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Logopenic progressive aphasia beyond Alzheimer's—an evolution towards dementia with Lewy bodies
  1. Marc Teichmann1,2,
  2. Raffaella Migliaccio2,3,
  3. Aurélie Kas4,5,
  4. Bruno Dubois1,2
  1. 1Department of Neurology, Centre de référence “Démences Rares”, Hôpital de la Pitié Salpêtrière, Paris, France
  2. 2INSERM UMRS 975 – CRICM, Hôpital de la Pitié Salpêtrière, Paris, France
  3. 3Department of Psychology, Catholic University, Milan, Italy
  4. 4Service de Médecine Nucléaire, AP-HP, Hôpital de la Pitié-Salpêtrière, Paris, France
  5. 5Université Pierre et Marie Curie-Paris 6, INSERM, UMR-S 678, Paris, France
  1. Correspondence to Dr Marc Teichmann, Department of Neurology, Centre de référence “Démences Rares”, Hôpital de la Pitié Salpêtrière, 47-83, boulevard de l'Hôpital, Paris 75013, France; marc.teichmann{at}psl.aphp.fr

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Introduction

Primary progressive aphasia (PPA) is an umbrella term which identifies a group of neurodegenerative diseases manifested by relatively isolated language disorders. The logopenic variant (LPA), is characterised by a ‘word-on-the-tip-of-the-tongue phenomenon’ and anomia as well as by comprehension and repetition difficulties for sentences due to verbal working memory deficits.1 Cortical atrophy typically affects the left temporal-parietal junction.1 According to most studies LPA is primarily due to underlying Alzheimer's Disease (AD) pathology as suggested by biomarker findings from positron emission tomography with Pittsburgh compound B binding to amyloid deposits (PET-PIB) imaging and CSF (cerebrospinal fluid) β-amyloid and τ measures as well as by neuropathological data.2

However, PET-PIB and CSF data are drawn from relatively small PPA populations, which makes it difficult to conclude that LPA is invariably underpinned by AD pathology. Furthermore, neuropathological results from the most important sample of LPA patients have demonstrated that only seven out of 11 patients had AD while the remaining four patients had pathology characteristic of frontotemporal lobar degeneration.2 Finally, follow-up studies showing that LPA …

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