Olivopontocerebellar atrophy (OPCA):20 years old female with progressive cerebellar ataxia
Axial T2W MRI
Axial PDW MRI
Sagittal T1W MRI
"The T2W spin-echo axial section shows atrophy of the pons and middle cerebellar peduncles with enlargement of the prepontine and ponto-cerebellar cisterns and
moderate atrophy of the cerebellar hemispheres. The PDW image shows slight signal hyperintensity of the transverse pontine fibers (arrows), sparing the pyramidal
tracts. The midline sagittal T1W spin-echo image
(far right) shows flattening of the pons and atrophy of the cerebellar vermis.
MRI findings, combined with the clinical symptoms, are highly suggestive of olivopontocerebellar atrophy.
Olivopontocerebellar atrophy (OPCA) is a degenerative disease characterized by atrophy of the pons, middle cerebellar peduncles and cerebellar
It can exist as an inherited form, transmitted in an autosomal dominant manner or as a sporadic form. Although the mean age of onset is
during the fourth decade, the condition can be encountered in children. Since the main clinical symptoms, ataxia, dysmetria, dysartria and
ophtalmoparesis are in common with other cerebellar degenerative and non degenerative diseases, MRI is essential for
diagnosis" Images and commentary from: Section of Neuroradiology;
Department of Radiology;University of Brescia;Policlinico Satellite;
Spedali Civili; 25123 BRESCIA; ITALY--by Roberto Gasparotti,