Poster Presentations: P2 P2-101
FINDINGS FROM A TUNISIAN COHORT OF DEMENTIA WITH LEWY BODIES
Amina Gargouri, Leila Sellami, Imen Kacem, Amira Nasri, Youssef Sidhom, Yosr Hizem, Mouna Ben Djebara, and Riadh Gouider, UR12SP21, Neurology, Razi Hospital, Tunis, Tunisia. Contact e-mail:
[email protected] Background: Dementia with Lewy bodies (DLB) is the second major cause of degenerative dementia (1.2- 13.6% of causes of dementia in memory centers and 20% of autopsy series). However, it remains underdiagnosed. We aimed to establish the characteristic profile of DLB in a Tunisian cohort and to compare it to western countries. Methods: A 12 years retrospective study (2002-
2014) including all the patients diagnosed with probable or possible DLB according to revised McKeith criteria of 2005. Demographic, clinical neuropsychological, paraclinical and therapeutic features were analyzed. Results: We included 128 patients (6%of all dementias). Sex-ratio was 1.17 and mean age of onset at 73.5 years [5192]. Mean time to diagnosis was 3,3years. Major clinical features were: visual hallucinations (92%), apraxia (90%), memory troubles (86%) and parkinsonian syndrome (84%). Fluctuations were found in 70% and RBD in 17.1%. Neuropsychological assessment found mainly dysexecutive (97%), memory (96%) and visuospatial/ constructive (44.5%) disorders. Electroencephalography found FIRDA pattern in 6%. Brain imaging showed diffuse atrophy (50%). Main used drugs were hypnotics (53%), anticholinestrasics (30%), levodopa (26%, with response in 25% cases). Intolerance was noted in a quarter of the patients that used neuroleptics before diagnosis. Conclusions: Male predominance and age of onset between 50 and 83 years were noted as classically described. However, clinically, we found a higher frequency of memory troubles and a lower frequency of visuospatial/constructive disorders and RBD than previously reported. FIRDA pattern in EEG and diffuse atrophy with preservation of hippocampus were considered as additional criteria for DLB. Dopa-responsiveness was found in 25% of patients especially at early stages. The cholinergic deficit in DLB may explain the responsiveness to anticholinesterasics in hallucinations and memory troubles.
P2-102
A SYSTEMATIC REVIEW OF FAMILIAL ALZHEIMER’S DISEASE 1991-2014: ASIANS AND WORLDWIDE DATA
Yat Fung Shea1, Leung Wing Chu1, Angel On-Kei Chan1, Joyce Ha1, YouQiang Song2, 1Queen Mary Hospital, Hong Kong, Hong Kong; 2University of Hong Kong, Hong Kong, Hong Kong. Contact e-mail: elphashea@gmail. com Background: There are great diversities of clinical phenotypes among
the familial Alzheimer’s disease (FAD) families. The objective of this study was to systematically review all the previously reported cases of FAD and to compare the phenotypic differences between Asian and Caucasian patients. Methods: Articles were searched through AD&FTDMDB, the ALZFORUM and PubMed with the terms “early onset Alzheimer’s disease”, “autosomal dominant Alzheimer’s disease”, “familial Alzheimer’s disease”, “presenilin”, “PSEN1”, “PSEN2”, and “APP”. Results: Individual level data were collected from 655 pedigrees with 1884 patients. Overall, presenilin 1 (PSEN1) mutations had the earliest age of onset (AOO) (43.368.6 years, p
