Amyotrophic lateral sclerosis with a sudden-onset history

• Main Authors: Takamichi Kanbayashi, Takashi Mikata, Yuki Hatanaka, Masahiro Sonoo
• Format: Article
• Language: English
• Published: Elsevier 2017-01-01
• Series: Clinical Neurophysiology Practice
• Online Access: http://www.sciencedirect.com/science/article/pii/S2467981X17300112

Objective: We report on two patients with amyotrophic lateral sclerosis (ALS) complaining of sudden-onset difficulty in finger elevation. Case report: A 65-year-old man (the first patient) and a 66-year-old man (the second patient) suddenly became aware of difficulty in finger elevation of one hand. They were not aware of any other symptoms prior to the onset. In the first patient, cerebral infarction at the precentral gyrus was initially suspected. In the second patient, cervical spondylosis was initially suspected, and cervical spine surgery was planned. However, needle EMG revealed widespread neurogenic changes and abundant fasciculation potentials for both patients. Widespread weakness emerged in time and relentlessly progressed, and finally the diagnosis of ALS was made. In both cases, notable weakness in the extensor digitorum (ED) muscle with relatively mild weakness in the other muscles in the affected limb was a characteristic finding. Loss of one motor unit in ED that has already enlarged due to reinnervation must have caused sudden awareness of the weakness. Significance: Clinicians should recognize the presence of ALS patients with a sudden-onset history because the risk of initial misdiagnosis is high for such patients. Keywords: Amyotrophic lateral sclerosis, Sudden-onset, Finger elevation, Needle electromyography, Manual muscle testing