Olivopontocerebellar atrophy in a young patient: A case report with radiologic findings

Abstract

Background

Olivopontocerebellar atrophy (OPCA) is a term that encompasses an increasing number of neurodegenerative syndromes that lack a unique pathogenesis but are known to result from a disruption in the relationship between the cerebellar cortex, dentate nucleus, and the inferior olivary nuclei. OPCA can be difficult to diagnose on clinical grounds only and recent data suggests that OPCA affects 3–5 individuals in every 100,000 and may constitute almost 6% of patients diagnosed with atypical parkinsonism.

Case report

Herein, we report the case of a 36-year-old female that initially developed dysarthria and gait abnormalities and subsequently presented with progressive neurologic signs and symptoms. A routine MRI of the brain was performed and high-resolution images were taken through the entire posterior fossa which showed prominent widening of the CSF spaces in the posterior fossa, cerebellar atrophy and reduction in the size of the pons, consistent with a diagnosis of OPCA.

Conclusion

Olivopontocerebellar atrophy is challenging condition to diagnose on a clinical basis, and hence requires corroboration of clinical and physical examination findings with radiologic findings. MRI of the brain is the gold standard in assessing OPCA and is an extremely valuable imaging modality in distinguishing OPCA from other neurological disorders and allows for the precise determination of OPCA subtypes.