What causes flail arm syndrome?
As many patients with flail arm syndrome develop upper motor neuron signs in the lower limbs this syndrome probably represents a variant of ALS. It seems likely that unknown factors linked to male sex predispose a proportion of patients to develop the flail arm phenotype.
What is flail limb syndrome?
Abstract. Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles.
What do the early signs of motor neurone disease MND include?
Early symptoms can include:
- weakness in your ankle or leg – you might trip, or find it harder to climb stairs.
- slurred speech, which may develop into difficulty swallowing some foods.
- a weak grip – you might drop things, or find it hard to open jars or do up buttons.
- muscle cramps and twitches.
What is limb onset ALS?
When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS, and when individuals first notice speech or swallowing problems, it is termed “bulbar onset” ALS. As the disease progresses, muscle weakness and atrophy spread to other parts of the body.
How long do PLS patients live?
The median duration of PLS is approximately 20 years, while the duration of ALS is two to five years, so PLS prevalence is high relative to incidence because people with the disease live longer.
How is flail arm syndrome different from motor neuron disease?
Flail arm syndrome (FAS) is a variant of motor neuron disease which is characterized by progressive, predominantly proximal weakness and atrophy of the upper limbs (UL). Because of its heterogeneous presentation and its relatively slow progression, differential diagnosis may be difficult particularly in the early stages of the disease.
Can a flail arm have a slower progression than MND?
Apparently flail arm can have a slower disease progress than other versions of MND. His MND specialist said that this is not always the case and I suspect he knew Tony was a faster progression. Hopefully your progress will be slower. We have lots of input from occupational therapy, physios, speech and language etc and they all make things easier.
What are the symptoms of upper motor neurone disease?
In this type of MND, both the upper motor neurones and lower motor neurones degenerate at the same time. Symptoms and signs depend upon the area of the body where the muscles are affected first (e.g. arm or leg) and can include muscle wasting, weakness, stiffness, cramps and rippling of the muscles, known as fasciculations.
Which is worse motor neurone disease or ALS?
Progressive muscular atrophy accounts for perhaps 10% of cases of MND. In this type of MND, only the lower motor neurones degenerate and life expectancy is longer than that in ALS. Sometimes MND can initially present as progressive muscular atrophy but over time the upper motor neurons can be involved and the diagnosis then changes to ALS.