Amino acids acting as transmitters in amyotrophic lateral sclerosis (ALS)

• Published in: Acta neurologica Scandinavica Vol. 100; no. 1; pp. 6 - 11
• Main Authors: Niebroj-Dobosz, I., Janik, P.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.07.1999; Blackwell
• Subjects: Adult; Aged; ALS; amino acids; Amyotrophic Lateral Sclerosis - blood; Amyotrophic Lateral Sclerosis - cerebrospinal fluid; Amyotrophic Lateral Sclerosis - diagnosis; Aspartic Acid - blood; Aspartic Acid - cerebrospinal fluid; Biological and medical sciences; Brain - metabolism; Cell Death - physiology; Chromatography, High Pressure Liquid - methods; CSF; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Excitatory Amino Acids - blood; Excitatory Amino Acids - cerebrospinal fluid; Female; gamma-Aminobutyric Acid - blood; gamma-Aminobutyric Acid - cerebrospinal fluid; Glycine - blood; Glycine - cerebrospinal fluid; Humans; Male; Medical sciences; Middle Aged; Neurology; Neurons - metabolism; Neurotransmitter Agents - blood; Neurotransmitter Agents - cerebrospinal fluid; plasma; Severity of Illness Index; Human; Nervous system diseases; Pathogenesis; Aspartate; Exploration; Amyotrophic lateral sclerosis; Cerebrospinal fluid; Glutamate; Glycine; Metabolism; Aminoacid; Central nervous system disease; GABA; Adult; Serum; Degenerative disease; Spinal cord disease; Quantitative analysis
• ISSN: 0001-6314; 1600-0404
• DOI: 10.1111/j.1600-0404.1999.tb00717.x

Objectives ‐ In amyotrophic lateral sclerosis (ALS), a neurodegenerative disease of unknown origin, excitotoxic mechanisms are supposed to be involved. Divergent results are, however, presented either because of the heterogeneity of this disease, and/or different methodologies used to evaluate the excitotoxic amino acids content. The results of the most sensitive high performance liquid chromatography (HPLC) techniques with precolumn derivatization of fasting serum and CSF glutamate, aspartate, glycine and γ‐aminobutyric acid (GABA) in mild and severely progressing ALS cases are presented here. Material and methods ‐ We studied 25 ALS patients with different course of the disease and controls, which consisted of 10 cases with other motor neuron diseases and 20 healthy, age‐matched subjects. Results ‐ In the ALS patients with a mild course of the disease serum glutamate and aspartate content was either normal or slightly decreased, in all of these cases a rise in GABA and glycine was present. In the severely progressing ALS cases serum glutamate and aspartate was increased. The GABA content was either normal or increased, the glycine level appeared to be either normal or decreased. In CSF the amino acids changes in ALS were less pronounced as compared to serum. The most frequent finding was the increase in GABA concentration both in the mild and the severely progressing group. CSF glutamate in ALS patients with mild course of the disease was decreased, in the severely progressing cases the glutamate level appeared in a broad range from decreased to increased values. CSF aspartate was either normal or elevated, glycine values were present in a broad range from decreased to increased values. In the other tested motor neuron diseases no consistent changes in serum and CSF amino acids concentration was observed. Conclusions ‐ The data from serum and CSF indicate that in ALS an imbalance between excitatory and inhibitory amino acids might be present in the brain, which may be induced in different ways in particular ALS patients. It may be an important factor for the mediation of neurons death.