Frontal presentation of Alzheimer's disease: a series of patients with biological evidence by CSF biomarkers.

Leonardo Cruz De Souza,Marie-Odile Habert,Marie Sarazin,Aurélie Funkiewiez,Aurélie Kas,Bruno Dubois,Maxime Bertoux,Foudil Lamari,Dalila Samri,Carole Azuar

doi:10.1590/s1980-57642013dn70100011

Leonardo Cruz De Souza, Marie-Odile Habert + Show 8 more

Open Access

https://doi.org/10.1590/s1980-57642013dn70100011

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Abstract

Besides its typical amnesic presentation, focal atypical presentations of Alzheimer's disease (AD) have been described in neuropathological studies. These phenotypical variants of AD (so-called "atypical AD") do not follow the typical amnestic pattern and include non-amnestic focal cortical syndromes, such as posterior cortical atrophy and frontal variant AD. These variants exhibit characteristic histological lesions of Alzheimer pathology at post-mortem exam. By using physiopathological markers, such as cerebrospinal fluid markers, it is now possible to establish in vivo a biological diagnosis of AD in these focal cortical syndromes. We report a series of eight patients who were diagnosed with behavioural variant frontotemporal dementia based on their clinical, neuropsychological and neuroimaging findings, while CSF biomarkers showed an AD biological profile, thus supporting a diagnosis of frontal variant of AD.

Highlights

Alzheimer’s disease (AD) has been classically defined as a progressive amnestic neurodegenerative disorder with subsequent emergence of other cognitive and neuropsychiatric changes that impair activities of daily living.[1]
We searched the database at the Memory and Alzheimer Institute of the Pitié-Salpêtrière Hospital for patients for whom a diagnosis of behavioural variant frontotemporal dementia (bvFTD) had been established according to clinical criteria
Clinical and neuropsychological data from patients with frontal AD were compared with three groups of subjects selected from the database of the Memory and Alzheimer Institute of the Pitié-Salpêtrière Hospital: [i] patients with typical amnestic AD (n=18), with cerebrospinal fluid (CSF) AD biological profile (P-Tau/Ab42 ratio higher than 0.21); [ii] patients with bvFTD (n=18) that fulfilled the last revised diagnostic criteria for bvFTD15 and who had normal CSF biomarker profile (P-Tau/Ab42 ratio lower than 0.21); and [iii] normal controls (n=18) selected according to the following criteria: Mini-Mental State Exam (MMSE) ≥27 and normal neuropsychological testing.[16]

Summary

INTRODUCTION

Alzheimer’s disease (AD) has been classically defined as a progressive amnestic neurodegenerative disorder with subsequent emergence of other cognitive and neuropsychiatric changes that impair activities of daily living.[1] In typical presentations, patients with AD manifest early episodic memory deficit followed by various associations with executive, language and visuospatial deficits. The identification of this specific clinical and cognitive profile has been the core of the clinical diagnosis of AD, as established by the NINCDS–ADRDA criteria.[2]. We report a series of eight patients who were diagnosed with bvFTD based on their clinical, neuropsychological and neuroimaging findings, while CSF biomarkers showed an AD biological profile, supporting a diagnosis of frontal variant of AD

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