Corticobasal degeneration initially developing motor versus non-motor symptoms: a comparative clinicopathological study

• Published in: Psychogeriatrics Vol. 14; no. 3; pp. 152 - 164
• Main Authors: Ikeda, Chikako, Yokota, Osamu, Nagao, Shigeto, Ishizu, Hideki, Morisada, Yumi, Terada, Seishi, Nakashima, Yoshihiko, Akiyama, Haruhiko, Uchitomi, Yosuke
• Format: Journal Article
• Language: English
• Published: England Blackwell Publishing Ltd 01.09.2014
• Subjects: Aged; Aged, 80 and over; Basal Ganglia - pathology; Basal Ganglia Diseases - diagnosis; Basal Ganglia Diseases - pathology; Basal Ganglia Diseases - psychology; Brain - pathology; corticobasal degeneration; delirium; depression; Female; hallucination; Humans; Japan; Male; Middle Aged; Neurodegenerative Diseases - diagnosis; Neurodegenerative Diseases - pathology; Neurodegenerative Diseases - psychology; Neurologic Examination; Neuropsychological Tests; Parkinson's disease; psychosis; Severity of Illness Index; tau; Japan; tau; depression; corticobasal degeneration; hallucination; delirium; psychosis
• ISSN: 1346-3500; 1479-8301; 1479-8301
• DOI: 10.1111/psyg.12054

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.