Amyotrophic lateral sclerosis: clinical features and current treatment approaches

Arsiv Kaynak Tarama Dergisi

Tuba Tulay Koca¹ 

[1] Uzm Dr/Fiziksel Tip ve Rehabilitasyon/sehir Devlet Hastanesi/Malatya/Turkiye;

关键词: Motor neuron diseases;    amyotrophic lateral sclerosis;    frontotemporal dementia;    riluzole;    treatment;   

DOI  :  10.17827/aktd.79454

来源: DOAJ

【 摘 要 】

Amyotrophic lateral sclerosisalso known as Lou Gehring's disease, is the most common motor neuron disease characterized by motor neuron degeneration in the primary cortex, brainstem and spinal cord. This leads to widespread paralysis, respiratory insufficiency and death within an average of 3-5 years from disease onset. Majority of cases is sporadic and only 10% have a family story.One of the most interesting discovery in the field of neurodegeneration in recent years is genetic mutation in the C9orf72 (chromosome 9 open reading frame 72) gene, the most common mutation found to be causative of frontotemporal dementia, amyotrophic lateral sclerosis and concomitant of these two diseases. Currently curative therapy for amyotrophic lateral sclerosis is lacking. To date, one medication, Riluzole, has been proved to prolong survival, approximately 3-5 months, in amyotrophic lateral sclerosis. Researches aim to slow disease progression by targeting known pathophysiological pathways or genetics defects. Only symptomatic care to improve quality of life and survival is suggested. These includes respiratory and nutrition support; dysphagia and gastrostomy management; communication and mobility programs; spasticity prevention; pain medication; management of cognitive dysfunction, depression, mood dysorders (especially apathy), fatigue, sleep disturbance and prevention of deep venous thrombosis. [Archives Medical Review Journal 2015; 24(2.000): 182-194]

【 授权许可】

Unknown