Onset and spreading patterns of upper and lower motor neuron symptoms in amyotrophic lateral sclerosis

• Published in: Muscle & nerve 2011-05, Vol.43 (5), p.636-642
• Main Authors: Körner, Sonja, Kollewe, Katja, Fahlbusch, Marion, Zapf, Antonia, Dengler, Reinhard, Krampfl, Klaus, Petri, Susanne
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - diagnosis; Amyotrophic Lateral Sclerosis - physiopathology; Cohort Studies; Disease Progression; Electromyography; Female; Humans; lower motor neuron; Male; Middle Aged; Motor Neuron Disease - diagnosis; Motor Neuron Disease - physiopathology; Motor Neurons - pathology; Motor Neurons - physiology; Muscle, Skeletal - physiopathology; Retrospective Studies; spreading pattern; upper motor neuron
• ISSN: 0148-639X; 1097-4598
• DOI: 10.1002/mus.21936

Introduction The potential linkage between upper (UMN) and lower motor neuron (LMN) involvement in amyotrophic lateral sclerosis (ALS) has not yet been fully elucidated. There is ongoing discussion as to whether ALS is primarily a disease of UMNs or LMNs. Methods We performed a retrospective analysis of 189 ALS patients from our ALS outpatient database to investigate the different spreading patterns of UMN and LMN affection in disease progression in relation to the onset region. Results The body region with the highest UMN involvement at onset in general also had the highest frequency of LMN signs and vice versa. This is in line with the hypothesis of a focal onset of disease, which then spreads to adjacent areas. However, there was a great variation between ALS phenotypes. Discussion These observations support the hypothesis of focal damage of a localized group of motor neurons, which then spreads to adjacent motor neurons. Muscle Nerve, 2011