Amytotrophic lateral sclerosis (ALS)
Amytotrophic Lateral Sclerosis (ALS) is a motor neuron disease (MND) known also as Charcot disease. ALS has the genetic background and can be inherited (familial ALS –FALS)or occurs due to spontaneous gene mutations (sporadic ALS –SALS). The common feature in patients with ALS is presence of protein aggregates or inclusions marked with ubiquitin (small protein important in protein degradation process consisting in marking proteins earmarked for degradation) in spinal and bulbar motor neurons. Genetic tests carried on patients with ALS showed presence of specific gene mutations occurring among others in genes encoding the proteins creating inclusions. In the latent phase of the disease the neurons in motor nerves undergo gradual degradation. When the number of neurons decreases below a critical level, the clinical symptoms appear.
ALS is diagnosed on the average a year after the first symptoms have occurred. The clinical phase of the disease starts with limb weakening and atrophy (a decrease in muscle mass) leading to the total paresis of limbs and difficulties with swallowing. Up to 50% of ALS patients show also frontotemporal dementia (the disease caused by abnormal structure of tau protein; it is characterised by progressing speech and personality disorders, antisocial behaviour and lack of personal hygiene, as well as changes in personality, mood, and also abnormal behaviour).
The hitherto test results show a positive impact of mesenchymal stem cells derived from Wharton’s jelly (WJ MSC)use in patients with ALS regarding slowing down/stopping the disease progression, and even improvement in body functions impairedby the disease. There is more and more evidence that WJ MSC support survival of motor neurons in ALS patients.WJ MSC are very well tolerated by the recipient’s immune system, and a detailed analysis of the clinical trials that have been carried out so far on over a thousand patients has proved that administering mesenchymal stem cell in patients suffering various diseases is safe.