What is the Difference Between ALS and SMA?
🆚 Go to Comparative Table 🆚Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA) are both neurodegenerative diseases that affect motor neurons and lead to muscle weakness and atrophy. However, they have distinct differences in their genetic causes, inheritance patterns, and typical age of onset.
Differences between ALS and SMA include:
- Genetic causes: SMA is caused by deletions or mutations in the survival motor neuron 1 (SMN1) gene, while ALS can be caused by mutations in various genes, such as superoxide dismutase 1 (SOD1), Fused in Sarcoma (FUS), TAR DNA-binding protein 43 (TDP-43), and chromosome 9 open reading frame 72 (C9orf72).
- Inheritance: SMA is an inherited genetic disease, passed from parent to child, while ALS is typically not inherited and has mostly sporadic cases.
- Age of onset: SMA can present at different ages, with types 0, 1, 2, and 3 affecting infants and children at varying ages, and type 4 affecting adults. ALS, on the other hand, usually occurs in adults.
- Molecular links: The ASC-1 complex has been identified as a molecular link between ALS and SMA, with both diseases showing disrupted molecular pathways involving the ASC-1 complex, FUS, SMN1, and other ALS/SMA-causative proteins.
Similarities between ALS and SMA include:
- Motor neuron dysfunction: Both diseases are motor neuron diseases that affect muscle movement or motor function.
- Progressive nature: Both ALS and SMA are progressive diseases, meaning they continue to worsen over time once they begin.
- Neuromuscular junction pathology: A common early feature of both ALS and SMA is the loss of contact between nerve and muscle at the neuromuscular junction.
In summary, ALS and SMA are both neurodegenerative diseases that affect motor neurons and muscle function, but they differ in their genetic causes, inheritance patterns, and typical age of onset. Both diseases share some common pathological features, such as the loss of contact between nerve and muscle at the neuromuscular junction.
Comparative Table: ALS vs SMA
Here is a table highlighting the differences and similarities between ALS and SMA:
Feature | ALS (Amyotrophic Lateral Sclerosis) | SMA (Spinal Muscular Atrophy) |
---|---|---|
Inheritance | Autosomal dominant when inherited, sporadic most of the time | Autosomal recessive when inherited |
Onset | Symptoms usually start in adulthood | Symptoms start in infancy or early childhood |
Types | One form, with varying degrees of severity | Five types, based on severity and age of onset |
Motor Neurons Affected | Differential vulnerability, with both upper and lower motor neurons affected | Differential vulnerability, with selective loss of lower motor neurons |
Neuromuscular Junction | Loss of contact between nerve and muscle, "dying-back" pathology | Similar pattern of early changes at the neuromuscular junction |
Progression | Progressive disease, with symptoms worsening over time | Progressive disease, with symptoms worsening over time |
Treatment | FDA-approved medications available to slow functional decline and help people live longer | Management focuses on supportive care and early intervention |
ALS and SMA are both motor neuron diseases that affect muscle movement or motor function. While they share some similarities, such as progressive worsening over time, they differ in areas like inheritance, age of onset, and types of affected motor neurons.
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