STUDY OF REINNERVATION PROCESS IN PATIENTS WITH 2 TYPEOF SPINAL MUSCULAR ATROPHY: CLINICAL EXPERIMENTAL STUDY

Abstract

Spinal muscle atrophy (SMA) of type 2 is an autosomal-recessive disease defined by a degenerative change in alpha-motor neurons of anterior horns. The disease is manifested in weakness of proximal muscles, pareses, respiratory disturbance and early death. Study of reinnervation process in patients with 2nd type of SMA can provide valuable information on a condition of adaptive mechanisms within the limits of neuron plasticity. According to electroneuromyography study in patients with 2nd type SMA reinnervation process is low intense. It was found out that concentration NGF (nerve growth factor) in serum of patients with 2nd type SMA is considerably higher than in control group. We studied the influence of blood serum of patients with 2 type SMA on growth neurites of sensory ganglia of 10-12 days old chicken embryos in organotypic culture. For the first time it was shown that blood serum of patients with 2nd type SMA dose-dependent inhibits of growth neurites of sensory ganglia. Apparently, neurite-inhibitory effect is caused by high concentration of neurotrophins in patient serum, due to that the reinnervation mechanisms in 2 type SMA patients are not so efficient.