Efficacy of neuromuscular electrical stimulation on Wilson’s disease patients          with dysphagia

Li, Xuewei; Li, Liang-Yong

doi:10.1589/jpts.31.971

Cited by 3 publications

(3 citation statements)

References 18 publications

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“…Meanwhile, aspiration pneumonia and scoliosis are possible complications in patients with neurological signs. 26,27 Other complications include osteoporosis, susceptibility to fractures, and osteoarthritis. [28][29][30] These complications may be related to copper accumulation without adequate treatment.…”

Section: Discussionmentioning

confidence: 99%

“…These complications can be detected or managed through immediate follow‐up consultation and may include decrease in white blood cell and platelet counts 24,25 and esophageal varices, which are a typical complication in patients with WD and splenomegaly. Meanwhile, aspiration pneumonia and scoliosis are possible complications in patients with neurological signs 26,27 . Other complications include osteoporosis, susceptibility to fractures, and osteoarthritis 28–30 .…”

Section: Discussionmentioning

confidence: 99%

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Factors affecting activities of daily living among patients with Wilson disease

Amemiya

Asakura

Nishiwaki

et al. 2023

J of Inher Metab Disea

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Wilson disease (WD) is a congenital copper metabolism disorder with various manifestations and can be treated with oral medication. This study examined the factors related to decline in activities of daily living (ADL) in patients with WD as research in this area remains limited. We enrolled 308 patients with WD, including patients who participated in a national survey and those who sought cares at the Department of Pediatrics, Toho University Ohashi Medical Center, from 2016 to 2017. We analyzed the association between ADL decline and factors including age at diagnosis, period from diagnosis to survey, hepatic symptoms, neurological signs, and psychiatric presentation at diagnosis. The relative risks (RRs) for ADL decline were estimated for each factor using multivariate modified Poisson regression analysis. Overall, 97 out of 308 (31.5%) patients experienced ADL decline. After adjusting for explanatory variables, regression analysis revealed that factors significantly associated with ADL decline were a period of ≥20 years from diagnosis to survey (adjusted RR = 2.34, 95% confidence interval [CI]: 1.47–3.74), hepatic symptoms with splenomegaly (adjusted RR = 2.57, 95% CI: 1.26–5.24), mild neurological signs (adjusted RR = 3.20, 95% CI: 1.96–5.23), and severe neurological signs (adjusted RR = 3.63, 95% CI: 2.28–5.77). Neurological signs, hepatic symptoms with splenomegaly, and a period of 20 years from diagnosis to survey are associated with ADL decline. Thus, careful assessment of patients for these factors is necessary, and these findings may guide future efforts to improve patient prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Factors affecting activities of daily living among patients with Wilson disease

Amemiya

Asakura

Nishiwaki

et al. 2023

J of Inher Metab Disea

View full text Add to dashboard Cite

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“…The higher the number, the higher the severity. 0, no measured stroke symptoms; 1-4, minor stroke; 5-15, moderate stroke; 16-20, moderate-to-severe stroke; and 21-42, severe stroke; modified Rankin Scale [10] : a disability classification model readjusted from the original Rankin scale score, including 7 separate categories from grades 0-6, 0 (no symptoms), 1 (no significant disability), 2 (slight disability), 3 (moderate disability), 4 (moderately severe disability), 5 (severe disability), and 6 (expired); water swallow test [11] : an experimental method for evaluating swallowing ability, which is divided into grades 0-5. The patient sat upright and drank 30 mL of warm water to observe the required time and choking; venous thromboembolism (VTE) assessment [12] : a well-established model for assessing VTE risk by calculating independent individual risk factors, which incorporates multiple risk factors for VTE and stratifies patients for prevention by calculating a total score.…”

Section: Clinical Evaluationmentioning

confidence: 99%

Long-term prognostic significance of sarcopenia in acute ischemic stroke

Hou

Liu

2022

Medicine

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Because sarcopenia is widely distributed in patients with acute ischemic stroke (AIS) and has not attracted enough attention, this study aims to explore the relationship between sarcopenia defined by temporal muscle thickness (TMT) and physical function and prognosis of patients with AIS. A total of 265 hospitalized nonsurgical AIS patients from 2015 to 2018, with an age range of 28 ~ 92, were analyzed retrospectively. The median value of TMT was used as the risk classification index of sarcopenia. The main results were the relationship between sarcopenia and Essen Stroke Risk Score, National Institutes of Health Stroke Scale, modified Rankin Score, water swallow test, venous thromboembolism assessment of medical inpatients, activities of daily living assessed by Barthel Index, and the relationship between TMT and final survival outcome. The mean TMT of men in the study cohort was higher than that of women. The measured values of TMT among different researchers had good consistency (intraclass correlation coefficient, 0.980; P < .001). After adjusting for confounding variables, logistic regression showed that sarcopenia was associated with Essen Stroke Risk Score (odds ratio, 1.89; P < .05) and Barthel Index (odds ratio, 1.67; P < .05). Kaplan-Meier analysis showed that the survival time of low TMT group was significantly lower than that of high TMT group (36 vs 49 months; P < .001). Multivariate Cox regression showed that there was causal correlation between sarcopenia and patient death (hazard ratio, 3.54; 95% confidence interval, 1.46–8.58; P < .01). As a potential comprehensive index, thickness of temporal muscle can be included in baseline evaluation to show the physical status, stroke recurrence, and survival prognosis of AIS patients.

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Dysphagia in Wilson’s Disease: A Case Report of One-Year Follow-Up

Kim

Park

et al. 2021

Journal of the Korean Dysphagia Society

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Wilson's disease (WD) is a genetic disease caused by an ATP7B gene mutation. Although dysphagia is known as a neurological manifestation of WD, clinical case reports with post-treatment long-term follow-up are scarce. A 17year-old male was admitted to hospital complaining of general weakness and swallowing difficulty. He was diagnosed with WD by genetic confirmation. Assessment of the videofluoroscopic swallowing study (VFSS) determined an initial videofluoroscopic dysphagia scale (VDS) score of 48. After 11 months treatment with D-penicillamine and neuromuscular electrical stimulation therapy (NMES) with oromotor exercises, the VDS score improved to 23, especially in the following areas: mastication, apraxia, premature bolus loss, triggering of pharyngeal swallow, and laryngeal elevation. Dysphagia is an early neurological symptom of WD, and is reversible when properly treated with early diagnosis. Thus, since WD is treatable, patients presenting with dysphagia should be indicative of high probability of the disease, and evaluated at the earliest. NMES treatment in combination with D-penicillamine helps to improve the deglutition function in both oral and pharyngeal phases. To investigate the characteristics of dysphagia and the specific clinical efficacy of NMES in WD, further studies with larger number of patients are required. (JKDS 2021;11:82-86

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Efficacy of neuromuscular electrical stimulation on Wilson’s disease patients with dysphagia

Abstract: Purpose] Dysphagia is a neurological symptom that is observed in more than half of patients with Wilson's disease. It is often associated with aspiration pneumonia, dehydration, and malnutrition, resulting in drastic reduction of the quality of life.

Cited by 3 publications

References 18 publications

Factors affecting activities of daily living among patients with Wilson disease

Factors affecting activities of daily living among patients with Wilson disease

Long-term prognostic significance of sarcopenia in acute ischemic stroke

Dysphagia in Wilson’s Disease: A Case Report of One-Year Follow-Up

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