In this condition both legs are spastic, weak and clumsy, and are held usually in extension and adduction. The gait is the typical scissor gait due to severe spasticity.
There is lesser involvement of the arms, face and bulbar muscles.
Mild disease may go undiagnosed initially and the child may have frequent falls, be clumsy, and show a lack of agility. These children tend to wear their shoes out on the toes and have difficulty hopping and running. Neurological examination reveals brisk tendon reflexes and an extensor planter response. Sometimes this condition may present with shortening of the tendo-Achilles and minimal neurological signs.
There is a delay in motor development and there is often mental retardation.
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