Specific codes in ICD-10 are unique alphanumeric designations used to identify and categorize diseases, disorders, and conditions. They consist of 3-5 characters, including both letters and numbers, that provide a high level of detail and specificity.
| Language | Translation |
|---|---|
English
|
Spinal muscular atrophy and related syndromes |
French
|
Amyotrophie spinale et syndromes apparentés |
| Level | Code | Title | |
|---|---|---|---|
| 1 | VI | Diseases of the nervous system | |
| 2 | G10-G14 | Systemic atrophies primarily affecting the central nervous system | |
| 3 | G12 | Spinal muscular atrophy and related syndromes |
| Active Ingredient | Description | |
|---|---|---|
| Nusinersen |
Nusinersen is an antisense oligonucleotide (ASO) which increases the proportion of exon 7 inclusion in survival motor neuron 2 (SMN2) messenger ribonucleic acid (mRNA) transcripts by binding to an intronic splice silencing site (ISS-N1) found in intron 7 of the SMN2 pre-messenger ribonucleic acid (pre-mRNA) and hence when SMN2 mRNA is produced, it can be translated into the functional full length SMN protein. SMA is a progressive neuromuscular disease resulting from mutations in chromosome 5q in the SMN1 gene. Gene SMN2, located near SMN1, is responsible for a small amount of SMN protein production. |
|
| Risdiplam |
Risdiplam is a survival of motor neuron 2 (SMN2) pre-mRNA splicing modifier designed to treat SMA caused by mutations of the SMN1 gene in chromosome 5q that lead to SMN protein deficiency. Functional SMN protein deficiency is directly linked to the SMA pathophysiology which includes progressive loss of motor neurons and muscle weakness. Risdiplam treats SMA by increasing and sustaining functional SMN protein levels. |
