Adaptation to Turkish and Reliability Study of the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R).
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).
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.
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.
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).
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
Received: 12.04.2015 Accepted: 08.09.2015
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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