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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