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Adaptation to Turkish and Reliability Study of the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R).

INTRODUCTION

Recent developments in neurological sciences have made us comprehensively think about clinically well-defined motor neuron diseases (MNDs) with unclear etiopathogenesis and treatment. Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by the involvement of the lower and upper motor neurons and has a degenerative and progressive course that has been tried to be explained by many causes (1). It is characterized by the involvement of motor neurons in the motor cortex, brain stem, and spinal cord (2,3).

The initial symptoms of the disease are generally asymmetrical weakness and atrophy, which may be localized to a certain part of an extremity and usually begin in the distal regions. These findings usually begin in the upper extremity in 30%-50% of cases, lower extremity in 20%-40% of cases, and bulbar muscles in 20%-30% of cases (4). Initial symptoms may include cramps, fasciculations, and feeling of muscle stiffness. Fasciculation first involves the hand and arm muscles. Other important findings of the disease are spasticity pathological reflexes, and some pyramidal signs such as clonus (5,6,7). In this neurodegenerative disease, the main diagnostic basis is clinical features and electromyography (EMG) findings.

The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) is used in various countries in the evaluation and follow-up of patients with ALS, and provides predictive information to physicians regarding the functional deterioration of patients (8,9,10). In the present study, we aimed to make a Turkish translation of ALSFRS-R, which has been previously translated to English, Spanish, Japanese, and Korean, and to test its reliability and validity in the Turkish language and make it available for Turkish patients with ALS (11,12,13).

METHODS

After approval from the Cukurova University Faculty of Medicine Ethics Committee (dated 09.01.2007-Meeting no:l-Decision no:14), a total of 41 patients (24 males, 58.53%; 17 females, 42.47%) who had been diagnosed with ALS according to modified El Escorial diagnostic criteria and who were seen at any stage of the disease were enrolled in the study. The mean age of the patients was 56.3+12.9 years (23-81).

First, ALSFRS-R was translated to Turkish independently by 3 teaching fellows who knew English very well. The resultant three different texts were transformed to a common text by two neurologists who knew English very well. This common text was again translated to English by two teaching fellows who were good at English. This resultant Turkish scale was applied to a group of 10 patients with ALS and then tested for its comprehensibility. This resultant Turkish version was applied to the test cases by two physicians (a 2nd year assistant doctor in the department of neurology and a teaching fellow experienced in neuromuscular diseases) in the same session but independently (Table 1).

ALSFRS-R consists of 12 parameters, such as speaking, saliva, swallowing, hand writing, eating, dressing and self-care, turning in the bed and covering up, walking, climbing stairs, dyspnea, orthopnea, and respiratory insufficiency with a maximum score of 48. Each question is scored between 0 and 4 points. Patients with normal functions are given 4 points. Scores drop down from 4 with increasing functional deterioration, with the worst functional status being scored as 0 points.

Statistical Analysis

In statistical evaluation, the scoring results performed by two physicians were summarized with descriptive statistics in terms of both sub-headings and global scores. The average difference between the evaluations of the two physicians and the 95% confidence intervals of this average were found. Following this, a reliability evaluation was performed by calculating the correlation (Pearson) coefficients for the inter-physician scorings. In the case of a coefficient higher than 0.80 and statistical significance, 'consistence' was accepted to be present. The Statistical Package for the Social Sciences packaged software version 19 (SPSS Inc; Chicago, IL, USA) was used in statistical analyses, and the level of critical significance was accepted as 0.05.

RESULTS

Averages of the evaluations made by the two physicians for the sub-headings are shown in Table 2. Score differences for the sub-headings between the two evaluators who applied the scale varied between -0.14 and 0.62. The difference was found to be 2.14 for the total score. It could be noted that there was generally a high level of correlation between the two evaluators with regard to the 12 sub-headings and total scores.

Among the sub-headings, only saliva, swallowing, eating (with gastrostomy), and dyspnea evaluations were found to be significantly different.

The correlation coefficient score (by the Pearson's test) for the total scores of the two evaluators was calculated as r=0.972, with p=0.000. Correlations in the sub-headings were generally higher than 0.80 (0.689 for respiration), and all coefficients were found to be significant (Table 3).

DISCUSSION

The clinical findings of ALS are related to the loss of neurons located in all levels of the motor system, from the cortex to the anterior horn of the spinal cord. The disease may begin as a spinal or bulbar form. In the bulbar form, the clinical signs may initially appear also as dysarthria, dysphagia, or as both, and may present with lower motor neuron findings (bulbar palsy), upper motor neuron findings (pseudobulbar palsy), or both. Bulbar palsy is characterized by tongue weakness, fasciculation, and a decrease in the blink reflex, while pseudobulbar palsy is characterized by emotional lability, which involves pathological laughing and crying, active jaw reflex, and dysarthria.

When symptoms begin in the upper extremity, the disease characterized by a difficulty in carrying out activities requiring grasping and fine skills distally and in carrying out shoulder-abduction-related activities (e.g., hair washing) proximally In the case of initiation in the lower extremity, the disease presents with difficulty in foot dorsiflexion (foot drop) and in climbing stairs (proximal weakness) (14).

Around 5%-10% of cases have a genetic origin, while 90%-95% occur sporadically. The genetic form mostly occurs in an autosomal dominant pattern, while the autosomal recessive form is commonly seen in Northern Africa societies in which consanguineous marriage is common (15). In 10%-20% of patients with the autosomal dominant inheritance form, there is a copper/zinc superoxide dismutase mutation localized on chromosome 21. The incidence of sporadic ALS is 1.5-2/100000 and its prevalence is 6/100000. The disease is more common in males than in females (1.6:1). As it is known, ALS has the same prevalence in all parts of the world, except for in some particular geographical regions, such as the Western Pacific Islands and the Kii Peninsula (16). In total, 50% of ALS patients die within three years after the appearance of initial signs.

Diagnosis is based on history neurological examination, electrophysiological studies, and modified El Escorial diagnostic criteria. El Escorial diagnostic criteria were proposed at the end of the 1980s and were later modified (17,18). In our study patients who had been diagnosed with ALS according to modified El Escorial criteria were enrolled.

There are some different ways to determine the severity of the symptoms and signs of ALS and its course, such as the Medical Research Council (MRC) scale evaluating muscle strength, Tufs quantitative neuromuscular examination, the Norris and Appel scale, and ALSFRS-R (19,20,21). ALSFRS-R is one of the most commonly used scales in clinical studies to assess the neurological and functional status of patients with ALS. Reliability studies of the scale have been performed in many languages, such as Japanese, Korean, and Spanish. The results of these reliability studies have shown that ALSFRS-R is a quite useful test to show the functional status of the patients, for each of the various languages it has been translated into (11,12,13). In a Korean language reliability study 51 patients who had been diagnosed with ALS according to El Escorial criteria were enrolled. Besides ALSFRS-R being translated to the Korean language, these subjects were also applied the Norris scale and Appel ALS status scale tests. The tests were applied twice with 7 days intervals, and it was shown that there was a statistically significant correlation between the Norris scale, the Appel ALS status scale, and the results of ALSFRS-R. From this point of view, it was reported that ALSFRS-R was a useful and reliable scale in the evaluation of the functional status of the tested Korean patients with ALS (13).

In another study conducted in Spain, ALSFRS-R was translated to the Spanish language. Four evaluators applied the Spanish version of ALSFRS-R, the Amyotrophic Lateral Sclerosis Assessment Questionnaire including 40 questions (ALSAQ-40), and the SRI scale evaluating respiratory status to 73 Spanish patients with ALS. It was reported that the scores of the Spanish version of ALSFRS-R were correlated to the scores obtained from ALSAQ-40 and the SRI scale evaluating respiratory status, indicating that ALSFRS-R could be safely used in ALS patients who speak Spanish (11). In an ALSFRS-R scale study by Ohashi et al. (12), in which the scale was translated to the Japanese language and applied to 27 Japanese patients, similar results were obtained. The number of patients enrolled in our present study was almost the same as the patient numbers of the Korean, Spanish, and Japanese studies. Results obtained with the use of the scales in our language and in the other three languages mentioned here overlap, and it is remarkable that the total scores obtained with ALSFRS-R and the findings of the sub-headings, especially the findings of the bulbar symptoms, were good indicators of disease survival (22,23). In addition, when twelve sub-headings of ALSFRS-R were evaluated individually, a high level consistency was detected between two physicians, in terms of both the total scores and sub-headings, which demonstrates that ALSFRS-R is a reliable and easy-to-apply test for daily practice. Although there was some inconsistency between the two appliers, the bulbar findings were found to be higher when compared to the other sub-headings, and the obtained findings were accepted to be statistically significant. The total score data were also found to be statistically significant (p=0.00).

In conclusion, we aimed to make a Turkish version study of this scale, which was developed to determine the functional status of Turkish patients with ALS and disease prognosis. In conclusion, considering this reliability study a more extensive use of ALSFRS-R may make a significant contribution to developing a common language for the follow-up of the functional status of patients. In addition, we suggest that determining the functional deterioration of patients with this scale will enable patients and their relatives to get ready for disease progression (making the home environment suitable, providing psychiatric support, preparations for gastrostomy, etc.).

Ethics Committee Approval: Ethics committee approval was received for this study from the ethics committee of Cukurova University Faculty of Medicine Ethics Committee (dated 09.01.2007-Meeting no:l-Decision no:14).

Informed Consent: Written informed consent was obtained from patients who participated in this study.

Author Contributions: Concept--FK., M.B.; Design--FK., M.B.; Supervision--FK., YS.; Resources--FK., M.B.; Materials--FK., M.B.; Data Collection and/or Processing--F.K., M.B.; Analysis and/or Interpretation --FK., M.B., TD., Z.N.A., YS.; Literature Search--FK., M.B., TD.; Writing Manuscript--FK., M.B.; Critical Review--FK., Z.N.A., M.B.; Other--FK., M.B., T.D., Z.N.A., Y.S.

Conflict of Interest: No conflict of interest was declared by the authors.

Financial Disclosure: The authors declared that this study has received no financial support.

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Filiz KOC (1) Mehmet BALAL (1), Turgay DEMiR (1), Z. Nazan ALPARSLAN (2), Yakup SARICA (1)

(1) Department of Neurology, Cukurova University School of Medicine, Adana, Turkey

(2) Department of Medical Biostatistics, Cukurova University School of Medicine, Adana, Turkey

Correspondence Address: Mehmet Balal, Cukurova Universitesi Tip Fakultesi, Noroloji Anabilim Dali, Adana, Turkiye

E-mail: memet_balal@hotmail.com

Received: 12.04.2015 Accepted: 08.09.2015

DOI: 10.5152/npa.2016.11334
Table 1. Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS)

The evaluated finding                 Score

1- Speech
   Normal speech processes              4
   Detectable speech disturbance        3
   Intelligible with repeating          2
   Speech combined with                 1
   nonvocal communication
   Loss of useful speech                0
2- Salivation
   Normal                               4
   Slight but definite                  3
   excess of saliva in mouth;
   may have nighttime drooling
   Moderately excessive saliva;         2
   may have minimal drooling
   Marked excess of                     1
   saliva with some drooling
   Marked drooling requires             0
   constant tissue or handkerchief
3- Swallowing
   Normal                               4
   Early eating problems,               3
   occasional choking
   Dietary consistency changes          2
   Needs supplemental                   1
   tube feeding
   Exclusively parenteral or            0
   enteral feeding
4- Handwriting
   Normal                               4
   Slow or sloppy;                      3
   all words are legible
   Not all words are                    2
   legible
   Able to grip pen                     1
   but unable to write
   Unable to grip pen                   0
5A-Cutting food
   without gastrostomy
   Normal                               4
   Somewhat slow and                    3
   clumsy, but no help needed
   Can cut most foods,                  2
   although clumsy and
   slow; some help needed
   Food must be cut by                  1
   someone but can
   still feed slowly
   Needs to be fed                      0
5B-Cutting food with gastrostomy
   Normal                               4
   Clumsy but able to                   3
   perform all manipulations
   independently
   Some help needed                     2
   with closures and fasteners
   Provides minimal                     1
   assistance to caregiver
   Unable to perform                    0
   any aspect of task
6- Dressing and hygiene
   Normal                               4
   Independent and complete             3
   self-care with effort or
   decreased efficiency
   Intermittent assistance or           2
   substitute methods
   Needs attendant for self-care        1
   Total dependence                     0
7- Turning in bed
   Normal                               4
   Somewhat slow and                    3
   clumsy, but no
   help needed
   Can turn alone or adjust             2
   sheets, but with
   great difficulty
   Can initiate, but not                1
   turn or adjust sheets
   alone
   Helpless                             0
8- Walking
   Normal                               4
   Early ambulation difficulties        3
   Walks with assistance                2
   Nonambulatory functional             1
   movement only
   No purposeful leg movement           0
9- Climbing stairs
   Normal                               4
   Slow                                 3
   Mild unsteadiness or fatigue         2
   Needs assistance                     1
   Cannot do                            0
10-Dsypnea
   None                                 4
   Occurs when walking                  3
   Occurs with one or                   2
   more of the following: eating,
   bathing, dressing
   Occurs at rest, difficulty           1
   in breathing when either
   sitting or lying
   Significant difficulty,              0
   considering using mechanical
   respiratory support
11-Orthopnea
   None                                 4
   Some difficulty in                   3
   sleeping at night due
   to shortness
   of breath. Does not
   routinely use more
   than two pillows
   Needs extra pillow                   2
   in order to sleep (more than two)
   Can only sleep sitting up            1
   Unable to sleep                      0
12-Respiratory insufficiency
   None                                 4
   Intermittent use of BiPAP            3
   Continuous use of                    2
   BiPAP during the night
   Continuous use of                    1
   BiPAP during the night and day
   Invasive mechanical                  0
   ventilation by intubation
   or tracheostomy
   Total score                        /48

Table 2. Evaluating the scores and differences between two specialists

                        First specialist               Second specialist

                  Median  Standard   Minimum  Maximum  Median  Standard
                  score   deviation                    score   deviation

Speech            2.67      1.16       0        4        2.81    1.32
Salivation        3.24      1.22       0        4        2.90    1.33
Swallowing        2.81      1.36       0        4        2.52    1.43
Handwriting       2.05      1.56       0        4        2.14    1.65
Gastrostomy       2.57      1.83       0        4        1.95    1.74
Dressing          1.57      1.50       0        4        1.48    1.53
Lying             1.95      1.62       0        4        1.76    1.64
Walking           2.52      1.43       0        4        2.24    1.41
Climbing stairs   1.62      1.65       0        4        1.48    1.60
Dsypnea           3.05      1.24       1        4        2.86    1.23
Orthopnea         3.24      1.13       1        4        3.05    1.07
Respiration       3.95      0.21       3        4        3.90    0.30
Score            31.23     11.30      14       48       29.09   12.02

                  Second specialist                  Difference

                 Minimum   Maximum   Median   Confidence            t
                                              interval
                                               Lower       Upper

Speech             0         4        -0.14    -0.44       0.16   -1.000
Salivation         0         4         0.33     0.00       0.67    2.092
Swallowing         0         4         0.29     0.03       0.54    2.335
Handwriting        0         4        -0.10    -0.29       0.10   -1.000
Gastrostomy        0         4         0.62     0.11       1.13    2.540
Dressing           0         4         0.10    -0.22       0.41    0.623
Lying              0         4         0.19    -0.18       0.56    1.073
Walking            0         4         0.29    -0.14       0.6l    1.826
Climbing stairs    0         4         0.14    -0.16       0.44    1.000
Dsypnea            1         4         0.19     0.01       0.37    2.169
Orthopnea          1         4         0.19    -0.04       0.42    1.706
Respiration        3         4         0.05    -0.05       0.15    1.000
Score             10        48         2.14     0.83       3.44    3.423

                 Difference

                   Sig.

Speech            0.329
Salivation        0.049
Swallowing        0.030
Handwriting       0.329
Gastrostomy       0.020
Dressing          0.540
Lying             0.296
Walking           0.183
Climbing stairs   0.329
Dsypnea           0.042
Orthopnea         0.104
Respiration       0.329
Score             0.003

Table 3. Sub-headings and total correlation coefficients

                  n    Compatibility     p
Speech            41      0.870        0.000
Salivation        41      0.841        0.000
Swallowing        41      0.921        0.000
Handwriting       41      0.965        0.000
Gastrostomy       41      0.806        0.000
Dressing          41      0.894        0.000
Lying             41      0.876        0.000
Walking           41      0.873        0.000
Climbing stairs   41      0.920        0.000
Dsypnea           41      0.947        0.000
Orthopnea         41      0.894        0.000
Respiration       41      0.689        0.00l
Score             41      0.972        0.000
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Title Annotation:Research Article
Author:Koc, Filiz; Balal, Mehmet; Demir, Turgay; Alparslan, Z. Nazan; Sarica, Yakup
Publication:Archives of Neuropsychiatry
Date:Sep 1, 2016
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