Motor unit hyperexcitability in amyotrophic lateral sclerosis vs amino acids acting as neurotransmitters

• Published in: Acta neurologica Scandinavica Vol. 106; no. 1; pp. 34 - 38
• Main Authors: Kostera-Pruszczyk, A., Niebroj-Dobosz, I., Emeryk-Szajewska, B., Karwańska, A., Rowińska-Marcińska, K.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Munksgaard International Publishers 01.07.2002; Blackwell
• Subjects: Adult; Aged; Amino Acids - blood; Amino Acids - cerebrospinal fluid; amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - physiopathology; Aspartic Acid - blood; Aspartic Acid - cerebrospinal fluid; Biological and medical sciences; Chromatography, High Pressure Liquid; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; double discharges; Electromyography; excitatory and inhibitory amino acids; fasciculation potentials; Female; gamma-Aminobutyric Acid - blood; gamma-Aminobutyric Acid - cerebrospinal fluid; Glutamic Acid - blood; Glutamic Acid - cerebrospinal fluid; Glycine - blood; Glycine - cerebrospinal fluid; Humans; hyperexcitability; Male; Medical sciences; Middle Aged; Motor Neurons - physiology; Muscle, Skeletal - physiopathology; Neurology; Neurotransmitter Agents - blood; Neurotransmitter Agents - cerebrospinal fluid; Reference Values; Human; Nervous system diseases; Aminoacid; Central nervous system disease; Electrophysiology; Excitability; Exploration; Amyotrophic lateral sclerosis; Degenerative disease; Spinal cord disease; Motor unit
• ISSN: 0001-6314; 1600-0404
• DOI: 10.1034/j.1600-0404.2002.00149.x

Objectives– Electrophysiological studies of amyotrophic lateral sclerosis (ALS) patients reveal not only lower motor neuron involvement, but also widespread signs of its hyperexcitability. They might be the consequence of changes in the level of amino acids acting as neurotransmitters. Material and methods– Electrophysiological examination of 31 patients with sporadic ALS was performed. A hyperexcitability index (HI) was created to describe the amount of double discharges, fasciculation potentials or `giant' F‐waves. Glutamate, aspartate, glycine and GABA concentration in serum and cerebrospinal fluid (CSF) were estimated, using the high performance liquid chromatography technique. Results– The electrophysiological studies revealed marked variability in HI in the patients group. HI did not correlate with duration of the disease and the degree of disability expressed with Norris score, as well as with the level of excitatory or inhibitory amino acids in the body fluids. Conclusion– Hyperexcitability of the motor unit observed in ALS is not directly related to changes in serum and CSF level of amino acids acting as neurotransmitters.