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Evaluation of the child with acute ataxia: a systematic review
Harry T Whelan1, Sumit Verma, Yan Guo
1Department of Neurology, Medical College of Wisconsin, Milwaukee, WI 53226, USA. hwhelan@mcw.edu
Insights
Evaluating acute ataxia in children requires careful test selection. Toxicology screens and neuroimaging are recommended, while cerebrospinal fluid analysis is useful only when clinically indicated for diagnosing pediatric ataxia.
Area of Science:
- Pediatric Neurology
- Diagnostic Imaging
- Clinical Toxicology
Background:
- Acute ataxia in children presents a diagnostic challenge.
- Determining the appropriate timing and scope of initial screening tests is crucial.
Purpose of the Study:
- To review the evidence on the diagnostic yield of commonly ordered tests for acute pediatric ataxia.
- To guide clinicians in selecting effective diagnostic strategies.
Main Methods:
- Systematic literature review of studies evaluating diagnostic tests for acute ataxia in children.
- Analysis of reported frequencies of abnormalities from CT, MRI, lumbar puncture, EEG, and toxicology screens.
Main Results:
- Toxicology screens (49%) and lumbar punctures (43%) showed the highest abnormality rates, though often nondiagnostic.
- Neuroimaging (CT ~2.5%, MRI ~5%) and EEG (42%) had lower abnormality yields.
- Limited evidence supports routine autoimmune or inborn error of metabolism screening; urine catecholamines are recommended for ataxia with opsoclonus myoclonus.
Conclusions:
- Toxicology screening and neuroimaging are recommended for all children with acute ataxia.
- Cerebrospinal fluid analysis has limited utility unless clinically indicated.
- Further research is needed for autoimmune disorders and inborn errors of metabolism in pediatric ataxia.
Abstract:
Evaluation of acute ataxia in a child poses a dilemma for the clinician in determining the extent and timing of initial screening tests. This article reviews the evidence concerning the diagnostic yield of commonly ordered tests in evaluating the child with acute ataxia. The literature revealed the following frequencies of laboratory screening abnormalities in children with acute ataxia: CT (∼2.5%), MRI (∼5%), lumbar puncture (43%), EEG (42%), and toxicology (49%). In most studies, abnormalities detected by these screening tests were nondiagnostic. There are insufficient data to assess yields of testing for autoimmune disorders or inborn errors of metabolism. A toxicology screen should be considered in all children presenting with acute ataxia. Neuroimaging should be considered in all children with new onset ataxia. Cerebrospinal fluid analysis has limited diagnostic specificity unless clinically indicated. Studies to examine neurophysiology testing did have sufficient evidence to support their use. There is insufficient evidence to establish a role for autoantibody testing or for routine screening for inborn error of metabolism in children presenting with acute ataxia. Finally, in a child presenting with ataxia and opsoclonus myoclonus, urine catecholamine testing for occult neuroblastoma is recommended. Nuclear scan may be considered, however, there is insufficient evidence for additional body imaging.
