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Although many entities have been established within the broad spectrum of Parkinson disease (PD) and atypical parkinsonisms, they are often difficult to differentiate. To clarify the current clinical diagnostic conditions and problems in PD and atypical parkinsonisms, we analyzed volumes of the Annuals of the Pathological Autopsy Cases in Japan. Among 130 105 autopsies conducted from 2007 to 2016 throughout Japan, patients were included in the study if they had been either clinically or pathologically diagnosed with PD, multiple system atrophy (MSA), progressive supranuclear palsy (PSP), or corticobasal degeneration (CBD). Autopsy rates were 6.4% for clinically diagnosed PD, 34.1% for MSA, 16.3% for PSP, and 17.4% for CBD. The specificities and sensitivities of clinical diagnoses were 88.0% and 82.0% for PD, 95.2% and 86.0% for MSA, 82.7% and 73.2% for PSP, and 55.4% and 57.7% for CBD, respectively. Clinical diagnoses had relatively high accuracy, but low autopsy rates are of concern. Many patients with rarer disorders were clinically misdiagnosed with PD, a more common disorder. Autopsy rates, irrespective of specific disorders, should be increased to detect rare diseases. Increasing autopsy rates will increase the available clinical information regarding pathologically confirmed patients and contribute to more accurate clinical diagnoses. [ABSTRACT FROM AUTHOR]

Background Clinical presentations of pathologically confirmed corticobasal degeneration ( CBD) vary, and the heterogeneity makes its clinical diagnosis difficult, especially when a patient lacks any motor disturbance in the early stage. Methods We compared clinical and pathological features of four pathologically confirmed CBD cases that initially developed non-motor symptoms, including behavioural and psychiatric symptoms but without motor disturbance ( CBD- NM), and five CBD cases that initially developed parkinsonism and/or falls ( CBD- M). The age range at death for the CBD- NM and CBD- M subjects (58-85 years vs 45-67 years) and the range of disease duration (2-18 years vs 2-6 years) did not significantly differ between the groups. Results Prominent symptoms in the early stage of CBD- NM cases included self-centred behaviours such as frontotemporal dementia ( n = 1), apathy with and without auditory hallucination ( n = 2), and aggressive behaviours with delusion and visual hallucination ( n = 1). Among the four CBD- NM cases, only one developed asymmetric motor disturbance, and two could walk without support throughout the course. Final clinical diagnoses of the CBD- NM cases were frontotemporal dementia ( n = 2), senile psychosis with delirium ( n = 1), and schizophrenia ( n = 1). Neuronal loss was significantly less severe in the subthalamic nucleus and substantia nigra in the CBD- NM cases than in the CBD- M cases. The severity of tau pathology in all regions examined was comparable in the two groups. Conclusion CBD cases that initially develop psychiatric and behavioural changes without motor symptoms may have less severe degenerative changes in the subthalamic nucleus and substantia nigra, and some CBD cases can lack motor disturbance not only in the early stage but also in the last stage of the course. [ABSTRACT FROM AUTHOR]