BackgroundNarcolepsy is a rare neurological sleep disorder especially in children who are younger than 10 years. In the beginning of 2010, an exceptionally large number of Finnish children suffered from an abrupt onset of excessive daytime sleepiness (EDS) and cataplexy. Therefore, we carried out a systematic analysis of the incidence of narcolepsy in Finland between the years 2002–2010.MethodsAll Finnish hospitals and sleep clinics were contacted to find out the incidence of narcolepsy in 2010. The national hospital discharge register from 2002 to 2009 was used as a reference.FindingsAltogether 335 cases (all ages) of narcolepsy were diagnosed in Finland during 2002–2009 giving an annual incidence of 0.79 per 100 000 inhabitants (95% confidence interval 0.62–0.96). The average annual incidence among subjects under 17 years of age was 0.31 (0.12–0.51) per 100 000 inhabitants. In 2010, 54 children under age 17 were diagnosed with narcolepsy (5.3/100 000; 17-fold increase). Among adults ≥20 years of age the incidence rate in 2010 was 0.87/100 000, which equals that in 2002–2009. Thirty-four of the 54 children were HLA-typed, and they were all positive for narcolepsy risk allele DQB1*0602/DRB1*15. 50/54 children had received Pandemrix vaccination 0 to 242 days (median 42) before onset. All 50 had EDS with abnormal multiple sleep latency test (sleep latency <8 min and ≥2 sleep onset REM periods). The symptoms started abruptly. Forty-seven (94%) had cataplexy, which started at the same time or soon after the onset of EDS. Psychiatric symptoms were common. Otherwise the clinical picture was similar to that described in childhood narcolepsy.InterpretationA sudden increase in the incidence of abrupt childhood narcolepsy was observed in Finland in 2010. We consider it likely that Pandemrix vaccination contributed, perhaps together with other environmental factors, to this increase in genetically susceptible children.
The objective of this study was to investigate the effects of botulinum toxin A (BTXA) treatment on impairment and function of the upper limb during a 2-year follow-up period. A prospective longitudinal study design with assessments before and after intervention was utilized, involving six patients with cerebral palsy (three boys and three girls) aged 3 years 4 months to 11 years 11 months at commencement of study. The outcome measures were spasticity (modified Ashworth, MAS), active and passive range of movement (ROM), grips (pinch, key grip, 3-finger grip, narrow cylinder grip, wide cylinder grip, pen grip and diagonal grip; grasping, releasing; pronation-supination), bimanual functions, fine motor functions (Melbourne Assessment of Unilateral Upper Limb Function), movement pattern (Upper Limb Physician's Rating Scale, ULPRS), functional skills and self-care capability (Paediatric Evaluation of Disability Inventory, PEDI), upper extremity use (House Classification) and cosmetic appearance. The assessments were repeated by the same examiners at baseline and at 1, 3 and 6 months after each BTXA treatment and then every 6 months until 24 months. One subject received a total of four injections (at 0, 6, 12 and 18 months), one two injections (at 0 and 12 months) and four one injection at the beginning of the study period. Upper extremity surgery was performed on two subjects during the study and one was operated on 2 months after completion of the study. All children benefited from the BTXA treatment in terms of reduction in muscle tone and increase in active and passive ROM. By 6 months, spasticity returned, but in four children passive and especially active ROM remained better than at baseline. No significant changes in grips, bimanual tasks or Melbourne Assessment scores were detected. The change in movement pattern (ULPRS) was maintained for 3 months in two children and beyond this in four, thus extending beyond the pharmacologic effects of botulinum toxin A. All but one child showed improvement in PEDI functional skill and caregiver assistance scale scores during the 2-year period. The House classification showed a one-grade improvement in one child at 1 month and in one child at 3 months and a three-grade improvement in one child at 3 months after BTXA treatment. After each treatment, the parents reported at least a one-grade improvement in cosmetic appearance in all children at 1 month and in four children maintained at least until 6 months. In two subjects operated during the study period, a distinct improvement in active and passive ROM and a two-grade improvement in the House classification were observed after the operation. In this limited series, the reduction in muscle tone after BTXA treatment did not translate into better gripping or quality of fine motor functions (Melbourne Assessment) of the affected hand, but seemed to have a positive effect on upper limb movement pattern (ULPRS), upper extremity use (House Classification) and cosmetic appearance. Assessment of upper limb function in a child with ...
Objective We conducted a randomized controlled trial to evaluate whether a combination of repeated botulinum toxin A (BTX-A) and conservative treatment is more effective in decreasing toe-walking than conservative treatment alone at 24 months follow-up. Patients and Methods Children between 2 and 9 years of age were randomized either into the conservative (CO) or botulinum treatment (BTX) group. The treatment in the CO group consisted of firm shoes, night splints, a home stretching program and physiotherapy. The BTX arm had all the same conservative treatments added with calf muscle BTX-A injections repeated in 6 months intervals if needed. Change in toe-walking pattern, ankle range of movement (ROM), and overall function were assessed at baseline and 6, 12, 18, and 24 months posttreatment. Results A total of 30 toe-walkers participated: 14 in CO and 16 in BTX group. At 24 months, all children in the BTX group and 85% in the CO group evaluated by the blinded physiotherapist (p ¼ 0.065), 75% in the BTX group and 70% in the CO group graded by the research physiotherapist (p ¼ 0.730), and 50% in the BTX group and 54% in the CO group reported by the parents ceased toe-walking (p ¼ 0.837). The most prominent change was noted during the 1st year. The BTX group seemed to reach the goal earlier. No significant differences between the treatment groups in function or in ankle ROM ensued. Conclusion Adding BTX injections did not significantly enhance the goal to walk either flat foot or with heel strike at 24 months posttreatment.
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