Olivopontocerebellar Atrophy- A case report

Clinical history: A 50-years old female presented with features of ataxic gait and imbalance. No history of seizures or hypertension. No history of any long term drug intake. MRI Brain was suggested for further evaluation.

Imaging findings on MRI:

  • Diffuse cerebellar and pontine atrophy with relative sparing of the supratentorial structures.
  •  Prominent fourth ventricle , due to the cerebellar atrophy.
  • Cruciform high signal intensity on T2W images and FLAIR sequence in the pons consistent with “Hot Cross Bun” sign – reflects degeneration of transverse pontocerebellar fibres.
  •  Severe thinning of both middle cerebellar peduncles which also showed increased signal intensity on T2WI.

Final Diagnosis: Olivopontocerebellar Atrophy.