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AimEvaluation of impaired quality of life (QOL) of Malaysian children with tuberous sclerosis complex (TSC) and its possible risk factors.MethodCross-sectional study on 68 parents of Malaysian children aged 2–18 years with TSC. QOL was assessed using proxy-report Paediatric Quality of Life Inventory (PedsQL) V.4.0, and scores compared with those from a previous cohort of healthy children. Parents also completed questionnaires on child behaviour (child behaviour checklist (CBCL)) and parenting stress (parenting stress index-short form). Multiple regression analysis was used to determine sociodemographic, medical, parenting stress and behavioural factors that impacted on QOL.ResultsThe mean proxy-report PedsQL V.4.0 total scale score, physical health summary score and psychosocial health summary score of the patients were 60.6 (SD 20.11), 65.9 (SD 28.05) and 57.8 (SD 19.48), respectively. Compared with healthy children, TSC patients had significantly lower mean PedsQL V.4.0 total scale, physical health and psychosocial health summary scores (mean difference (95% CI): 24 (18–29), 20 (12–27) and 26 (21–31) respectively). Lower total scale scores were associated with clinically significant CBCL internalising behaviour scores, age 8–18 years and Chinese ethnicity. Lower psychosocial health summary scale scores were associated with clinically significant CBCL internalising behaviour scores, Chinese ethnicity or >1 antiepileptic drug (AED).ConclusionParents of children with TSC reported lower PedsQL V.4.0 QOL scores in all domains, with psychosocial health most affected. Older children, those with internalising behaviour problems, of Chinese ethnicity or on >1 AED was at higher risk of lower QOL. Clinicians need to be vigilant of QOL needs among children with TSC particularly with these additional risk factors.

Acute necrotizing encephalopathy (ANE) in childhood is a rare clinico-radiological syndrome with distinctive bilateral thalamic lesions. The diagnosis is often based on the criteria proposed by Mizuguchi et al. 1 It comprises a spectrum of virus-associated encephalopathy, 2 often but not exclusively associated with influenza infection and a familial and a relapsing form have been reported. The mortality rates are varied, ranging from 0% to 43% and survivors often have severe disabilities. [3][4][5][6][7][8] Whilst there is no clear consensus for ANE treatment, 9 the key therapeutic strategy is the early initiation of firstline immunotherapy, particularly of corticosteroids. 4,9 More C