In contrast, a new scale has been recently proposed for assessing function in patients with neuromuscular disorders with more severe functional impairment but this was designed to be used in non-ambulatory patients only. but although they all undoubtedly have their uses, none has been shown to be sufficiently sensitive when assessing functional change in weaker, non-ambulant children. There are also other scales, such as the Vignos scale 7 which determines levels of mobility and others assessing ability in function and ‘activities of daily living’,8., 9., 10. The scale is also useful when assessing children with other neuromuscular disorders who have deteriorating ambulation but it is not a reliable indicator of change in ability for many of the weaker non-ambulant children. The scale helps to monitor disease progression and gives an indication of when boys with Duchenne Muscular Dystrophy are about to lose independent ambulation. In our department, functional motor scoring was based almost exclusively on the scale developed by Scott, Hyde et al published in 1982 6 devised for boys with Duchenne Muscular Dystrophy. There are numerous functional motor scales that can be used for patients with neuromuscular disorders, but these cannot always be used in non-ambulant children or in those with limited ambulation. As a result a significant proportion of children with SMA type 3 can lose the ability to walk independently.4., 5. Long term survival is expected for children with the type 2 and type 3 and currently available medical treatment is directed at secondary complications such as respiratory problems or management of deformities.Īlthough the weakness itself does not progress in SMA, several studies have documented a deterioration in functional abilities due to increased body size without an increase in power to cope with these extra demands. Prognosis is dependent on severity and almost invariably children with Werdnig–Hoffman disease will die of respiratory complications by the age of 2 years. Children with the intermediate form (SMA type 2) are, in contrast, able to sit but not to walk independently.2., 3. The mildest form is Kugelberg–Welander disease (SMA type 3) in which independent ambulation is achieved but mild to moderate proximal muscle weakness may be experienced, causing difficulty with stairs and rising from the floor. There is a wide range of severity, the most severe form being Werdnig–Hoffman disease (or SMA type 1), in which affected children are unable to sit unsupported. 1 It is the second most common neuromuscular disease of childhood affecting approximately 1:10,000–12,000 children. SMA is a neuromuscular condition due to the degeneration of the anterior horn cells, inherited in an autosomal recessive way.
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