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J Alzheimers Dis. 2015;47(1):95-101. doi: 10.3233/JAD-150203.

Progressive Supranuclear Palsy Syndrome and Semantic Dementia in Neuropathologically Proven Lewy Body Disease: A Report of Two Cases.

Author information

1
Neurology of Memory and Language Unit, Université Paris Descartes, Sorbonne Paris Cité, INSERM UMR S894, Centre Hospitalier Sainte Anne, Paris, France.
2
AP-HP, Groupe hospitalier Pitié-Salpêtrière, Service de Médecine Nucléaire and Université Pierre et Marie Curie-Paris 6, INSERM, UMR-S 678, Paris, France.
3
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.

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.

KEYWORDS:

Alzheimer’s disease; Lewy body; neuropathology; neuropsychology; progressive; supranuclear palsy

PMID:
26402758
DOI:
10.3233/JAD-150203
[Indexed for MEDLINE]

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