Logopenic Variant of Primary Progressive Aphasia in Lewy Body Dementia: Beyond Alzheimer's Disease (P05.038)

Abstract

Objective: To describe a patient affected by the logopenic variant of primary progressive aphasia (lv-PPA) with a clinical evolution towards dementia with Lewy bodies (DLB). Background Lv-PPA is a progressive language disorder characterized by slow speech, word finding problems, sentence repetition and comprehension deficits, and relative sparing of motor speech, grammar, and single-word comprehension. Brain atrophy is located at the left temporo-parietal junction. Biological and neuropathological studies suggest that the main cause of lv-PPA is Alzheimer9s disease (AD). Design/Methods: Clinical, cognitive and language data were acquired in a 65-years-old patient with lv-PPA (right-handed man, disease duration at presentation 24 months). Furthermore, we analyzed cerebrospinal fluid (CSF) biomarkers, structural MRI, brain Single-photon emission computed tomography (SPECT) and dopamine transporter (DAT)-scan. Results: For the first two years (2006-2008), the patient demonstrated isolated language deficits with typical logopenic profile. In 2008: MRI was normal and SPECT showed hypoperfusion in left posterior temporal and inferior parietal regions. CSF analysis revealed a non-AD profile, with normal levels of beta-amyloid, tau and phosphorylated tau. On the basis of these elements, the diagnosis of lv-PPA was made. Two years later, visual hallucinations, cognitive fluctuations and parkinsonian signs appeared; neuroleptic medication was not tolerated. DAT-scan demonstrated a bilateral DAT loss. The second SPECT showed a posterior extension of cortical hypoperfusion including parietal, temporal and occipital regions, with involvement of the left primary visual cortex. Accordingly with this clinical and imaging evolution, the clinical diagnosis of DLB was made. Conclusions: We provided clinical, imaging and biological findings showing that the lv-PPA is not necessarily related to AD pathology. Hence, the medical community should not systematically lump together lv-PPA and AD. Conversely, DLB should be considered as a heterogeneous disease, which can initially present with a progressive aphasia profile, namely lv-PPA. Supported by: Dr. Migliaccio was funded by the Neuropole de Recherche Francilien (NeRF). Disclosure: Dr. Migliaccio has nothing to disclose. Dr. Roue-Jagot has nothing to disclose. Dr. Dubois has received personal compensation for activities with Bristol-Myers Squibb, Roche, Elan, Eli Lilly, Eisai, and Janssen as a consultantDr. Dubois has received personal compensation for serving on the advisory board of Bristol-Myers Squibb, Roche, Elan, Eli Lilly, Eisai, and Janssen.Dr. Dubois has received research support from Novartis and Sanofi-Aventis. Dr. Teichmann has nothing to disclose.