[Neuropathology of Cerebellar Cortical Atrophy]

Cerebellar cortical atrophy (CCA) is a neurodegenerative disease characterized by the loss of Purkinje cells, frequently associated with atrophy of the inferior olivary nucleus. The diagnosis of CCA requires a pathologic assessment; however, this term has also been used in clinical practice as a diagnosis of exclusion for sporadic, adult-onset, and progressive ataxia. For the clinical diagnosis of CCA, diagnostic criteria for idiopathic cerebellar ataxia (IDCA) have been proposed. We herein describe two patients with a pathologic diagnosis of CCA. The first patient was clinically suspected to have Creutzfeldt-Jakob disease due to rapid progressive dementia and ataxia. The second patient was clinically diagnosed with progressive supranuclear palsy based on imbalance, frequent falls, and vertical gaze palsy. The se cases suggest that the clinical presentation of CCA is heterogeneous, and CCA does not always meet the criteria for IDCA. Therefore, the term CCA should be used solely for a pathologic diagnosis.