Amyotrophic lateral sclerosis (ALS) is the most common motor neurone disorder in human adults. It is characterized by selective and progressive degeneration of upper and lower motor neurones in the spinal cord, brain stem and motor cortex. Clinically this process results in progressive muscle weakness, muscle atrophy and eventual paralysis. In ALS and other degenerative diseases, endocrine alterations have been reported. In ALS in particular, a decreased TRH content of the anterior horn region and alterations of arginine vasopressin (AVP) secretion are known to occur both in animals and humans.
1- Growth hormone (GH) secretion was studied in myotonic dystrophy, a multisystem disease characterized by myotonia and skeletal muscle atrophy. An abnormal pattern of 24 h-GH secretion was observed, despite normal basal serum GH.
2- A significant decrease in GH response to GHRH stimulus in myotonic dystrophy patients as compared to controls was reported.
3- In contrast, scanty data is available on GH secretion in ALS, although reduction in IGF-I levels have been reported.
4- In addition, recombinant IGF-I was proposed as a treatment for ALS, as this protein possesses both the capacity to promote motor neurone survival and to enhance motor nerve regeneration from surviving neurones.
5- The aim of the present study was to investigate the GH-IGF-I axis in ALS patients.