14800-DvRappard

67 Intrathecal baclofen treatment: metachromatic leukodystrophy versus spastic cerebral palsy 4 Ethics The medical ethical committee of the VU University Medical Center Amsterdam approved the study. RESULTS Eleven patients with MLD were treated with ITB between 2006 and 2016 (Figure 1). One of them was excluded from the study because of removal of the baclofen pump due to infection with staphylococcus aureus within two weeks; reimplantation was followed by re-infection and definitive pump removal. Eight patients had SMLD, two DMLD. The eight matched SCP patients for the SMLD group had a pump implantation between 2002 and 2016. Patient characteristics are summarized in table 1. In oneMLD patient and in one SCP patient, catheter disconnection occurred as a complication, necessitating operative revision. In one SCP patient, the baclofen pump became infected within 1 month after implantation, leading to pump removal. The patient was included in the study after reimplantation. All MLD patients were wheelchair dependent within three years after diagnosis (mean 8y 10m, range 6y - 17y). Six out of eight patients with SMLD (aged 6 to 8 years) were started on ITB therapy within two years after wheelchair dependency. In two older children (13 and 17 years old at the moment of wheelchair dependency), ITB treatment started seven years later. In both patients with DMLD, ITB therapy started one year after wheelchair dependency. Metachromatic leukodystrophy In the eight SMLD patients, mean starting dose was 68μg per day (SD 23.60, range 40 – 100). Both DMLD patients started at 50μg per day. At six months, five out of eight SMLD patients and both DMLD patients had a flexible programwith bolus administration. The mean dose in SMLD and DMLD at six months was 210μg (SD 119.97, range 70 – 428) and 308μg (SD 81.32, range 250 – 365), respectively (Figure 2). In patients who had a flexible program, mean dose of baclofen given by boluses was 112μg per day (SD 128.29, range 10 – 335) administered in 2.2 (SD 0.45, range 2 – 3) boluses in SMLD, and 140μg per day (SD 84.85, range 80 – 200) in 2.5 (SD 0.71, range 2 – 3) boluses in DMLD (Table 2). No correlation was found between age at pump implantation and baclofen dosage at six months (R = 0.33, p = 0.35 ).