COMORBIDITIES IN ALS PATIENTS FROM EMILIA
ROMAGNA: A POPULATION BASED STUDY.
J. Mandrioli, N. Fini, E. Georgoulopoulou, S. Biguzzi, E. Venturini, C. Guidi, E. Sette, E. Terlizzi, A. Ravasio, M.
Casmiro, F. Salvi, R. Liguori, R. Rizzi, V. Pietrini, E. Chierici, M. Santangelo, E. Granieri, V. Mussuto, A. Borghi, R.
Rinaldi, S. De Pasqua, R. D’Alessandro and ERRALS Group.
Neurological Departments of Modena, Cesena, Forlì, Ferrara, Piacenza, Rimini, Ravenna, Bologna, Reggio Emilia,
Parma, Fidenza, Carpi, Imola
INTRODUCTION
There have been few studies of comorbidities among patients with ALS, reporting contrasting results on cardiovascular, autoimmune, metabolic or
neurological diseases (1). Among clinicians, there is a general perception that patients with ALS can be premorbidly healthier than the general
population, and previous papers reported an increased risk of ALS among people doing professional physical activity (2), with a lower body mass
index (3), and reduced cardiovascular risk (4). We performed a population-based study in Emilia Romagna Region focusing on ALS comorbidities.
METHODS
From 2009 onwards, a prospective registry, involving 9 provinces has been collecting all cases of incident ALS among residents in Emilia
Romagna region (population 4.4 million inhabitants). For each patient, the main demographic and clinical information, including comorbidities, were
collected by the caring physicians and input in a dedicated website at diagnosis.
RESULTS
From 1.1.2009 to 31.12.2013 in Emilia Romagna newly diagnosed ALS patients were 566 (M/F: 1.2; mean age at onset: 66.4 years). Patients with
a history of chronic/ongoing diseases were 445 (78.6%): 247 (55.0%) had hypertension, 76 (17.07%) heart diseases, 57 (12.81%) neoplasms, 55
(12.36%) thyroid disorders, 50 (11.24%) diabetes, 50 (11.24%) chronic obstructive pulmonary disease (COPD), 39 (8.76%) dyslipidemia, 29
depression (6.52%), 26 (5.84%) cerebrovascular diseases, 23 (5.17%) rheumatic diseases. Among neurological diseases: 46 (10.33%) dementia,
12 (2.70%) Parkinson’s Disease, 5 (1.12%) epilepsy, 2 (0.45%) mental retardation, only 1 case of myasthenia, and 1 case of multiple sclerosis
were observed. Patients with comorbidities were older (68 vs 60 yrs at onset, p<0.01), more often with bulbar onset (39.55% vs 21.49%, p<0.01).
Flail phenotypes were more often represented among patients without comorbidities; the opposite for respiratory and bulbar phenotype. At
univariate analysis patients with comorbidities had an HR of 1.34 (p=0.03), but at multivariate analysis disease duration was not significnatly
different between patients having comorbidities or not.
1.00
0.75
14.22
60.28
26 (21.49%)
95 (78.51%)
28 (23.14%)
54 (44.62%)
31 (25.6%)
6 (4.95%)
>4 diseases
5.6%
4 diseases
12.5%
No comorbidities
21.4%
no comorbidities
1 disease
0.50
12.55
68.11
176 (39.55%)
269 (60.35%)
165 (37.07%)
186 (41.79%)
73 (16.40%)
21 (4.72%)
N. of diseases
Survival estimates by comorbidities
2 diseases
3 diseases
15.7%
1 disease
20.1%
0.25
No comorbidities
70/51; 1.37
2 diseases
24.6%
0.00
sex (M/F; ratio)
onset-diagnosis
(months)
age at onset
bulbar onset
spinal onset
bulbar phenotype
classic phenotype
flail phenotype
UMNp phenotype
Respiratory
phenotype
Comorbidities
242/203; 1.19
0
12
24
36
48
60
72
84
96
108
120
132
3 diseases
4 diseases
>4 diseases
144
survival from onset (months)
17 (3.82%)
2 (1.65%)
no comorbidities
comorbidities
DISCUSSION
Patients with ALS had great comorbidities with hypertension, diabetes, neoplasm, heart diseases,
COPD, and thyroid disorders, as expected from a population with a mean age at onset of 66 years.
The presence of these diseases does not modify disease progression and survival in patients with
ALS. We confirm existing data on neurological comorbidities in ALS. Some relatively common
diseases seem to be relatively less represented in our ALS cohort (e.g. dyslipidemia) but a
possible role of these diseases as protective factors should be examined through ad hoc studies.
References
1. Turner MR, Goldacre R, Ramagopalan S, Talbot K, Goldacre MJ. Autoimmune disease preceding amyotrophic lateral
sclerosis: an epidemiologic study. Neurology. 2013;81(14):1222-5.
2. Chio A, Benzi G, Dossena M, Mutani R, Mora G. Severely increased risk of amyotrophic lateral sclerosis among Italian
professional football players. Brain 2005;128(pt 3):472–476.
3. Gallo V, Wark PA, Jenab M, et al. Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis: the EPIC
cohort. Neurology 2013;80:829–838.
4. Turner MR, Wotton C, Talbot K, Goldacre MJ. Cardiovascular fitness as a risk factor for amyotrophic lateral sclerosis:
indirect evidence from record linkage study. J Neurol Neurosurg Psychiatry 2012;83:395–398.
ERRALS Group:
Bologna: F. Salvi, I. Bartolomei, R. Michelucci; P.
Avoni, S. DePasqua, R. Liguori; A. Borghi, A.
Gabellini, T. Sacquegna; R. Rinaldi, F Cirignotta; R.
D’Alessandro; Carpi: M. Santangelo, S. Amidei, G.
Greco; Cesena: S. Biguzzi, E. Venturini, M.G.
Passarin; Faenza and Ravenna: M. Casmiro,F.
Rasi; Ferrara: E. Sette, V. Tugnoli, M.R. Tola, I.
Casetta, E. Groppo, E. Granieri; Fidenza : E.
Chierici, E. Montanari; Forlì: C. Guidi, W. Neri;
Imola: P. De Massis, V. Mussuto; Modena: J.
Mandrioli, N. Fini, E. Georgoulopoulou, P. Nichelli;
Parma: A. Grassi, L. Delay, M. Aiello, V. Pietrini;
Piacenza: E. Terlizzi, D. Guidetti; Reggio Emilia:
R. Rizzi, E. Canali, N. Marcello; Rimini: M. Currò
Dossi, M. Pasquinelli, J. Andruccioli, A. Ravasio;
Department of Hospital Services, Emilia
Romagna Regional Health Authority, Bologna: S.
Ferro
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