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Breathlessness in amyotrophic lateral sclerosis: A case report on the role of osteoporosis in the worsening of respiratory failure

机译:肌萎缩性侧索硬化症的呼吸困难:骨质疏松在呼吸衰竭加重中的作用的一例报告

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Rationale: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative multisystem disorder, presenting with limb or bulbar onset. To date, there is no cure for ALS. At some stage of the disease, patients may complain of breathlessness due to respiratory failure, thus needing a noninvasive mechanical ventilation (NIMV) support. However, breathlessness is a symptom that may be induced by different causes that must be taken into consideration in ALS management. Patients concerns: We report the case of an 81-year-old man, with a spinal onset ALS, who was admitted to our clinic to start NIMV because of respiratory involvement. After 3 weeks from NIMV performed at night time, with beneficial effects, he suddenly complained of breathlessness even at rest and in standing position. Diagnosis: Respiratory and cardiac assessments did not show new clinical events, indicating the worsening respiratory function. Due to a history of osteoporosis which was treated with biphosphonates and even though no previous bone trauma or falls were reported, we performed a spine computed tomography scan. The findings indicated multiple dorsal vertebral fractures which was a probable cause for breathlessness . Interventions and outcomes: Considering the neurodegenerative disease associated to respiratory failure, the cardiovascular risk factors and the age, the patient refused to undergo a surgery with kyphoplasty. A spine support was then prescribed, together with analgesic medications, with significant alleviation of pain and breathlessness . Lessons: The occurrence of breathlessness in a patient with ALS cannot always be related to the bulbar involvement. Other causes should be taken into account, especially when there is sudden worsening of symptoms in spite of good clinical response and compliance to NIMV treatment.
机译:原理:肌萎缩性侧索硬化症(ALS)是一种进行性神经退行性多系统疾病,表现为四肢或延髓发作。迄今为止,还没有治愈ALS的方法。在疾病的某个阶段,患者可能会抱怨由于呼吸衰竭而出现呼吸困难,因此需要无创机械通气(NIMV)支持。但是,呼吸困难是由ALS管理中必须考虑的各种原因引起的症状。患者关注的问题:我们报告了一名81岁的男性患者,他患有脊髓性ALS,他因呼吸系统受累而入我们的诊所开始NIMV。在夜间NIMV进行3周的治疗后,产生了有益的效果,他突然抱怨甚至在休息和站立时都出现呼吸困难。诊断:呼吸和心脏评估未显示新的临床事件,表明呼吸功能恶化。由于有使用双膦酸盐治疗的骨质疏松病史,即使以前没有骨伤或跌倒的报道,我们进行了脊柱计算机断层扫描。研究结果表明多发性脊椎骨折是呼吸困难的原因。干预措施和结果:考虑到与呼吸衰竭相关的神经退行性疾病,心血管危险因素和年龄,患者拒绝接受后凸成形术手术。然后开了脊椎支撑支架,并配以止痛药,可明显减轻疼痛和呼吸困难。经验教训:ALS患者的呼吸困难并不总是与延髓受累有关。还应考虑其他原因,尤其是尽管临床反应良好且对NIMV治疗依从性时症状突然恶化。

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