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Neuropediatrics 2015; 46(06): 359-370
DOI: 10.1055/s-0035-1564620
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Differential Diagnosis of Cerebellar Atrophy in Childhood: An Update

Andrea Poretti
1   Department of Pediatric Neurology, University Children's Hospital of Zurich, Switzerland
2   Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Nicole I. Wolf
3   Department of Child Neurology, VU University Medical Center and Neuroscience Campus, Amsterdam, The Netherlands
,
Eugen Boltshauser
1   Department of Pediatric Neurology, University Children's Hospital of Zurich, Switzerland
› Author Affiliations
Further Information

Publication History

16 June 2015

12 August 2015

Publication Date:
07 October 2015 (online)

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Abstract

Cerebellar atrophy (CA) is a relatively common, but nonspecific finding in pediatric neurology and neuroradiology. Here, we provide an update of checklists for postnatally acquired CA, unilateral CA, and hereditary CA. In addition, we include a list of disorders with ataxia as a symptom, but without CA. These checklists may help the evaluation of differential diagnosis and planning of additional investigations. For diseases associated with hereditary CA, we provide an updated version of our neuroimaging-based pattern-recognition approach that classify CA as isolated (“pure”) or associated (“plus”) with other neuroimaging findings including hypomyelination, progressive white matter abnormalities, signal changes of the dentate nucleus, cerebellar cortex T2-hyperintensity, and basal ganglia involvement. Finally, we discuss some rules with their exceptions related to pediatric CA, discrepancies between clinical and neuroimaging course, and the difficulties to differentiate CA from cerebellar hypoplasia.

Keywords

cerebellum - cerebellar atrophy - children - neuroimaging - pattern-recognition

Supplementary Data

Supplementary data (Figs. S1 to S6, and Table S1) are available at: www.thieme-connect.com/products/ejournals/html/10.1055/s-0035-1564620 .


Note


Dr. Nicole I. Wolf is an Associate Editor and Dr. Eugen Boltshauser is the Editor-in-Chief of Neuropediatrics.


Supplementary Material

 

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