A recent University Hospital Essen research effort finds that people with spinal muscular atrophy (SMA) do not require higher radiation to monitor the administration of Spinraza (nusinersen) in the spinal canal despite having severe anatomical alternations as reported in Springer and in SMA News Today.
Titled “Radiation Exposure of Image-Guided Intrathecal administration of nusinersen to adult patient with spinal muscular atrophy” was published recently in Neurology.
SMA is made of a group of neurodegenerative disorders characterized by the gradual loss of motor neurons. These represent the nerve cells that help the human body control voluntary muscles—in the spinal cord—leading to muscle weakness. It is typically caused by mutations in the SMN1 gene, which directs the making of the SMN protein—an essential contributor to motor neuron survival. Biogen’s Spinraza is a treatment that works by increasing production of the SMN protein by the SMN2 gene—similar to the SMN1 gene and usually unaffected in SMA patients.