","wikidata":"https://www.wikidata.org/wiki/Q105790271","display_name":"Myelin","level":3,"score":0.6539621353149414},{"id":"https://openalex.org/C2778583010","wikidata":"https://www.wikidata.org/wiki/Q3237081","display_name":"Leukoencephalopathy","level":3,"score":0.5168598294258118},{"id":"https://openalex.org/C2777827993","wikidata":"https://www.wikidata.org/wiki/Q3985306","display_name":"Spastic quadriplegia","level":3,"score":0.5013444423675537},{"id":"https://openalex.org/C2776985911","wikidata":"https://www.wikidata.org/wiki/Q916698","display_name":"Oligodendrocyte","level":4,"score":0.498676061630249},{"id":"https://openalex.org/C2780640218","wikidata":"https://www.wikidata.org/wiki/Q8277","display_name":"Multiple sclerosis","level":2,"score":0.4389016926288605},{"id":"https://openalex.org/C2776195251","wikidata":"https://www.wikidata.org/wiki/Q7573993","display_name":"Spastic","level":3,"score":0.42126280069351196},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.4197325110435486},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.2144235074520111},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.17239949107170105},{"id":"https://openalex.org/C143409427","wikidata":"https://www.wikidata.org/wiki/Q161238","display_name":"Magnetic resonance imaging","level":2,"score":0.13937222957611084},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.13670381903648376},{"id":"https://openalex.org/C529278444","wikidata":"https://www.wikidata.org/wiki/Q47273","display_name":"Central nervous system","level":2,"score":0.12849640846252441},{"id":"https://openalex.org/C2779421357","wikidata":"https://www.wikidata.org/wiki/Q210427","display_name":"Cerebral palsy","level":2,"score":0.12109258770942688},{"id":"https://openalex.org/C126838900","wikidata":"https://www.wikidata.org/wiki/Q77604","display_name":"Radiology","level":1,"score":0.1159270703792572},{"id":"https://openalex.org/C118552586","wikidata":"https://www.wikidata.org/wiki/Q7867","display_name":"Psychiatry","level":1,"score":0.0683642029762268}],"mesh":[{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D002455","descriptor_name":"Cell Division","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002479","descriptor_name":"Inclusion Bodies","qualifier_ui":"Q000648","qualifier_name":"ultrastructure","is_major_topic":true},{"descriptor_ui":"D002549","descriptor_name":"Diffuse Cerebral Sclerosis of Schilder","qualifier_ui":"Q000000981","qualifier_name":"diagnostic imaging","is_major_topic":false},{"descriptor_ui":"D002549","descriptor_name":"Diffuse Cerebral Sclerosis of Schilder","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D003937","descriptor_name":"Diagnosis, Differential","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005625","descriptor_name":"Frontal Lobe","qualifier_ui":"Q000000981","qualifier_name":"diagnostic imaging","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009457","descriptor_name":"Neuroglia","qualifier_ui":"Q000648","qualifier_name":"ultrastructure","is_major_topic":true},{"descriptor_ui":"D009836","descriptor_name":"Oligodendroglia","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D009836","descriptor_name":"Oligodendroglia","qualifier_ui":"Q000648","qualifier_name":"ultrastructure","is_major_topic":true},{"descriptor_ui":"D014057","descriptor_name":"Tomography, X-Ray Computed","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"pmid:6093215","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/6093215","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Revue neurologique","raw_type":"Case Reports"},{"id":"mag:2432991705","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/6093215","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"score":0.9907000064849854,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/case-report","display_name":"Case Report"}],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W2405856410","https://openalex.org/W1604347834","https://openalex.org/W2019634173","https://openalex.org/W2415564006","https://openalex.org/W1995839198","https://openalex.org/W2409280145","https://openalex.org/W2027384845","https://openalex.org/W2466682872","https://openalex.org/W2010363394","https://openalex.org/W121323723"],"abstract_inverted_index":{"A":[0],"41":[1],"year-old":[2],"woman":[3],"presented":[4],"with":[5,93],"two":[6],"Grand":[7],"Mal":[8],"fits":[9,39],"followed":[10],"by":[11],"progressive":[12],"spastic":[13],"paraparesis":[14],"and":[15,37,50,70,77,85,99,124],"intellectual":[16],"impairment.":[17],"After":[18],"18":[19],"months":[20],"there":[21],"was":[22,61,91],"a":[23,42,51,74,78],"remission":[24],"which":[25],"lasted":[26],"for":[27],"4":[28],"years":[29,53],"but":[30],"thereafter":[31],"inexorable":[32],"progression":[33],"of":[34,44,57,73,87,96,105,113,117,121],"the":[35,55,58,88,94,103,106,111],"disease":[36],"many":[38],"led":[40],"to":[41],"state":[43],"akinetic":[45],"mutism.":[46],"Death":[47],"occurred":[48],"nine":[49],"half":[52],"after":[54],"onset":[56],"illness.":[59],"There":[60],"no":[62],"family":[63],"history.":[64],"The":[65,115],"neuropathological":[66],"studies":[67],"included":[68],"light":[69],"electron":[71],"microscopy":[72],"cerebral":[75],"biopsy":[76],"complete":[79],"post":[80],"mortem":[81],"examination.":[82],"Myelin":[83],"loss":[84],"cavitation":[86,120],"white":[89,122],"matter":[90,123],"associated":[92],"presence":[95],"sudanophilic":[97,118],"lipid":[98],"abundant":[100],"cells":[101,109],"at":[102],"periphery":[104],"lesions.":[107],"These":[108],"had":[110],"characteristics":[112],"oligodendrocytes.":[114],"combination":[116],"leucodystrophy,":[119],"oligodendrocyte":[125],"proliferation":[126],"has":[127],"previously":[128],"been":[129],"observed":[130],"only":[131],"in":[132],"childhood.":[133]},"counts_by_year":[{"year":2024,"cited_by_count":1},{"year":2022,"cited_by_count":1},{"year":2018,"cited_by_count":1},{"year":2017,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}
