Talks：	Sleep Disorders and Respiratory Management of ALS
Name：	楊智超
Position：	Director of Neuromuscular disease
Affiliation：	Department of neurology, National Taiwan University Hospital
Email：	jesse@ntuh.gov.tw
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Research Interests：	Neuromuscular disorders, Rare neurological diseases
Selected Publications：	◆林光華(Kwan-Hwa Lin);胡名霞(Ming-Hsia Hu);簡文仁(Wen-Jen Chien);楊智超(Chih-Chao Yang);蔡清標(Ching-Piao Tsai);鄭悅承(Yueh-Chen Cheng);蔡美文(Mei-Wun Tsai): 肌肉萎縮性側索硬化症之居家物理治療服務模式之建立與其療效：初步結果。物理治療 2004; 29(6): 384-395. ◆Yang CC, Chien YH, Lee NC, Chiang SC, Lin SP, Kuo YT, Chen SS, Jong YJ and Hwu WL: Rapid progressive course of later-onset Pompe disease in Chinese patients. Molecular Genetics and Metabolism 2011 Nov;104(3):284-8. ◆Lai YC, Chen JY, Wu HD, Yang CC, Lin CH, Lee PL: Sleep Disordered Breathing Mimicking Myasthenia Crisis. J Clin Sleep Med 2016
Abstract：	Amyotrophic lateral sclerosis (ALS) also known as Lou Gehrig’s disease is a neurodegenerative disorder characterized by a progressive loss of lower and upper motor neurons . The course of the disease is inexorably progressive. Median survival time varies from 36 to 48 mo. During the course of the disease, the involvement of various respiratory muscle groups leads to a restrictive ventilatory defect and ultimately to hypercapnic respiratory insufficiency. In the majority of cases, death is related to respiratory events . ALS can be associated with profound sleep disturbances resulting from factors such as reduced mobility, muscle cramps, swallowing problems and anxiety. In addition, sleep-disordered breathing, exhibiting as hypoventilation, has been reported in patients with ALS. Respiratory weakness can impact the ability to fall asleep and stay asleep. Diaphragmatic dysfunction in patients with ALS is associated with a dramatic reduction of REM sleep duration, and has impact on survival. Interference with sleep patterns may produce daytime symptoms and activities of daily living can be further affected by an increased incidence of depression. Respiratory function has been proposed to be a strong predictor of quality of life (QoL) and survival in ALS. So far, no single test could predict the respiratory function perfectly and abnormal blood gas is the late event. Forced vital capacity (FVC), the most widely test to assess the respiratory function, is insensitive to substantial deterioration to muscle function. Maximal inspiratory pressure (PI max) and Sniff nasal pressure have been reported to be more sensitive for detecting early respiratory insufficiency than FVC. Nocturnal hypoventilation (NH), an early sign of respiratory failure, usually progresses to daytime hypercapnia within two years in 90% of ALS patients. Non-invasive ventilation (NIV) has been shown to be beneficial for survival and QoL. Though previous guideline recommended FVC<50% of predicted as the criteria for starting NIV, recent guidelines suggest even in patients with FVC <80% of predicted, NIV treatment should be discussed if the symptoms associated with nocturnal hypoventilation developed. One of the ways that sleep centers can help support sleep in those with ALS is by providing a sleep environment with ventilation support to help combat the effects of respiratory weakness in patients.