Clinical Features and Clinical Variants of Amyotrophic Lateral Sclerosis

Kim, Nam-Hee; Yoon, Byoung Jun

Review article

Korean Journal of Neuromuscular Disorders 2014;6(2):35-47.

Nam-Hee Kim, Byoung Jun Yoon

Department of Neurology, Dongguk University Ilsan Hospital, Goyang, Korea

근위축측삭경화증의 임상양상과 아형

김남희, 윤병준

동국대학교 일산병원 신경과

Abstract

Amyotrophic lateral sclerosis (ALS) is a degenerative central nervous system disorder caused by death of motor neurons in the brain and spinal cord. The clinical syndrome is characterized by muscle weakness, often beginning focally and gradually progressing over 1 or more years to involve muscles diffusely. The diagnosis is established by documenting clinical evidence of both upper and lower motor neuron dysfunction and by performing diagnostic tests to exclude many other disorders of the central and peripheral nervous system that might alternatively account for the clinical presentation. The revised El Escorial criteria for ALS may be useful for patients participating in research studies. Recognition of clinical features, clinical variants, and mimicking disorders is important because several are potentially treatable or may not carry the same grave prognosis as ALS and the precise diagnosis of ALS can promote more clinical trials for ALS.