Family history of frontotemporal lobar degeneration in Asia--an international multi-center research.
BACKGROUND
Previous studies in western countries have shown that about 30%-50% of patients with frontotemporal lobar degeneration (FTLD) have a positive family history, whereas the few epidemiological studies on FTLD done in Asia reported much lower frequencies. It is not clear the reason why the frequencies of FTLD with positive family history were lower in Asia. Furthermore, these findings were not from studies focused on family history. Therefore, it is necessary to conduct further studies on the family history of FTLD in Asia. This international multi-center research aims to investigate the family histories in patients with FTLD and related neurodegenerative diseases such as progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), and motor neuron diseases in a larger Asian cohort.


METHODS
Participants were collected from five countries: India, Indonesia, Japan, Taiwan, and Philippines. All patients were diagnosed with behavioral variant frontotemporal dementia (bvFTD), semantic dementia (SD), progressive non-fluent aphasia (PA), frontotemporal dementia with motor neuron disease (FTD/MND), PSP, and corticobasal degeneration (CBD) according to international consensus criteria. Family histories of FTLD and related neurodegenerative diseases were investigated in each patient.


RESULTS
Ninety-one patients were included in this study. Forty-two patients were diagnosed to have bvFTD, two patients had FTD/MND, 22 had SD, 15 had PA, one had PA/CBS, five had CBS and four patients had PSP. Family history of any FTLD spectrum disorder was reported in 9.5% in bvFTD patients but in none of the SD or PA.


CONCLUSIONS
In contrast to patients of the western countries, few Asian FTLD patients have positive family histories of dementia.
Department of Neuropsychiatry,Faculty of Life Science,Kumamoto University,1-1-1,Honjo,Chuo-ku,Kumamoto-city,Japan.
Ryuji
R
Fukuhara
Department of Neurology and Cognitive Neurology Unit,Apollo Gleneagles Hospitals,No. 58,Canal Circular Road,Kolkata 700 054,West Bengal,India.
Amitabha
A
Ghosh
Department of Neurology,Taipei Veterans General Hospital,No. 201,Sec. 2,Shipai Road,Beitou District,Taipei City,Taiwan.
Jong-Ling
JL
Fuh
St. Lukes Medical Center,279 E. Rodrtiguez Sr. Boulevard,Quezon City,Metro Manila,Philippines.
Jacqueline
J
Dominguez
Department of Neurology,Hasan Sadikin Hospital,Faculty of Medicine,Padjadjaran University,Bandung,West Java,Indonesia.
Paulus Anam
PA
Ong
Department of Neurology and Cognitive Neurology Unit,Apollo Gleneagles Hospitals,No. 58,Canal Circular Road,Kolkata 700 054,West Bengal,India.
Aparna
A
Dutt
Neurological Center,Cardinal Tien Hospital,No. 362,Zhongzheng Road,Xindian District,New Taipei City,231,Taiwan.
Yi-Chien
YC
Liu
Department of Neuropsychiatry,Faculty of Life Science,Kumamoto University,1-1-1,Honjo,Chuo-ku,Kumamoto-city,Japan.
Hibiki
H
Tanaka
Department of Neuropsychiatry,Faculty of Life Science,Kumamoto University,1-1-1,Honjo,Chuo-ku,Kumamoto-city,Japan.
Manabu
M
Ikeda
D001208:Asia / Q000453:epidemiology; D001526:Behavioral Symptoms / Q000175:diagnosis; D005190:Family; D005192:Family Health / Q000706:statistics & numerical data; D005260:Female; D057174:Frontotemporal Lobar Degeneration / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000523:psychology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010045:Outpatients / Q000706:statistics & numerical data; D010375:Pedigree