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Table 1 Progressive nature of AGU: Growth, cognition, personality, speech, self-help, and motor skills according age. ID = intellectual disability

From: Aspartylglycosaminuria: a review

Age in years Growth Cognition Personality Speech Self-help Motor skills
<2 Early growth spurt +1- +2sd Normal “An easy baby” Normal Normal Stiffness in hips
2–5 +1SD Subnormal Well-behaved and fussy Delayed Delayed Walks clumsily
6–9 0- + 1sd Mild ID Talkative, kind, stubborn Unclear Delayed Can bike and ski, not skate
10–15 Slight and short
pubertal growth spurt,
early menarche,
macro-orchidism
Moderate ID Fond of children, joyful Clear Independent in toileting, dresses and undresses No change
16–19 Growth ceases,
-1SD
Moderate/severe ID Tends to withdraw “Soft” Able to do little shopping No change
20–24 Girls gain weight Severe ID Attached to parents,
shows no interest
to opposite sex
“Soft” Able to move outdoors in familiar surroundings No more biking nor skiing
25–34 No change Severe ID Calm, passive Vocabulary decreases Active and passive periods May walk without a goal
35–44 No change Severe ID Confused Few words Constant need of help Legs seem not to respond
45+ Loss of weight Profound ID Sits still for hours,
angry when disturbed
No speech Constant need of help Poor balance/wheel chair