Daniel E. Lumsden , Apostolos Papandreou , Nicholas M. Allen , Jean-Piere Lin
{"title":"The utility of creatine kinase in status dystonicus and pre-status dystonicus","authors":"Daniel E. Lumsden , Apostolos Papandreou , Nicholas M. Allen , Jean-Piere Lin","doi":"10.1016/j.ejpn.2025.05.013","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div>Individuals with dystonia may experience acute exacerbations of symptoms.</div></div><div><h3>Objectives</h3><div>We aimed to explore the role of serum creatinine kinase (CK) levels as a biomarker for dystonia severity during episodes of exacerbation.</div></div><div><h3>Methods</h3><div>A retrospective review of admissions to a paediatric tertiary centre due to Status Dystonicus over a 5-year period. A comprehensive scoping review of the published literature for SD and pre-SD was also undertaken.</div></div><div><h3>Results</h3><div>In total 58 admissions for 45 patients were identified. Dystonia Severity Action Plan (DSAP) was Grade 3 (pre-SD) for 41/58 admissions and Grade 4–5 (SD) for 17 admissions. Length of admission was significantly longer for SD (P < 0.005), with poorer outcomes (Fishers Exact test P < 0.001). CK levels were measured in 24/41 episodes of pre-SD, and 16/17 episodes of SD. Median peak CK levels were higher (729 IU/L) in the SD compared to pre-SD group (179.5 IU/L) (p = 0.009). For patients with SD, serial CK measurements tracked dystonia severity over time. Literature review identified 201 episodes of SD in 190 subjects. Note was made of CK measurement in 92/201 (45.8 %) episodes: pre-SD (DSAP 3) in 8 and SD in 84 [DSAP 4 (n = 30), and DSAP 5 (n = 54)] respectively, with a numerical value provided in in 73/90 episodes/cases. Median CK value was 4066 IU/L (884–22,105, 25th to 75th Centile). In the literature review, for 11 episodes serial CK measures were shown to correlate with severity of dystonic symptoms.</div></div><div><h3>Conclusions</h3><div>serum CK levels represent a potentially useful biomarker for dystonia severity that differs between pre-SD and SD, and provide a measure to track dystonia severity at an individual patient basis.</div></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"57 ","pages":"Pages 57-63"},"PeriodicalIF":2.3000,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Paediatric Neurology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1090379825000996","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background
Individuals with dystonia may experience acute exacerbations of symptoms.
Objectives
We aimed to explore the role of serum creatinine kinase (CK) levels as a biomarker for dystonia severity during episodes of exacerbation.
Methods
A retrospective review of admissions to a paediatric tertiary centre due to Status Dystonicus over a 5-year period. A comprehensive scoping review of the published literature for SD and pre-SD was also undertaken.
Results
In total 58 admissions for 45 patients were identified. Dystonia Severity Action Plan (DSAP) was Grade 3 (pre-SD) for 41/58 admissions and Grade 4–5 (SD) for 17 admissions. Length of admission was significantly longer for SD (P < 0.005), with poorer outcomes (Fishers Exact test P < 0.001). CK levels were measured in 24/41 episodes of pre-SD, and 16/17 episodes of SD. Median peak CK levels were higher (729 IU/L) in the SD compared to pre-SD group (179.5 IU/L) (p = 0.009). For patients with SD, serial CK measurements tracked dystonia severity over time. Literature review identified 201 episodes of SD in 190 subjects. Note was made of CK measurement in 92/201 (45.8 %) episodes: pre-SD (DSAP 3) in 8 and SD in 84 [DSAP 4 (n = 30), and DSAP 5 (n = 54)] respectively, with a numerical value provided in in 73/90 episodes/cases. Median CK value was 4066 IU/L (884–22,105, 25th to 75th Centile). In the literature review, for 11 episodes serial CK measures were shown to correlate with severity of dystonic symptoms.
Conclusions
serum CK levels represent a potentially useful biomarker for dystonia severity that differs between pre-SD and SD, and provide a measure to track dystonia severity at an individual patient basis.
期刊介绍:
The European Journal of Paediatric Neurology is the Official Journal of the European Paediatric Neurology Society, successor to the long-established European Federation of Child Neurology Societies.
Under the guidance of a prestigious International editorial board, this multi-disciplinary journal publishes exciting clinical and experimental research in this rapidly expanding field. High quality papers written by leading experts encompass all the major diseases including epilepsy, movement disorders, neuromuscular disorders, neurodegenerative disorders and intellectual disability.
Other exciting highlights include articles on brain imaging and neonatal neurology, and the publication of regularly updated tables relating to the main groups of disorders.