Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study.

BACKGROUND: Progressive encephalopathy (PE) in children is a heterogeneous group of diseases mainly composed of metabolic diseases, but it consists also of neurodegenerative disorders where neither metabolic nor other causes are found. We wanted to estimate the incidence rate and aetiology of PE, as well as the age of onset of the disease. METHODS: We included PE cases born between 1985 and 2003, living in Oslo, and registered the number presenting annually between 1985 and 2004. Person-years at risk between 0 and 15 years were based on the number of live births during the observation period which was divided into four 5-year intervals. We calculated incidence rates according to age at onset which was classified as neonatal (0-4 weeks), infantile (1-12 months), late infantile (1-5 years), and juvenile (6-12 years). RESULTS: We found 84 PE cases representing 28 diagnoses among 1,305,997 person years, giving an incidence rate of 6.43 per 100,000 person years. The age-specific incidence rates per 100,000 were: 79.89 (<1 year), 8.64 (1-2 years), 1.90 (2-5 years), and 0.65 (>5 years). 66% (55/84) of the cases were metabolic, 32% (27/54) were neurodegenerative, and 2% (2/84) had HIV encephalopathy. 71% (60/84) of the cases presented at < 1 year, 24% (20/84) were late infantile presentations, and 5% (4/84) were juvenile presentations. Neonatal onset was more common in the metabolic (46%) (25/55) compared to the neurodegenerative group (7%) (2/27). 20% (17/84) of all cases were classified as unspecified neurodegenerative disease. CONCLUSION: The overall incidence rate of PE was 6.43 per 100,000 person years. There was a strong reduction in incidence rates with increasing age. Two-thirds of the cases were metabolic, of which almost half presented in the neonatal period.

[Experiences with botulinum toxin injections against spasticity in children]. / Erfaringer med botulinumtoksininjeksjoner mot spastisitet hos barn.

BACKGROUND: Botulinum toxin injection is one of the newer options in the treatment of spasticity. Treatment with botulinum toxin is always combined with physiotherapy and often with casting. We show the extent to which botulinum toxin treatment has been taken into use in our department and discuss advantages and disadvantages of giving botulinum toxin injections at local hospitals. MATERIAL AND METHODS: 88 children with cerebral palsy aged 14 months to 16 years were treated with botulinum toxin between March 2000 and Dec. 2004. Injections were given in an outpatient setting, usually with the patient sedated with midazolam. Clinical examination after injection included assessment of spasticity and range of movement of joints. Motor function was videotaped. Side effects were continuously supervised. RESULTS: We performed 278 treatments; during the last year (2004) 7 treatments a month on average. 59 children were injected in lower limbs only, 14 were injected in upper limbs only, and 15 were injected in both upper and lower limbs. No serious side effects were recorded, neither from the botulinum toxin itself nor related to the injection procedure. INTERPRETATION: Assessment of indications for the use of botulinum toxin is now part of the medical follow up for children with spastic cerebral palsy. Treatment can safely be given at the paediatrics department in a local hospital. Injection in superficial muscles of the lower limbs is an easy task, while injection in small muscles and deep-seated muscles requires more special skills.