title: Progressive Supranuclear Palsy Syndrome and Semantic Dementia in Neuropathologically Proven Lewy Body Disease: A Report of Two Cases.
abstract: The impact of neuropathological lesions on the clinical symptoms and progression of Lewy body disease (LBD) remains unclear. To address this issue, we describe two illustrative cases of autopsy-proven LBD that presented atypical phenotypes of progressive supranuclear palsy syndrome and semantic dementia. Postmortem examination revealed diffuse LBD with massive brainstem involvement in case 1, whereas Lewy bodies predominated in the amygdala and neocortex in case 2. Alzheimer's disease pathology was present in both cases, and TDP-43 inclusions were noted in case 2. These cases illustrate two contrasted clinical presentations and highlight the heterogeneity within the underlying proteinopathies of neurodegenerative diseases.
authors.affiliation: Neurology of Memory and Language Unit, Université Paris Descartes, Sorbonne Paris Cité, INSERM UMR S894, Centre Hospitalier Sainte Anne, Paris, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Lagarde
authors.affiliation: Neurology of Memory and Language Unit, Université Paris Descartes, Sorbonne Paris Cité, INSERM UMR S894, Centre Hospitalier Sainte Anne, Paris, France.
authors.forename: Lorraine
authors.initials: L
authors.lastname: Hamelin
authors.affiliation: Neurology of Memory and Language Unit, Université Paris Descartes, Sorbonne Paris Cité, INSERM UMR S894, Centre Hospitalier Sainte Anne, Paris, France.
authors.forename: Valé
authors.initials: V
authors.lastname: Hahn
authors.affiliation: AP-HP, Groupe hospitalier Pitié-Salpêtrière, Service de Médecine Nucléaire and Université Pierre et Marie Curie-Paris 6, INSERM, UMR-S 678, Paris, France.
authors.forename: Marie-Odile
authors.initials: MO
authors.lastname: Habert
authors.affiliation: Laboratoire de Neuropathologie Escourolle, Assistance Publique-Hôpitaux de Paris, Université Pierre and Marie Curie, and Institut du Cerveau et de la Moelle, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Danielle
authors.initials: D
authors.lastname: Seilhean
authors.affiliation: Laboratoire de Neuropathologie Escourolle, Assistance Publique-Hôpitaux de Paris, Université Pierre and Marie Curie, and Institut du Cerveau et de la Moelle, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Charles
authors.initials: C
authors.lastname: Duyckaerts
authors.affiliation: Neurology of Memory and Language Unit, Université Paris Descartes, Sorbonne Paris Cité, INSERM UMR S894, Centre Hospitalier Sainte Anne, Paris, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Sarazin
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000378:metabolism; D001921:Brain / Q000378:metabolism / Q000473:pathology; D004268:DNA-Binding Proteins / Q000378:metabolism; D005260:Female; D057180:Frontotemporal Dementia / Q000209:etiology*; D006801:Humans; D020961:Lewy Body Disease / Q000150:complications* / Q000473:pathology; D008297:Male; D008609:Mental Status Schedule; D009483:Neuropsychological Tests; D034702:RNA-Binding Protein FUS / Q000378:metabolism; D013494:Supranuclear Palsy, Progressive / Q000209:etiology*; D025801:Ubiquitin / Q000378:metabolism; D016875:tau Proteins / Q000378:metabolism
chemical_list: D016229:Amyloid beta-Peptides; D004268:DNA-Binding Proteins; D034702:RNA-Binding Protein FUS; C000598624:TARDBP protein, human; D025801:Ubiquitin; D016875:tau Proteins
keywords: Alzheimer’s disease; Lewy body; neuropathology; neuropsychology; progressive; supranuclear palsy