Table 1.
Patient characteristics
| Patient characteristics | sMLD | dMLD | Spastic CP |
|---|---|---|---|
| Sex (n) | |||
| Male | 4 | 4 | |
| Female | 4 | 2 | 4 |
| Clinical subtype (n) | |||
| Late infantile | 0 | 0 | |
| Early juvenile | 3 | 0 | |
| Late juvenile | 5 | 2 | |
| Adult | 0 | 0 | |
| Type of predominant movement disorder (n) | |||
| Spastic | 8 | 0 | 8 |
| Dyskinetic | 0 | 2 | 0 |
| Age at diagnosis | |||
| Mean (range) | 8y (5–14y) | 8y (7–9y) | |
| Age when wheelchair dependent | |||
| Mean (range) | 9y (6–17y) | 8y 6mo (8–9y) | |
| Age at pump implantation | |||
| Mean (range) | 11y (6–24y) | 9y 6mo (9–10y) | 11y (5–26y) |
| GMFC‐MLD/GMFCS levela (n) | |||
| III | 1 | 0 | 0 |
| IV | 2 | 0 | 3 |
| V | 4 | 2 | 5 |
| VI | 1 | 0 | |
| Position of catheter tip (n) | |||
| C4 | 0 | 2 | 0 |
| Th4 | 5 | 0 | 3 |
| Th10 | 3 | 0 | 5 |
| Purpose of ITB (multiple goals possible) (n) | |||
| Improve care | 7 | 0 | 8 |
| Decrease pain | 1 | 2 | 2 |
| Improve transfers | 1 | 0 | 0 |
| Improve sitting position | 3 | 1 | 1 |
| Prevent contractures | 0 | 0 | 1 |
| Complications (n) | |||
| Pump infectionb | 0 | 0 | 1 |
| Catheter disconnectionc | 1 | 0 | 1 |
aScore at pump implantation. bAt 21 days: negative cultures, removal of the pump. Second implantation included in the study. cOne patient with sMLD at 20 days: revision of the catheter; one patient with spastic CP at 1mo: revision of the catheter. sMLD, metachromatic leukodystrophy with a predominant spastic movement disorder; dMLD, metachromatic leukodystrophy with a predominant dyskinetic movement disorder; CP, cerebral palsy; GMFC‐MLD, Gross Motor Function Classification in metachromatic leukodystrophy; GMFCS, Gross Motor Function Classification System; ITB, intrathecal baclofen.