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.
Cerebellar atrophy
Non-SOL
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Cerebellum
Cerebellar atrophy is observed, but there is no abnormal signal in transverse pontine fibers.
Loss of hypointensity in Dentate nucleus
Non-SOL
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Dentate nucleus
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.