Clinical Manifestation and Management of Amyotrophic Lateral Sclerosis

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Ashok Verma, MD, DM, MBA

ABSTRACT


Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive neurodegenerative disease resulting in death in 2 to 4 years in most cases. There are several clinical subtypes of ALS depending on the degree of upper and lower motor neuron involvement, and recognition of these subtypes is important because certain subtypes have better prognosis. Without a reliable biomarker, ALS is a clinical diagnosis supported by laboratory investigations. The etiology of ALS remains unknown. However, mutations in certain genes cause ALS in about 5–8% of cases and understanding molecular pathogenetic pathways in these cases may pave a way for effective therapies. There is currently no cure or meaningfully effective therapy for ALS. Supportive and palliative measures in multidisciplinary ALS clinics are exceedingly important to maintain and improve the quality of life in patients with ALS. This chapter summarizes the clinical features and management of ALS.

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Section
Chapter 1