Cortical cerebellar atrophy (CCA)

Neurodegenerative diseases

General description

Cortical cerebellar atrophy (CCA) is a type of spinocerebellar degeneration showing pure cerebellar ataxia. Typical onset is late 50's, and there is no cognitive impairment or sensory impairment, and vision and hearing are preserved.

Neuropathologically, the main pathological change is degeneration and loss of cerebellar cortical Purkinje cells, often accompanied by secondary degeneration of the inferior olivary nuclei.

Non-Space occupying lesion

Cerebellar atrophy

Anatomical regions
  • Cerebellum
Symmetric
Bilateral
Morphology
Atrophy

Cerebellar atrophy is observed, but there is no abnormal signal in transverse pontine fibers.

Non-Space occupying lesion

Loss of hypointensity in Dentate nucleus

Anatomical regions
  • Dentate nucleus
Symmetric
Bilateral
T2WI
Hyperintensity

Physiologic low signal in the cerebellar dentate nucleus disappears on T2WI, and the signal is often isointensity to that of the brainstem and middle cerebellar peduncle.