Matthias W. Wagner MD, Abstract Co-Author: Nothing to Disclose

Eugen Boltshauser MD, Abstract Co-Author: Nothing to Disclose

Thangamadhan Bosemani MD, FRCR, Presenter: Nothing to Disclose

Thierry Huisman MD, Abstract Co-Author: Nothing to Disclose

Andrea Poretti MD, Abstract Co-Author: Nothing to Disclose

Cerebellar atrophy (CA) implies loss of cerebellar parenchyma and is a nonspecific pediatric neuroimaging finding. In the majority of cases, the vermis is more affected compared to the cerebellar hemispheres. Involvement of the brainstem as a small pons is unusual and occurs in prenatally acquired CA, neurodegenerative diseases with prenatal onset (as pontocerebellar hypoplasias) or CA as a sequela of extreme prematurity. CA may result from genetic or metabolic diseases or be acquired. In pediatric CA, neuroimaging findings are rarely diagnostic. In the majority of cases, a neuroimaging pattern-recognition approach is helpful in the evaluation of children with CA to narrow the list of differential diagnoses, plan targeted additional investigations and interpret their results.

The literature was reviewed for etiologies of pediatric CA. A pattern-recognition approach is suggested for hereditary CA considering “pure” (isolated) CA and CA “plus” (associated with other neuroimaging findings such as hypomyelination, progressive infra- or supratentorial white matter abnormalities, involvement of basal ganglia, T2-hyperintense cerebellar cortex). Additionally, checklists are provided for postnatally acquired CA, unilateral CA and pediatric diseases with ataxia as a symptom without CA on neuroimaging.

http://abstract.rsna.org/uploads/2014/14011535/14011535_fzck.pdf

Wagner, M, Boltshauser, E, Bosemani, T, Huisman, T, Poretti, A, Differential Diagnosis of Cerebellar Atrophy in Childhood: A Pattern-recognition Approach.  Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL. http://archive.rsna.org/2014/14011535.html