Sialorrhoea, dystonia reduce HRQoL in kids with leukoencephalopathy
Health-related quality of life (HRQoL) is lower in patients with more severe genetically determined leukoencephalopathy, according to a recent study.
Using the Paediatric Quality of Life Inventory (PedsQL), researchers assessed HRQoL in 59 patients diagnosed with genetically determined leukoencephalopathies, of whom 38 were male (mean age 8 years, 10 months) and 21 were female (mean age 9 years, 3 months). T-tests were used to determine differences in HRQoL.
Mean PedsQL scores were significantly lower in patients with at least one severe clinical involvement (52.35±18.40 vs 84.03±9.845; p<0.001). This was also true for the physical (40.73±27.79 vs 87.38±19.99; p<0.001), emotional (64.45±24.14 vs 89.39±7.736; p<0.001) and social (54.24±23.72 vs 80.83±18.55; p=0.01) functioning scores.
In terms of specific clinical features, patients with sialorrhoea had significantly lower mean PedsQL scores (48.18±20.12 vs 61.63±18.90), gastrotomy (41.59±21.54 vs 59.49±19.33) and dystonia (47.5±19.4 vs 67.33±14.80; p<0.05 for all) relative to those without the nonmotor symptom.
Wheelchair use also led to significantly lower PedsQL scores (47.66±18.83 vs 63.00±19.49; p<0.05). All four severe clinical presentations likewise resulted in significantly lower physical functioning scores, while only sialorrhoea was associated with significantly poorer emotional functioning.
“By assessing HRQoL, we have identified areas of concern that can be targeted and prioritized when developing care strategies. Interventions should address these specific concerns regarding physical and social functioning,” said researchers, referring to sialorrhoea, gastrotomy, dystonia and wheelchair use.
“Clinicians should pay particular attention to these features when deciding on supportive treatment strategies,” they added.