Diagnostic Approach in Isolated Bulbar Amyotrophic Lateral Sclerosis: A Case Report

Nunung Nugroho, Albert Setiawan
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Abstract

ABSTRACTIntroduction: Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease. Based on the clinical manifestation, it is divided into spinal form and bulbar form. Bulbar form ALS is divided into two, progressive bulbar and isolated bulbar ALS (IBALS). Patients are diagnosed with IBALS whensymptoms are isolated in the bulbar region for at least six months. The prevalence of IBALS represents only 4% of the neuron disease.Objective: To provide the diagnostic approach of IBALS case based on the clinical signs and symptoms, physical examination, laboratory and radiology findings, electromyography, and fiber optic laryngoscopy examination.Case: A 64-year-old woman presented with slurred speech, the symptoms appeared 2 years ago. Within the last 6 months, the symptoms deteriorated, the patient was unable to speak, and had major difficulty in swallowing food and drinks. The functional status impairment was limited in the bulbar region.Laboratory and radiology findings did not lead to any certain disease. Electromyography and fiber optic laryngoscopy results suggesting a motor neuron disease.Conclusion: It is important to diagnose ALS patients to determine the treatment, progressivity, and prognosis of the disease.Keywords: amyotrophic lateral sclerosis, bulbar form, diagnosis
孤立性球肌萎缩侧索硬化的诊断方法:1例报告
摘要简介:肌萎缩侧索硬化症(ALS)是最常见的运动神经元疾病。根据临床表现分为脊髓型和球型。球型ALS分为进行性球型ALS和孤立性球型ALS (IBALS)。当症状在球区被隔离至少6个月时,患者被诊断为IBALS。IBALS的患病率仅占神经元疾病的4%。目的:根据临床体征、症状、体格检查、实验室及影像学检查、肌电图及纤维喉镜检查,提供IBALS病例的诊断方法。病例:一名64岁女性,表现为言语不清,症状出现于2年前。在过去6个月内,症状恶化,患者不能说话,吞咽食物和饮料有很大困难。功能状态损害仅限于球区。实验室和放射检查结果没有导致任何特定疾病。肌电图和纤维喉镜检查结果提示运动神经元疾病。结论:对ALS患者的诊断对确定治疗、病情进展及预后具有重要意义。关键词:肌萎缩性侧索硬化症,球型,诊断
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