Treatment of Spinal Muscolar Atrophy with Intrathecal Mesenchymal Cells

Treatment of Spinal Muscolar Atrophy with Intrathecal Mesenchymal Cells

Author or authors of report : Marino Andolina
Date of report : 2012-05-05
Spinal Muscular Atrophy (SMA)
Background and Objectives:
  • SMA1 is a genetic disease causing progressive apoptosis of the second motoneuron, leading to complete paralysis.
  • Given the reported efficacy of mesenchymal cells in treating other neurological diseases, the study aimed to treat children with SMA1 using these cells.
Methods and Results:
  • Four children with SMA1 were treated with intrathecal injections of mesenchymal cells.
  • All patients showed improvement in motility after three weeks, especially in distal muscles. Proximal muscles were less affected.
  • The treatment's effect lasted for about 30 days, necessitating repeated treatments once a month for 3 to 8 months.
  • One patient who stopped the treatment died after 45 days. Another became completely paralyzed two months after stopping the therapy but regained skills after a new injection. The remaining two patients remained stable after initial improvement.
Conclusions:
  • Intrathecal injections of mesenchymal cells enhance the motility of children with SMA1.
  • Early treatment, before irreversible neurological damage, might potentially cure the disease.
Additional Information:
  • Spinal muscular atrophy (SMA) is a group of diseases caused by a defect in the SMN1 gene. The most severe form, SMA1, manifests in the first few months of life, leading to severe muscle activity decline and eventual respiratory failure.
  • Current drugs can slow the disease's progression, but a definitive cure is not yet available.
  • Mesenchymal cells have been used to treat neurological diseases like Parkinson's and brain injuries. They can cross the blood-brain barrier, produce factors that interfere with apoptosis and neuronal growth, and can mature into tissues like neurons and glia.
  • The study suggests that the short-lived effect of intrathecal mesenchymal cells is due to the local release of soluble factors, not regeneration.