A Retrospective Study of the Clinical Phenotype and Predictors of Survival in non-Caucasian Hispanic Patients with Amyotrophic Lateral Sclerosis.

doi:10.21203/rs.2.11620/v1

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A Retrospective Study of the Clinical Phenotype and Predictors of Survival in non-Caucasian Hispanic Patients with Amyotrophic Lateral Sclerosis.

https://doi.org/10.21203/rs.2.11620/v1

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published 29 Oct, 2019

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Background. Little is known about the clinical behaviour of amyotrophic lateral sclerosis (ALS) in non-Caucasian populations. Here, we aimed to describe the clinical characteristics, prognostic factors and survival of Mexican patients with ALS. Methods. We conducted a retrospective study by reviewing the medical records of patients with ALS that attended and were regularly followed at a third level hospital in Mexico City from 2000 to 2015. We calculated absolute and relative frequencies of the clinical characteristics from all the participants. We also estimated correlation coefficients between continuous variables and overall survival. Additionally, survival rates were compared for all participants grouped according to different clinical features using the Kaplan-Meier method and the log-rank test. Results. We enrolled 45 ALS patients, 53.33% had spinal-onset and 46.66% presented bulbar ALS. The male/female ratio was 0.8. The mean age at onset of symptoms was 58.11 years. Mean survival time from onset was 64.73 ± 34.83 months. Cumulative survival rate after 5 years of disease onset was 44.44%. Age at onset and age at diagnosis inversely correlated with overall survival time. Also, we found that bulbar-onset, short diagnostic delay, percutaneous endoscopic gastrostomy, mechanical ventilation, and lower total cholesterol serum levels were associated with short survival. Conclusions. The clinical characteristics of Mexican ALS patients differ from the disease phenotype observed in Caucasians. Nonetheless, the predictive value of certain well-recognized clinical prognostic factors remains consistent in our population. The current study may provide relevant information for the management of ALS patients from Mexico and other Latin American countries.

Neurology

amyotrophic lateral sclerosis

motor neuron disease

spinal-onset ALS

bulbar-onset ALS

prognostic factors.

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the selective loss of motor neurons along different regions of the central nervous system. This disorder manifests in the form of progressive painless weakness of several muscle groups including those involved in respiratory movements, which invariably leads to respiratory failure and death (1). Frequency of ALS vary according to ethnicity, showing stable incidence and clinical features among Caucasians (2,3). However, little is known about statistics and clinical behaviour of ALS in other groups.

Recent findings have demonstrated that genetic risk factors associated with ALS are differentially distributed among individuals with distinct ethnic background (4). This suggests that frequency of clinical and demographic characteristics with prognostic significance of patients with ALS may also vary according to race. Thus, studies evaluating clinical characteristics and prognostic factors associated with survival in non-white Caucasian individuals with ALS are needed. For such purpose, we performed a retrospective study of the clinical features and survival of Hispanic ALS patients from Mexico City. Our results showed particularities in the phenotype of our populations of patients with ALS. Moreover, we also confirmed the well-known prognostic value of certain clinical variables previously described in other populations. Therefore, the current study may provide relevant information for the specific management of this disease among Hispanic ALS patients from Mexico and other Latin American countries.

Participants

We reviewed the medical records of all the individuals with ALS that were admitted and regularly followed at the Centro Médico Nacional “20 de Noviembre” of the Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado (ISSSTE) in Mexico City, from January of 2000 to December of 2015. We enrolled patients with probable or definitive ALS according to the “El Escorial” diagnostic criteria (5). Clinical and demographic data of all participants were retrieved from our medical records database. The obtained information included age at onset, age at diagnosis, gender, weight at onset, body weight loss after diagnosis, anatomical region of disease onset, relevant comorbidities, family history of ALS, onset to diagnosis interval (ODI), diagnosis to death interval (DDI), major medical interventions such as percutaneous endoscopic gastrostomy (PEG), mechanical ventilation (MV), onset to gastrotomy interval (OGI), gastrostomy to death interval (GDI), onset to mechanical ventilation interval (OMVI), mechanical ventilation to death interval (MVDI), time of survival from onset, cause of death, and total cholesterol serum levels at onset. The survival and other clinical data from patients that remained alive were censored at the last known follow-up.

Statistical analysis

For descriptive statistics, we calculated absolute and relative frequencies of the clinical characteristics from all the participants, overall and grouped according to the anatomical region of disease onset: spinal-onset or bulbar-onset. Continuous variables were reported as medians, means, standard deviation (SD) or 95% confidential intervals (95% CI). Categorical variables were reported as percentages. Normal distribution of the data was determined by Shapiro-Wilk test. Differences in categorical variables between spinal-onset and bulbar-onset participants were assessed by Fisher exact Xi² test, whereas comparisons of continuous variables were performed using the Student-T test or Mann-Whitney U test. To estimate how different clinical variables affected the overall survival, we first estimated correlations between continuous variables and overall survival using the Pearson or Spearman correlation coefficient. Additionally, survival rates were compared for all participants, as well as for separated spinal- and bulbar-onset groups, according to the different clinical features evaluated. For this purpose, we used the Kaplan-Meier method and the log-rank test to compare two survival curves, or the log-rank test for trend for three survival curves. Finally, to further estimate the magnitude of the association between clinical features and survival, we divided participants in two groups depending on whether their survival was found below or above the overall mean. Then, we calculated the prognostic value of each clinical factor expressed in terms of Odds ratio (OR) for poor survival using a bivariate logistic regression analysis. We also compared the means of continuous clinical variables between subjects with short and long survival by Student-T test or Mann-Whitney U test. All analyses were performed using the software package GraphPad Prism Version 8.0 (San Diego, CA, USA). A p value ˂0.05 was considered statistically significant.

Ethical statements

The current study was reviewed and approved by the Ethics committee of the Centro Médico Nacional “20 de Noviembre”, Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado (ISSSTE) in Mexico City.

Participants characteristics

We enrolled 45 ALS patients, 20 men and 25 women; the male/female ratio was 0.8. From these, 53.33% had spinal-onset whereas 46.66% presented bulbar ALS. Their mean age at onset of symptoms was 58.11 years. Thirteen patients had other relevant comorbidities, among which systemic arterial hypertension (SAH) and ischemic cardiopathy were the most frequent. Only 2 participants had family history of ALS. All the patients had at least one first-degree relative from indigenous ethnic background. Clinical and demographic characteristics of enrolled individuals are summarized in Table 1.

Survival of Patients with ALS

Mean survival time from onset of ALS patients was 64.73 ± 34.83 months, whereas mean survival time after diagnosis was 42.51 ± 28.44 months. Spinal-onset patients had a higher mean survival time compared with bulbar-onset ALS (Table 1). Respiratory failure was the most common cause of death observed in our population followed by pneumonia. Indeed, 71.11% of enrolled individuals required MV during the period of the study, whereas 68.88% were subjected to PEG. We did not find differences in the number of patients requiring such major medical interventions between spinal- and bulbar-onset groups, but the OMVI was significatively shorter in individuals with bulbar ALS (Table 1). On the other hand, we estimated cumulative survival rates of all ALS patients and stratified according to the site of disease onset. After one year of disease onset the overall survival rate was 95.55%, however, at this time point, deaths accounted only for bulbar-onset ALS cases, as 100% of spinal-onset patients remained alive (Table 2). Cumulative survival at 5 years of disease onset was 44.44% for all ALS patients, 54.16% for spinal-onset cases, and 33.33% for bulbar-onset patients.

Effect of Clinical Features on Survival of ALS Patients

Using a linear correlation analysis, we found that age at onset and age at diagnosis inversely correlated with overall survival time, whereas ODI, OGI, and OMVI showed a positive correlation with such outcome (Figure 1). We also compared survival curves of ALS patients categorized according to different clinical variables. This analysis revealed that site of disease onset significatively affected the survival, as spinal-onset ALS patients survived longer than individuals with the bulbar variant (Figure 2a, Table 3). Interestingly, we also found that survival was significatively longer for patients with delayed diagnosis compared with those with an ODI < 24 months (p < 0.0001; Figure 2f, Table 3). This could be related with a trend of older ALS patients to have a shorter ODI (Supplementary Figure 1).

PEG per se did not affect the overall survival of ALS patients, but individuals with OGI >18 months had a significant longer survival compared with those requiring an early intervention (Fig. 2g-h, Table 3). Meanwhile, as expected, MV and a short OMVI (<24 months) significatively worsened survival of ALS patients, especially in those with spinal-onset (Figure 2i-j, Table 3). In addition, we evaluated the predictive significance of total cholesterol levels at onset. Although we were able to collect this value only from 26 participants, we observed that higher levels of cholesterol at onset positively correlated with longer survival (Figure 1g). Moreover, those patients with cholesterol levels below the overall mean (< 4.63mmol/L) had a shorter survival rate (Figure 2k). Finally, to have an approximation of the magnitude of the effect of clinical characteristics on ALS prognosis, we grouped patients into two categories depending on whether their survival was found below or above the overall mean (~5 years). Then, we compared the means of continuous clinical variables between both groups and calculated the OR for short survival conferred by each categorical variable. Using this approach, we confirmed that a short ODI was associated with worse prognosis, with an OR for short survival of 14.48 (Table 4). We also found statistically significant ORs for short survival conferred by an OGI < 18 moths (OR = 11.92), necessity of MV (OR = 22.91), and lower serum levels of total cholesterol at onset (OR = 7.33).

Other variables such as gender, body weight decrease after diagnosis, and the presence of comorbidities did not affect the prognosis of ALS individuals. Nonetheless, we observed that mean survival was longer for patients ALS younger than 60 years-old compared to older individuals, although the difference did not reach statistical significance (Table 3 and Figure 2b).

Most of what it is currently known about the clinical characteristics of ALS comes from studies in white-Caucasians from Europe and North America. However, recent works have shown that epidemiology and manifestations of this disease vary in relation to ethnicity of different populations (2–4). Despite this, only few investigations have evaluated the behaviour of ALS in patients from Latin American countries (6–8). In this context, our results provide a valuable description of the neurological characteristics and survival of Hispanic Mexican ALS patients. Notably, we found certain particularities in the phenotype of our population compared with what it is described in the literature. First, the mean age at onset in the current study was lower than reported in Caucasian series from Europe (4,9–13), Japan (14), and Israel (15), but it coincides with values observed in the United States (16), Brazil (17), and Uruguay (6). Nonetheless, a previous investigation performed in the northern region of Mexico showed that the mean age at onset was around 47 years (8), much lower than observed in the current study. This discrepancy may reflect differences in the proportion of individuals with indigenous ethnic background between both regions of our country. Secondly, we also found a higher incidence of ALS in women, and an increased percentage of bulbar ALS cases, which differs from most of previous reports [4, 6–19]. Meanwhile, the proportion of familial ALS in our population was about 5%, as described in the global literature (4).

On the other hand, we addressed the effect of different clinical factors on the prognosis of ALS patients. Of note, the mean time of survival observed in our population is one the highest reported in the literature (4), together with values from another study carried out in our country (8). It is possible that genetic and environmental factors could be related with this observation, but further investigations are needed to clarify this point. Our results also showed that gender did not affected the overall survival of ALS patients, contrary to other studies showing a slightly higher mortality among women (14,17,18). Similarly, body weight reduction after diagnosis, a well-recognized independent predictor of mortality in ALS (19), had non predictive significance in our study. Regarding age and onset region, it is currently accepted that older age and bulbar onset are related to poor survival (4). In agreement with this, we found that age at onset was inversely correlated with mean time of survival from onset, whereas patients with spinal ALS had a better prognosis compared with bulbar-onset subjects. Interestingly, our data also add evidence showing that a delayed diagnosis is a protective factor for mortality in ALS cases (8,16). This may result from variations in the rate of disease progression among patients, although our results also suggest that age could have played a role in this phenomenon, since we found that individuals with higher age at disease onset had a shorter ODI. Apart from that, the time of delay in diagnosis reported here doubles the value observed in most of the studies from Europe and the United States (4), which may reflect limitations in the diagnostic approach to ALS patients in our settings. Finally, we confirmed that the need for PEG and MV during the course of the disease was a predictor of poor outcome in our patients, whereas higher serum levels of total cholesterol at onset were associated with longer survival, as previously described in European series (20).

Our study possesses all the limitations of a retrospective design in relation to the access and collection of the clinical information. In addition, the fact that the current study was conducted at a single third-level medical centre made us unable to perform a population-based comparison, which limited our ability to estimate the local incidence of ALS and may restrict the representativeness of our data. Moreover, a better description of the ethnic background of the participants would provide useful information for future investigations aimed to compare clinical factors and prognosis of ALS patients across populations with different genetic ancestry. Despite these limitations, our study provides an important description of ALS Mexican patients which may be helpful for the management of this disorder in other Hispanic populations.

In summary, the clinical characteristics and survival of Mexican-Mestizo ALS patients differ from the disease phenotype observed in individuals from other regions of the world. Nonetheless, the predictive value of certain clinical factors described in studies from Europe and the United States remains consistent in our population.

95% CI, 95% confidential intervals; ALS, amyotrophic lateral sclerosis; DDI, diagnosis to death interval; GDI, gastrostomy to death interval; ISSSTE, Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado; MV, mechanical ventilation; MVDI, mechanical ventilation to death interval; ODI, onset to diagnosis interval (ODI); OGI, onset to gastrotomy interval; OMVI, onset to mechanical ventilation interval; PEG, percutaneous endoscopic gastrostomy; SAH, systemic arterial hypertension; SD, standard deviation.

Ethics approval and consent to participate

The current study was reviewed and approved by the review board of the Centro Medico Nacional “20 de Noviembre” ISSSTE, in Mexico City. All data has been collected in accordance with local institutional review boards.

Consent for publication

The current study does not contain any individual person’s data in any form.

Availability of data and material

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

Competing interests of interest

The authors declare that they have no competing interests.

Funding

The study did not receive funding. JCP was supported by the National Council of Science and Technology of Mexico (CONACyT, CVU 737347) to achieve his Ph.D. degree.

Authors’ contributions

SMC designed the study and acquired data. CPJ designed the study, analyzed the data and drafted the manuscript. NOL, PAN, and ACL acquired data and revised the manuscript for intellectual content. GOP revised the manuscript for intellectual content. All the authors approved the final version of the manuscript.

Acknowledgements

To the medical staff of the Centro Medico Nacional “20 de Noviembre” ISSSTE in Mexico City.

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Table 1. Clinical characteristics of the study participants.
Clinical characteristics	Total participants (n = 45)	Spinal onset (n = 24)	Bulbar onset (n = 21)	p value
Age at onset (years), mean (range)	58.11 (38 – 82)	55.96 (42 – 82)	60.57 (38 – 81)	0.1566
Age at diagnosis (years), mean (range)	58.89 (40 - 83)	58.17 (43 - 83)	61.86 (40 - 81)	0.2432
Gender Males, n (%) Females, n (%)	20 (44.44) 25 (55.55)	12 (50) 12 (50)	8 (38.09) 13 (61.90)	0.4227
Weight at diagnosis (Kg), mean (SD)	50.11 (12.06)	48.61 (12.18)	51.07 (12.34)	0.6536
Family history of ALS, n (%)	2 (4.44)	1 (4.16)	1 (4.76)	0.4227
Comorbidities, n (%) SAH, n (%) Ischemic cardiopathy, n (%) Hypothyroidism, n (%) Diabetes, n (%) Cancer, n (%) Rheumatoid arthritis, n (%) Gout, n (%)	13 (28.88) 8 (17.77) 3 (6.66) 2 (4.44) 1 (2.22) 1 (2.22) 1 (2.22) 1 (2.22)	6 (25) 4 (16.66) 1 (4.16) 1 (4.16) 1 (4.16) 0 (0) 0 (0) 1 (4.16)	7 (33.33) 4 (19.04) 2 (9.52) 0 (0) 0 (0) 1 (4.76) 1 (4.76) 0 (0)	0.9230 0.5384 0.8349 0.4723 0.4723 0.2796 0.2796 0.4723
Weight loss (Kg), mean (SD)	11.79 (6.12)	10.83 (4.44)	11.76 (8.29)	0.8334
Survival time from onset (mo), mean (SD)	64.73 (34.83)	73.96 (35.60)	54.19 (31.51)	0.0565
ODI (mo), mean (SD)	22.22 (16.51)	27.50 (19.96)	16.19 (8.34)	0.0483
DDI (mo), mean (SD)	42.51 (28.44)	46.46 (30.34)	38 (26.09)	0.3252
PEG, n (%)	31 (68.88)	17 (70.83)	14 (66.66)	0.5384
OGI (mo), mean (SD)	18.61 (18.03)	20.47 (21.65)	16.36 (12.79)	0.9291
GDI (mo), mean (SD)	44.90 (28.50)	48.88 (31.74)	40.07 (24.27)	0.4009
MV, n (%)	32 (71.11)	18 (75)	14 (66.66)	0.4723
OMVI (mo), mean (SD)	26.84 (25.12)	34.94 (30.40)	16.43 (9.37)	0.0266
MVDI (mo), mean (SD)	29.69 (24.41)	28.33 (21.95)	31.43 (28.03)	0.8880
Cause of death Respiratory failure, n (%) Pneumonia, n (%) Sudden cardiac arrest, n (%) Myocardial infarction, n (%) Abdominal sepsis, n (%)	28 (62.22) 11 (24.44) 3 (6.66) 2 (4.44) 1 (2.22)	17 (70.83) 4 (16.66) 1 (4.16) 1 (4.16) 1 (4.16)	11 (52.38) 7 (33.33) 2 (9.52) 1 (4.76) 0 (0)	0.2028 0.1943 0.4723 0.4227 0.4723
Total cholesterol levels at onset (mmol/L), mean (SD)*	4.63 (1.11)	4.66 (1.29)	4.58 (0.86)	0.8588
* These data were available only for 26 participants. Values of p were estimated with the Fisher exact Xi² test and Student-T test or Mann-Whitney U test. DDI, diagnosis to death interval; Kg, kilograms; mo, months; mmol/L, millimoles per liter; MV, mechanical ventilation; ODI, onset to diagnosis interval; OGI, onset to gastrostomy interval; OMVI, onset to mechanical ventilation interval; PEG, percutaneous endoscopic gastrostomy; SD, standard deviation; SAH, systemic arterial hypertension.

Table 2. Cumulative survival rates in patients with ALS.
Years	Survival (%, 95% CI)
Years	Total participants	Spinal onset	Bulbar onset
1 year	95.55 (3.31 – 12.17)	100	90.47 (7.05 – 23.47)
2 years	88.88 (6.32 – 13.54)	100	76.19 (13.13 – 24.25)
3 years	71.11 (10.97 – 15.60)	75.00 (12.90 – 22.38)	66.66 (15.83 – 24.13)
4 years	53.33 (13.23 – 15.46)	58.33 (16.65 – 21.88)	47.61 (19.05 – 21.90)
5 years	44.44 (13.71 – 14.72)	54.16 (17.26 – 21.45)	33.33 (19.74 – 18.45)
Survival rates and their 95% CI were estimated using the log rank test for trend.

Table 3. Clinical characteristics affecting overall survival of patients with ALS.

Clinical characteristic

Total participants

(n = 45)

Survival time from onset (mo), mean (95% CI)

Log rank

p value

Spinal onset

(n = 24)

Survival time from onset (mo), mean (95% CI)

Log rank

p value

Bulbar onset

(n = 21)

Survival time from onset (mo), mean (95% CI)

Log rank

p value

Age at onset, n (%)

˂ 60 years, n (%)

˃ 60 years, n (%)

23 (51.11)

22 (48.88)

72.30 (57.84 – 86.77)

56.82 (41.20 – 72.44)

0.3841

15 (62.50)

9 (37.50)

77.33 (58.71 – 95.95)

68.33 (37.51 – 99.15)

0.8671

8 (38.09)

13 (61.90)

62.88 (35.13 – 90.62)

48.85 (30.41 – 67.28)

0.3895

Gender

Males, n (%)

Females, n (%)

20 (44.44)

25 (55.55)

65.15 (47.57 – 82.73)

64.40 (50.67 – 78.13)

0.4147

12 (50)

78.17 (53.11 – 103.20)

69.75 (49.10 – 90.40)

0.1925

8 (38.09)

13 (61.90)

45.63 (24.14 – 67.11)

59.46 (38.61 – 80.31)

0.2401

Comorbidities

Yes, n (%)

No, n (%)

13 (28.88)

32 (71.11)

54.08 (31.67 – 76.48)

69.06 (56.98 – 81.14)

0.6441

6 (25)

18 (75)

64.00 (13.37– 114.6)

77.28 (61.69 – 92.87)

0.7702

7 (33.33)

14 (66.66)

45.57 (22.43 – 68.71)

58.50 (38.68 – 78.32)

0.2386

Weight loss

˂10 kg, n (%)

˃10 kg, n (%)

18 (40)

27 (60)

65.33 (45.89 – 84.78)

64.33 (51.50 – 77.17)

0.6698

9 (37.5)

15 (62.5)

70.44 (38.73 – 102.20)

76.07 (57.73 – 94.41)

0.8376

9 (42.85)

12 (57.14)

60.22 (30.57 – 89.88)

49.67 (33.21 – 66.12)

0.2239

ODI (mo)

˂24 mo, n (%)

˃24 mo, n (%)

26 (57.77)

19(42.22)

43.58 (34.03 – 53.12)

93.98 (81.31 – 106.10)

<0.0001

11 (45.83)

13 (54.16)

48.64 (30.99 – 66.28)

95.38 (78.63 – 112.1)

0.0010

15 (71.42)

6 (28.57)

39.87 (27.87 – 51.86)

90.00 (66.44 – 113.60)

0.0007

PEG

Yes, n (%)

No, n (%)

31 (68.88)

14 (31.11)

63.52 (50.63 – 76.40)

67.43 (47.04 – 87.82)

0.7315

17 (79.83)

7 (29.16)

69.35 (51.33 – 87.38)

85.14 (50.87 – 119.40)

0.4801

14 (66.66)

7 (33.33)

56.43 (36.13 – 76.73)

49.71 (27.13 – 72.30)

0.3952

OGI (mo)

˂18 mo, n (%)

˃18 mo, n (%)

(n =31)

16 (51.61)

15 (48.38)

42.38 (29.69 – 55.06)

86.07 (68.72 – 103.40)

0.0002

(n =17)

9 (52.94)

8 (47.05)

53.78 (35.69 – 71.86)

86.88 (54.34 – 119.40)

0.0286

(n = 14)

7 (50)

27.71 (13.60 – 41.83)

85.14 (64.03 – 106.30)

0.0006

Yes, n (%)

No, n (%)

32 (71.11)

13 (28.88)

56.53 (44.75 – 68.32)

84.92 (65.18 – 104.7)

0.0256

18 (75)

6 (25)

63.28 (46.74 – 79.81)

106.0 (84.31 – 127.70)

0.0273

14 (66.66)

7 (33.33)

47.86 (29.99 – 65.73)

66.86 (38.30 – 95.41)

0.2478

OMVI (mo)

˂24 mo, n (%)

˃24 mo, n (%)

(n = 32)

16 (50)

45.06 (28.34 – 61.79)

68.00 (51.64 – 84.36)

0.0283

(n = 18)

8 (44.44)

10 (55.55)

50.88 (28.31 – 73.44)

73.20 (47.65 – 98.75)

0.0953

(n = 14)

8 (57.14)

6 (42.85)

39.25 (9.04 – 69.46)

59.33 (38.70 – 79.97)

0.3413

Total cholesterol at onset

˂ 4.63 mmol/L, n (%)

˃4.63 mmol/L, n (%)

(n = 26)

14 (53.84)

12 (46.15)

46.64 (31.14 – 62.15)

77.33 (52.28 – 102.40)

0.0271

(n = 15)

8 (53.33)

7 (46.66)

55.88 (30.23 – 81.52)

91.43 (53.86 – 129.0)

0.0496

(n = 11)

6 (54.54)

5 (45.45)

34.44 (18.11 – 50.56)

57.60 (19.03 – 96.17)

0.0860

Kg, kilograms; mo, months; mmol/L, millimoles per liter; MV, mechanical ventilation; ODI, onset to diagnosis interval; OGI, onset to gastrostomy interval; OMVI, onset to mechanical ventilation interval; PEG, percutaneous endoscopic gastrostomy; 95% CI, 95% confidence interval.

Table 4. Clinical features of ALS patients with short and long survival.

Characteristic

Short survival

(n = 22)

Long survival

(n = 23)

OR (95% CI)

Age at onset (years), mean (range)

61.50 (42 - 82)

54.87 (38 – 78)

Age group

˂ 60 years, n (%)

˃ 60 years, n (%)

11 (50)

12 (52.17)

11 (47.82)

0.91 (0.30 – 2.79)

Gender

Males, n (%)

Females, n (%)

12 (54.54)

10 (45.45)

8 (34.78)

15 (65.21)

2.25 (0.63 – 7.45)

Weight at diagnosis (Kg), mean (SD)

52.14 (11.61)

49.22 (12.52)

Weight loss (Kg), mean (SD)

˂10 kg, n (%)

˃10 kg, n (%)

10.77 (5.30)

9 (40.90)

13 (59.09)

12.86 (6.83)

9 (39.13)

14 (60.86)

1.07 (0.35 – 3.32)

Comorbidities

Yes, n (%)

No, n (%)

8 (36.36)

14 (63.63)

5 (21.73)

18 (78.26)

0.48 (0.13 – 1.80)

ODI (mo), mean (SD)

˂24 mo, n (%)

˃24 mo, n (%)

14.27 (8.44)

19 (86.36)

3 (13.63)

*** 29.83 (18.80)

7 (30.43)

16 (69.56)

14.48 (3.38 – 53.99)

PEG, n (%)

17 (77.27)

14 (60.86)

2.18 (0.62 – 7.65)

OGI (mo), mean (SD)

˂18 mo, n (%)

˃18 mo, n (%)

10.94 (8.43)

13 (76.47)

4 (23.52)

**27.93 (22.20)

3 (21.42)

11 (78.57)

11.92 (2.27 – 50.84)

MV, n (%)

21 (95.45)

11 (47.82)

22.91 (2.88 – 254.80)

OMVI (mo), mean (SD)

˂24 mo, n (%)

˃24 mo, n (%)

17.33 (7.73)

12 (57.14)

9 (42.85)

*45.00 (20.93)

4 (36.36)

7 (63.63)

2.33 (0.58 – 8.66)

Total cholesterol at onset (mmol/L)

˂ 4.63 mmol/L, n (%)

˃4.63 mmol/L, n (%)

4.13 (0.72)

11 (73.33)

4 (26.66)

**5.31 (1.20)

3 (27.27)

8 (72.72)

7.33 (1.29 – 33.10)

* p<0.05 ** p<0.01, ***p<0.001. Differences between means of continuous variables were estimated by Student-T test or Mann-Whitney U test. OR values were calculated by bivariate logistic regression analysis. Kg, kilograms; mo, months; mmol/L, millimoles per liter; NA, not applicable; OR, Odds ratio; PEG, percutaneous endoscopic gastrostomy; SD, standard deviation; 95% CI, 95% confidence interval.

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A Retrospective Study of the Clinical Phenotype and Predictors of Survival in non-Caucasian Hispanic Patients with Amyotrophic Lateral Sclerosis.

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