The infant’s development starts to slow down from 6 to 12 months of age. If he / she has learnt to walk, this skill is lost and co-ordination and manipulation become increasingly difficult. Over the following months the normal developmental skills ( talking, sitting, responding to surroundings ) go into reverse so that he / she becomes as dependant as a new born baby again and vision deteriorates until it is eventually almost lost. Many young children will develop repetitive hand movements ( that look like knitting movements) and become very floppy ( hypotonic) and increasingly unsteady ( ataxic). If the head is measured the growth is extremely slow and the size small for the child’s age. From about 2 years of age the child will experience body jerks / jumps, which are epileptic (Myoclonic) attacks and sometimes, but not always, generalised epileptic seizures may develop. From about the age of 3 years the jerks often subside; at about this age the child’s limbs will then tend to become stiffer rather than floppy. The condition is not a painful one and the child will not be aware of what is happening. The brain’s control of the muscles responsible for chewing, swallowing, coughing etc eventually becomes affected so that assistance with a feeding tube may be needed, and chestiness will develop and may lead to infections and increasing physical weakness. Eventually the combination of the diseased brain and physical weakness becomes too great to sustain life, and death usually occurs between the ages of 3 – 5 years ( very occasionally up to 8 – 9 years). Parents and carers will be aware of the child’s increasing frailty, and death is usually relatively peaceful and expected when the time comes.