Asked by: Lyubka Dosal
asked in category: General Last Updated: 7th January, 2020

What is Werdnig Hoffmann disease?

Werdnig-Hoffman disease (spinal muscular atrophy type 1 or SMA-1): one of the types of spinal muscular atrophy (SMA) diseases. SMAs are a group of inherited diseases that are characterized by progressive weakness of the muscles and reduced muscle tone.

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Also question is, what is the life expectancy of someone with SMA?

Life expectancy Most children with type 1 SMA will only live a few years. However, people who've been treated with new SMA drugs have seen promising improvements in their quality of life — and life expectancy. Children with other types of SMA can survive long into adulthood and live healthy, fulfilling lives.

Subsequently, question is, what is SMA medical condition? Spinal muscular atrophy (SMA) is a group of neuromuscular disorders that result in the loss of motor neurons and progressive muscle wasting. The severity of symptoms and age of onset varies by the type. SMA is a leading genetic cause of death in infants.

Herein, what is sma1?

Spinal muscular atrophy 1 (SMA1), also known as Werdnig Hoffmann disease, is a genetic neuromuscular disorder that affects the nerve cells that control voluntary muscles (motor neurons). This occurred because treatments are being developed that are changing the course of the disease.

Which clinical manifestations in an infant would be suggestive of spinal muscular atrophy Werdnig Hoffmann disease )?

Infants with SMA1 experience severe weakness before 6 months of age, and the patient never achieves the ability to sit independently when placed. Muscle weakness, lack of motor development and poor muscle tone are the major clinical manifestations of SMA1.

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