Premium
P2–191: Cerebral amyloid angiopathy in a multicenter cohort of people with MCI and Alzheimer's disease
Author(s) -
Enderlé Adeline,
Salleron Julia,
Delmaire Christine,
Gabelle Audrey,
Cordonnier Charlotte,
Blanc Frederic,
Pasquier Florence,
Ha Olivier,
Bombois Stéphanie
Publication year - 2013
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2013.05.836
Subject(s) - superficial siderosis , cerebral amyloid angiopathy , medicine , cohort , hyperintensity , memory clinic , white matter , magnetic resonance imaging , alzheimer's disease , lesion , cardiology , cognitive impairment , disease , pathology , radiology , dementia
We set out to identify the earliest clinical, psychometric, and imaging characteristics of LPA, and to characterize longitudinal clinical outcome. Methods: 9 patients were identified as meeting criteria for LPA, based on consensus between two neurologists and a speech pathologist. Clinical and psychometric characteristics were assessed at baseline and longitudinally. Quantitative MRI analysis was performed to measure regional cortical atrophy. Molecular markers of AD (CSF or PiB-PET) were assessed when available. Results: All patients were identified in early stage LPA (global CDR 0.5), with average follow up of 3.8 years. Quantitative speech and language assessment with our Progressive Aphasia Severity Scale (PASS) demonstrated a distinct initial profile, preserved over longitudinal assessment. Cortical thickness analysis demonstrated focal atrophy in the dominant temporoparietal junction, detectable at an individual level on quantitative MRI. 5 out of 7 subjects had molecular markers consistent with AD. All subjects are still alive, and thus pathologic confirmation is not yet available. Conclusions: It is possible to identify patients with the prototypical clinical phenotype of LPA at the clinical stage of mild cognitive impairment or very mild dementia. Initial disease progression is characterized by worsening language dysfunction, out of proportion to other cognitive domains, and with a profile distinct from other progressive aphasias. There is a signature cortical atrophy pattern in the dominant temporoparietal junction, also identifiable at the earliest clinical stages. These findings provide further support for the consistency of this clinico-anatomic phenotype and potential biomarkers for use in clinical trials.