335.0 Werdnig-Hoffman disease
335.1 Spinal muscular atrophy
335.10 Spinal muscular atrophy unspecified
335.19 Other spinal muscular atrophy
G12.0 Infantile spinal muscular atrophy, type I [Werdnig-Hoffman]
G12.8 Other spinal muscular atrophy and related syndromes
G12.9 Spinal muscular atrophy, unspecified
PREFERRED PRACTICE PATTERN
A 10-month-old child is referred for physical therapy in the natural environment (at home) through an early intervention program with a diagnosis of infantile spinal muscular atrophy type 1. The child is on a ventilator with an oxygen saturation machine and G-tube for all feeding. There is no active muscle function or head control. Passive dorsiflexion is limited to –10 degrees. The child is dependent for all mobility and ADLs. The family is interested in assistance with obtaining the appropriate equipment for positioning and transportation.
Summary of changes occurring in a neuron and the structure it innervates when its axon is crushed or cut at the point marked X. Hypersensitivity of the postsynaptic structure to the transmitter previously secreted by the axon occurs largely due to the synthesis or activation of more receptors. There is both orthograde (wallerian) degeneration from the point of damage to the terminal and retrograde degeneration of the axon stump to the nearest collateral (sustaining collateral). Changes also occur in the cell body, including chromatolysis. The nerve starts to regrow, with multiple small branches projecting along the path the axon previously followed (regenerative sprouting). (From Barrett KE, Berman SM, Boitano S, Brooks HL. Ganong’s Review of Medical Physiology. 24th ed. New York, NY: McGraw-Hill; 2012.)
SMA type I: Weakness between birth and 3 months of age
SMA type II: Signs and symptoms by 3 years of age
SMA type III: Signs and symptoms between 2 and 9 years of age
SMA type IV: Adult onset
Type I: 27% of all SMA cases
Types II and III: 46% of all SMA cases
Type IV: 8% of all SMA cases...
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