Characterisation of Primary Progressives Aphasias

Launched by ASSISTANCE PUBLIQUE - HÔPITAUX DE PARIS · Nov 18, 2014

Keywords

ClinConnect Summary

Rationale Primary progressive aphasia (PPA) is an umbrella term which identifies a group of neurodegenerative diseases characterized by language deficits. By definition, language disorders are isolated for at least two years; subsequently the disease extends to other cognitive areas leading to a global dementia. Usually PPA affects relatively young patients between 50 and 65 years of age. The outcome is fatal after 10 to 15 years. No treatment is currently available. Three variants of PPA have been described: progressive nonfluent aphasia (PNFA), fluent PPA or semantic dementia (SD), and lo...

Gender

ALL

Eligibility criteria

• • Inclusion criteria A) Patients responding to current diagnostic criteria for Primary Progressive Aphasia
• • 1. Language disorders without important impairments of other cognitive functions
• • 2. Insidious onset and gradual progression
• • Diagnostic criteria for non fluent PPA
• • Non fluent language out-put
• • Phonemic paraphasias and/or agrammatism
• • Relative preservation of speech comprehension
• • Diagnostic criteria for fluent PPA
• • Fluent language out-put
• • Impairment of the access to word meanings leading to comprehension disorders and naming deficits
• • Associative agnosia and/or prosopagnosia may be present. This will allow for the inclusion of patients with multi-modal disorders of meaning (semantic dementia patients).
• • Diagnostic criteria for logopenic PPA
• • Speech out-put with frequent interruptions due to word finding deficits
• • Disorders of sentence comprehension and repetition due to impairment of working memory B) Patients at age of majority C) Patients having given informed and written consent
• • Exclusion criteria A) Cognitive criteria
• • Aphasia severity rating scale of the BDAE \< 3
• • MMS \< 20
• • Severe disorders of executive functions, praxis or episodic memory B) MADRS ≥ 20 (major depression as defined by criteria of the DSM-IV-R) C) Patients whose mother tongue is not French D) Patients affected by of other neurological diseases than PPA or general diseases or physical problems that may impact on cognitive functioning E) Counter-indication for MRI or PET scanning (the lumbar puncture is optional / separated informed consent) F) MRI compatible with pathological processes other than PPA. A mild to moderate leucoaraiosis will not been considered as an exclusion criteria (only patients at stage \> 2 will be excluded from the study) G) Non affiliation at the French healthcare system