지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
이용수
초록· 키워드
오류제보하기
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 17- year-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.
목차
등록된 정보가 없습니다.
참고문헌 (10)
참고문헌 신청
Seon Yeong Lee / 2012 / Neuromuscular Electrical Stimulation Therapy for Dysphagia Caused by Wilson’s Disease / Annals of Rehabilitation Medicine 36(3):409~413
Li X / 2019 / Efficacy of neuromuscular electrical stimulation in Wilson’s disease patients with dysphagia / J Phys Ther Sci 31:971~974
Jean A / 2001 / Brain stem control of swallowing : neuronal network and cellular mechanisms / Physiol Rev 81:929~969
Jean A / 1979 / Inputs to the swallowing medullary neurons from the peripheral afferent fibers and the swallowing cortical area / Brain Res 178:567~572
Hamdy S / 1998 / Long-term reorganization of human motor cortex driven by short-term sensory stimulation / Nat Neurosci 1:64~68
Li X / 2019 / Efficacy of neuromuscular electrical stimulation in Wilson’s disease patients with dysphagia / J Phys Ther Sci 31:971~974
Jean A / 2001 / Brain stem control of swallowing : neuronal network and cellular mechanisms / Physiol Rev 81:929~969
Jean A / 1979 / Inputs to the swallowing medullary neurons from the peripheral afferent fibers and the swallowing cortical area / Brain Res 178:567~572
Hamdy S / 1998 / Long-term reorganization of human motor cortex driven by short-term sensory stimulation / Nat Neurosci 1:64~68
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