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

Title

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

Creator

Stromme P; Kanavin OJ; Abdelnoor M; Woldseth B; Rootwelt T; Diderichsen J; Bjurulf B; Sommer F; Magnus P

Publisher

Bmc Pediatrics

Date

2007

Description

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 (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.
2007

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Pages

25-25

Volume

7

Citation

Stromme P; Kanavin OJ; Abdelnoor M; Woldseth B; Rootwelt T; Diderichsen J; Bjurulf B; Sommer F; Magnus P, “Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study,” Pediatric Palliative Care Library, accessed September 21, 2021, https://pedpalascnetlibrary.omeka.net/items/show/14063.

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