Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

Neurological Sciences

Volumn 36, Issue 12, 2015, Pages 2243-2252

  Mandrioli, J ^a   Biguzzi, S ^b   Guidi, C ^t   Sette, E ^d   Terlizzi, E ^e   Ravasio, A ^f   Casmiro, M ^g   Salvi, F ^h   Liguori, R ^u   Rizzi, R ^j   Pietrini, V ^k   Borghi, A ^l   Rinaldi, R ^m   Fini, N ^a   Chierici, E ⁿ   Santangelo, M ^o   Granieri, E ^p   Mussuto, V ^q   De Pasqua, S ^u   Georgoulopoulou, E ^a   Fasano, A ^a   Ferro, S ^s   D’Alessandro, Roberto ⁱ   Michelucci, R ^h   Venturini, E ^b   Passarin, M G ^b   Neri, W ^t   Tugnoli, V ^d   Tola, M R ^d   Guidetti, D ^e   Curro Dossi M ^f   Pasquinelli, M ^f   Andruccioli, J ^f   Rasi, F ^g   Bartolomei, I ^h   Avoni, P ^u   Canali, E ^j   Marcello, N ^j   Grassi, A ^k   Delay, L ^k   Aiello, M ^k   Montanari, E ⁿ   Amidei, S ^o   Greco, G ^o   Casetta, I ^p   Groppo, E ^p   De Massis, P ^q   Gabellini, A ^l   Sacquegna, T ^l   Cirignotta, F ^m   D'Alessandro, R ⁱ   more..

^a Gastroenterology and Digestive Endoscopy Unit New S Agostino Estense Hospital   (Italy)

^b BUFALINI HOSPITAL   (Italy)

^c Forli Hospital   (Italy)

^d S ANNA HOSPITAL   (Italy)

^e GUGLIELMO DA SALICETO HOSPITAL   (Italy)

^f Infermi Hospital   (Italy)

^g Faenza and Ravenna Hospital ^*  (Italy)

^h BELLARIA HOSPITAL   (Italy)

ⁱ IRCCS ISTITUTO DELLE SCIENZE NEUROLOGICHE DI BOLOGNA   (Italy)

^j S MARIA NUOVA HOSPITAL   (Italy)

^k UNIVERSITY OF PARMA   (Italy)

^l MAGGIORE HOSPITAL   (Italy)

^m UNIVERSITY OF BOLOGNA   (Italy)

ⁿ Fidenza Hospital   (Italy)

^o Carpi Hospital   (Italy)

^p UNIVERSITY OF FERRARA   (Italy)

^q Hospital of Imola   (Italy)

^r UNIVERSITY OF MODENA AND REGGIO EMILIA   (Italy)

^s Drug Policy Service   (Italy)

^t Forl Hospital   (Italy)

^u UNIVERSITY OF BOLOGNA   (Italy)

more..

Author keywords

ALSFRS R; Amyotrophic lateral sclerosis; Disease progression; Heterogeneity; Population based study

Indexed keywords

RILUZOLE;

ADULT; AGED; AMYOTROPHIC LATERAL SCLEROSIS; AMYOTROPHIC LATERAL SCLEROSIS FUNCTIONAL RATING SCALE; ARTICLE; BODY MASS; DETERIORATION; FEMALE; HUMAN; ITALY; MAJOR CLINICAL STUDY; MALE; NEUROLOGIC DISEASE ASSESSMENT; ONSET AGE; OVERALL SURVIVAL; PHENOTYPE; POPULATION RESEARCH; PROGNOSTIC ASSESSMENT; REGISTER; SURVIVAL; SURVIVAL RATE; AGE; DISEASE COURSE; MIDDLE AGED; MORTALITY; PROGNOSIS; PROSPECTIVE STUDY; VERY ELDERLY;

ADULT; AGE FACTORS; AGE OF ONSET; AGED; AGED, 80 AND OVER; AMYOTROPHIC LATERAL SCLEROSIS; DISEASE PROGRESSION; FEMALE; HUMANS; ITALY; MALE; MIDDLE AGED; PROGNOSIS; PROSPECTIVE STUDIES; REGISTRIES;

EID: 84937820094     PISSN: 15901874     EISSN: 15903478     Source Type: Journal    
DOI: 10.1007/s10072-015-2343-6     Document Type: Article

References (24)

1. Hardiman O, van den Berg LH, Kiernan MC (2011) Clinical diagnosis and management of amyotrophic lateral sclerosis. Nat Rev Neurol 7:639–649. doi:10.1038/nrneurol.2011.153
2. Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169:13–21
3. Kaufmann P, Levy G, Montes J, Buchsbaum R, Barsdorf AI, Battista V, Arbing R, Gordon PH, Mitsumoto H, Levin B (2007) Thompson JL; QALS study group. Excellent inter-rater, intra-rater, and telephone-administered reliability of the ALSFRS-R in a multicenter clinical trial. Amyotroph Lateral Scler 8:42–46
4. Kasarskis EJ, Dempsey-Hall L, Thompson MM, Luu LC, Mendiondo M, Kryscio R (2005) Rating the severity of ALS by caregivers over the telephone using the ALSFRS-R. Amyotroph Lateral Scler Other Motor Neuron Disord. 6:50–54
5. Miano B, Stoddard GJ, Davis S, Bromberg MB (2004) Interevaluator reliability of the ALS functional rating scale. Amyotroph Lateral Scler Other Motor Neuron Disord 5:235–239
6. The Amyotrophic Lateral Sclerosis Functional Rating Scale (1996) Assessment of activities of daily living in patients with amyotrophic lateral sclerosis. The ALS CNTF treatment study (ACTS) phase I-II Study Group. Arch Neurol 53:141–147
7. Kaufmann P, Levy G, Thompson JL, Delbene ML, Battista V, Gordon PH, Rowland LP, Levin B, Mitsumoto H (2005) The ALSFRS-R predicts survival time in an ALS clinic population. Neurology 64:38–43
8. Gordon PH, Cheng B, Salachas F, Pradat PF, Bruneteau G, Corcia P, Lacomblez L, Meininger V (2010) Progression in ALS is not linear but is curvilinear. J Neurol 257:1713–1717. doi:10.1007/s00415-010-5609-1
9. Mandrioli J, Biguzzi S, Guidi C, Venturini E, Sette E, Terlizzi E, Ravasio A, Casmiro M, Salvi F, Liguori R, Rizzi R, Pietrini V, Chierici E, Santangelo M, Granieri E, Mussuto V, Borghi A, Rinaldi R, Fini N, Georgoulopoulou E, De Pasqua S, Vinceti M, Bonvicini F, Errals Group, Ferro S, D’Alessandro R (2014) Epidemiology of amyotrophic lateral sclerosis in Emilia Romagna Region (Italy): a population based study. Amyotroph Lateral Scler Frontotemporal Degener 15:262–268. doi:10.3109/21678421.2013.865752
10. Chiò A, Calvo A, Moglia C, Mazzini L, Mora G, PARALS study group (2011) Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry 82:740–746. doi:10.1136/jnnp.2010.235952
11. Kimura F, Fujimura C, Ishida S, Nakajima H, Furutama D, Uehara H, Shinoda K, Sugino M, Hanafusa T (2006) Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology 66:265–267
12. Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1:293–299
13. Gomeni R, Fava M, Pooled Resource Open-Access ALS Clinical Trials Consortium (2014) Amyotrophic lateral sclerosis disease progression model. Amyotroph Lateral Scler Frontotemporal Degener. 15:119–129. doi:10.3109/21678421.2013.838970
14. Creemers H, Grupstra H, Nollet F, van den Berg LH, Beelen A (2015) Prognostic factors for the course of functional status of patients with ALS: a systematic review. J Neurol 262:1407–1423. doi:10.1007/s00415-014-7564-8
15. Gordon PH, Cheng B, Montes J, Doorish C, Albert SM, Mitsumoto H (2007) Outcome measures for early phase clinical trials. Amyotroph Lateral Scler 8:270–273
16. Watanabe H, Atsuta N, Nakamura R, Hirakawa A, Watanabe H, Ito M, Senda J, Katsuno M, Izumi Y, Morita M, Tomiyama H, Taniguchi A, Aiba I, Abe K, Mizoguchi K, Oda M, Kano O, Okamoto K, Kuwabara S, Hasegawa K, Imai T, Aoki M, Tsuji S, Nakano I, Kaji R, Sobue G (2015) Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener 16:230–236. doi:10.3109/21678421.2014.990036
17. Franchignoni F, Mora G, Giordano A, Volanti P, Chiò A (2013) Evidence of multidimensionality in the ALSFRS-R Scale: a critical appraisal on its measurement properties using Rasch analysis. J Neurol Neurosurg Psychiatry 84:1340–1345. doi:10.1136/jnnp-2012-304701
18. Tanaka Y, Yoshikura N, Harada N, Yamada M, Koumura A, Sakurai T, Hayashi Y, Kimura A, Hozumi I, Inuzuka T (2012) Late-onset patients with sporadic amyotrophic lateral sclerosis in Japan have a higher progression rate of ALSFRS-R at the time of diagnosis. Intern Med 51:579–584
19. Magnus T, Beck M, Giess R, Puls I, Naumann M, Toyka KV (2002) Disease progression in amyotrophic lateral sclerosis: predictors of survival. Muscle Nerve 25:709–714
20. Castrillo-Viguera C, Grasso DL, Simpson E, Shefner J, Cudkowicz ME (2010) Clinical significance in the change of decline in ALSFRS-R. Amyotroph Lateral Scler. 11:178–180. doi:10.3109/17482960903093710
21. Chiò A, Canosa A, Gallo S et al (2011) ALS clinical trials: do enrolled patients accurately represent the ALS population? Neurology 77:1432–1437
22. Franchignoni F, Mandrioli J, Giordano A, Ferro S, ERRALS Group (2015) A further Rasch study confirms that ALSFRS-R does not conform to fundamental measurement requirements. Amyotroph Lateral Scler Frontotemporal Degener 27:1–7 [Epub ahead of print]
23. Wicks P, Massagli MP, Wolf C, Heywood J (2009) Measuring function in advanced ALS: validation of ALSFRS-EX extension items. J Eur Neurol 16:353–359
24. Miller RG, Moore DH, Spitalny GM, Gordon PH (2007) Is the ALSFRS-R rate of decline linear over time? Amyotroph Lateral Scler 8(Suppl 1):140–155