Table 1.
Severity score including 5 items, each being rated from 0 (worst score) to 3 (best score)
| Score | |
|---|---|
| Head circumference | |
| Normal head circumference for age | 3 |
| Head circumference between − 2 SD and − 3 SD (limits included) | 2 |
| Head circumference between − 3 SD and − 5 SD (limits excluded) | 1 |
| Head circumference equal to or below − 5 SD | 0 |
| Weight/height | |
| Absence of growth delay | 3 |
| Weight and/or height between − 2 SD and − 3 SD (limits included) | 2 |
| Weight and/or height between − 3 SD and − 5 SD (limits excluded) | 1 |
| Weight and/or height equal to or below − 5 SD | 0 |
| Neurosensorial symptoms | |
| No neurosensorial symptom | 3 |
| Neurosensorial symptoms from one or two categoriesa | 2 |
| Neurosensorial symptoms from three categoriesa | 1 |
| Neurosensorial symptoms from four categoriesa | 0 |
| Autonomy/motor development | |
| Normal motor skills for age | 3 |
| Moderate motor impairment/motor delay | 2 |
| Severe motor impairment/motor delay (or no standing position after 2 years of age) | 1 |
| No motor development/bedridden | 0 |
| Communication | |
| Normal communication and language for age | 3 |
| Moderate communication impairment/speech delay | 2 |
| Severe communication impairment (or non-verbal communication only after 2 years) | 1 |
| No communication | 0 |
| Total | 15 |
SD standard deviation
aNeurosensorial categories: 1—cerebellar signs (ataxia, action tremor, cerebellar dysarthria); 2—pyramidal or extrapyramidal signs (spasticity, rigidity, akinesia); 3—peripheral nerve involvement (hyporeflexia or areflexia); 4—sensorial impairment (neurosensorial hearing loss, retinopathy, cataracts)