Natural History of ALS among Different Populations in PACTALS

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1 Natural History of ALS among Different Populations in PACTALS Dongsheng Fan Peking University Third Hospital Beijing, China

2 REVIEW Amyotrophic lateral sclerosis and motor neuron syndromes in Asia N Shahrizaila, 1,2 G Sobue, 3 S Kuwabara, 4 S H Kim, 5 Carol Birks, 6 D S Fan, 7 J S Bae, 8 C J Hu, 9 M Gourie-Devi, 10 Y Noto, 2,11 K Shibuya, 2,4 K J Goh, 1 R Kaji, 12 CP Tsai, 13 L Cui, 14 P Talman, 15 R D Henderson, 16 S Vucic, 17 M C Kiernan 2

3 Background ALS research largely originate from North America and Europe However, Pan-Asia represents >50% of the world population Pan-Asia represents a diverse population in terms of ethnic, social and cultural backgrounds

4 Comparisons between Pan-Asia and other regions Mean/median age at onset (years) India China Japan Australia North America Europe Youngest Eldest

5 Comparisons between Pan-Asia and other regions M:F ratio Australia Japan China India 3 North America Europe Lowest Highest

6 Comparisons between Pan-Asia and other regions Percentage of bulbar-onset ALS India China Japan Australia 36.8 North America Europe Lowest Highest

7 Comparisons between Pan-Asia and other regions Mean/median survival (months) Auatralia* Japan 48 China India North America Europe Shortest Longest *:The difference of survival in Australia was because of different phenotypes

8 Comparisons between developing and developed Pan-Asia areas Age at onset (years) Developing Asia- Pacific areas Developed Asia- Pacific areas North America Europe Lowest Highest *Developed Asia-Pacific areas include Japan, China Hong Kong, China Taiwan and Australia; Developing Asia-Pacific areas include Mainland China and India

9 Canada Incidence: M:F 0.98:1 Age at onset: 62.8 Survival: 25.2 months Europe West Incidence: Prevalence: M:F :1 Age at onset: Survival: 34.8 months Europe North Incidence: Prevalence: M:F :1 Age at onset: Survival: months Mainland China Incidence: NA M:F :1 Age at onset: Survival: 71 months USA Incidence: Prevalence: M:F :1 Age at onset: Survival: months Japan Incidence: Prevalence: M:F :1 Age at onset: Survival: 48 months Europe South Incidence: Prevalence: M:F :1 Age at onset: Survival: months Taiwan, China Incidence: 0.51 Prevalence: 1.97 M:F 1.65:1 Age at onset: Survival: 66.6 months Hong Kong, China Incidence: Prevalence: M:F :1 Age at onset: Survival: NA Brazil Incidence: 0.4 Prevalence: 0.9 M:F :1 Age at onset: Survival: months Argentina Incidence: 6 (based on OPD attendance) M:F 1.59:1 Age at diagnosis: 55 Survival: NA Australia Incidence: NA M:F :1 Age at onset: Survival: months (different phenotypes) Uruguay Incidence: Prevalence: 1.9 M:F 2:1 Age at onset: 58.7 Survival: NA Africa Incidence: NA M:F 1.4-4:1 Age at onset: 50 Survival: 35.2 months India Incidence: (based on OPD attendance) M:F 3:1 Age at onset: 46.2 Survival: months

10 Comparisons between developing and developed Pan-Asia areas M:F ratio Developing Asia- Pacific areas Developed Asia- Pacific areas North America Europe Lowest Highest *Developed Asia-Pacific areas include Japan, China Hong Kong, China Taiwan and Australia; Developing Asia-Pacific areas include Mainland China and India

11 Comparisons between developing and developed Pan-Asia areas Percentage of bulbar-onset ALS Developing Asia- Pacific areas Developed Asia- Pacific areas North America Europe Lowest Highest *Developed Asia-Pacific areas include Japan, China Hong Kong, China Taiwan and Australia; Developing Asia-Pacific areas include Mainland China and India

12 Comparisons between developing and developed Pan-Asia areas Mean/median Diagnostic delay (months) Developing Asia- Pacific areas Developed Asia- Pacific areas North America Europe Shortest Longest *Developed Asia-Pacific areas include Japan, China Hong Kong, China Taiwan and Australia; Developing Asia-Pacific areas include Mainland China and India

13 Comparisons between developing and developed Pan-Asia areas Mean/median survival (months) Developing Asia- Pacific areas Developed Asia- Pacific areas North America Europe Shortest Longest *Developed Asia-Pacific areas include Japan, China Hong Kong, China Taiwan and Australia; Developing Asia-Pacific areas include Mainland China and India

14 Canada Incidence: M:F 0.98:1 Age at onset: 62.8 Survival: 25.2 months Europe West Incidence: Prevalence: M:F :1 Age at onset: Survival: 34.8 months Europe North Incidence: Prevalence: M:F :1 Age at onset: Survival: months Mainland China Incidence: NA M:F :1 Age at onset: Survival: 71 months USA Incidence: Prevalence: M:F :1 Age at onset: Survival: months Japan Incidence: Prevalence: M:F :1 Age at onset: Survival: 48 months Europe South Incidence: Prevalence: M:F :1 Age at onset: Survival: months Taiwan, China Incidence: 0.51 Prevalence: 1.97 M:F 1.65:1 Age at onset: Survival: 66.6 months Hong Kong, China Incidence: Prevalence: M:F :1 Age at onset: Survival: NA Brazil Incidence: 0.4 Prevalence: 0.9 M:F :1 Age at onset: Survival: months Argentina Incidence: 6 (based on OPD attendance) M:F 1.59:1 Age at diagnosis: 55 Survival: NA Australia Incidence: NA M:F :1 Age at onset: Survival: months (different phenotypes) Uruguay Incidence: Prevalence: 1.9 M:F 2:1 Age at onset: 58.7 Survival: NA Africa Incidence: NA M:F 1.4-4:1 Age at onset: 50 Survival: 35.2 months India Incidence: (based on OPD attendance) M:F 3:1 Age at onset: 46.2 Survival: months

15 Advantages for the natural history study of ALS in Pan-Asia 1. A large and growing number of patients 2. Multiple ethnic populations 3. Both developed and developing countries 4. Diverse habits and customs 5. Diverse climate

16 Aims and Objectives 1. To describe the natural history of ALS in Pan-Asian countries 2. To identify prognostic factors for survival in Pan-Asian countries 3. To compare the natural history, clinical features and prognostic factors both among different Pan-Asian countries and between the Pan-Asian region and other regions 4. To uncover environmental, ethnic, social and cultural factors underlying certain features specific to Pan-Asian ALS patients 5. To accelerate to establish the multicenter database for PACTALS

17 Brief methodology ALS patients in Pan-Asian countries will be collected with uniform criteria and CRF. ü Make the diagnosis clean (excluded SBMA, other LMNS, ALS-mimic, etc.) ü Various phenotypes of ALS (such as FAS, UNM-D ALS, IBALS, but not Hirayama disease, Madras MND, and ALS- PD-dementia complex) ü Non-motor impairment (including to cognitive, behavioral, even sleeping)

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19 Paresis Death Vacuolation Loss of MN 40d 90d 125d 136d

20 orexins

21 Expected outcomes 1. Pan-Asian ALS patients have different natural history, clinical features and prognostic factors compared to European and North American patients. 2. Multiple environmental, ethnic, social and cultural factors account for the heterogeneity within Pan- Asia countries, and these factors may not have been identified in previous studies