Longitudinal analysis of progression of dysphagia in amyotrophic lateral sclerosis
Introduction
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that involves, primarily, motor neurons in the cerebral cortex, brainstem, and spinal cord [1]. ALS patients show wide-ranging clinical features, since the disease affects bulbar and/or spinal systems according to the site of motor neuron involvement. As the disease progresses, both upper motor neurons (UMN) and lower motor neurons (LMN) may be affected, and various symptoms overlap [2].
Among various clinical features, bulbar impairment, such as dysarthria and/or dysphagia, generally occurs late in the course of the disease, but it is present at the onset in about one-third of cases [3]. Dysphagia is one of the most critical problems for the ALS patient. ALS patients with swallowing disturbance not only suffer from dehydration and malnutrition but also face aspiration, which may result in aspiration pneumonia and can lead to death. As such, swallowing function in ALS patients should be carefully and appropriately evaluated and followed up during the course of disease progression.
Videofluoroscopy (VF) has so far been the most reliable examination to evaluate swallowing function in ALS patients [4], and many videofluoroscopic studies have been reported [4], [5], [6], [7], [8], [9], [10]. These reports well described the general patterns of swallowing disturbance in ALS, but they did not assess the progression of dysphagia in ALS patients, and it remains unclear how these dysfunctions change over time. Hillel and Miller found the rate of the progression of symptoms to be extremely variable [3]. The rate of progression of bulbar symptoms also varies [2]. Little research has been done to examine the progression of swallowing problems in ALS patients [2], and in particular, no study has used such an objective examination as VF to evaluate the changes of swallowing function in ALS. This report is the first detailed analysis of the progression of dysphagia in ALS patients, by VF examination, seeking to contribute to elucidation of this intractable problem in ALS.
Section snippets
Patients selection
Fifty patients, diagnosed as having ALS at the Department of Neurology in our institute, were referred to our swallowing and speech clinic, for evaluation of swallowing function. According to the El Escorial criteria [1], the following features were evaluated for each patient: (1) signs of LMN degeneration by clinical, electrophysiological, or neuropathologic examination, (2) signs of UMN degeneration by clinical examination, and (3) progressive spread of signs within a region or to other
Demographics of the patients
The total of 72 ALS patients on VF examinations included 41 males (age range, 41–89 years; average, 62.2) and 31 females (age range, 35–84 years; average, 62.5). They were divided into six groups according to the duration following the onset of bulbar symptoms (Table 1): no bulbar complaints (Group 1), 1–6 months following bulbar symptom onset (Group 2); and 7–12 months (Group 3), 13–18 months (Group 4), 19–24 months (Group 5), and more than 24 months (Group 6) following bulbar symptom onset.
Discussion
Progression of dysphagia in ALS has not yet been well studied. This is partly because the rate of progression of symptoms is extremely variable [2], [3]. However, while the patterns of progression of spinal ALS are unpredictable, bulbar ALS most often follows a progressive, recognizable course [3]. In addition, clinically, ALS patients with bulbar symptoms often follow a rapidly progressive course. As such, in this study, we aimed to elucidate the patterns of progression of dysphagia in ALS
Conclusion
We analyzed the progression of dysphagia in ALS patients using an objective evaluation method, videofluoroscopy. VF is a useful tool to follow swallowing function in ALS patients. In early-stage dysphagia in ALS, VF will contribute to rehabilitation or introducing an appropriate form of diet; it will be useful to perform VF by 6 months following bulbar symptom onset. Later, around 1 year following bulbar symptom onset, VF should also be performed, since ALS patients show wide-ranging nutrition
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