Clinical characteristics of Japanese patients with corticobasal degeneration

Abstract

Introduction: Corticobasal degeneration (CBD) is a clinically heterogeneous neurodegenerative disorder, for which pathological investigations are essential for a definitive diagnosis. This study explored the clinical characteristics of Japanese patients with pathologically confirmed CBD.

Methods: We reviewed the data of Japanese patients with pathologically confirmed CBD who were consecutively autopsied at our institute. Clinical data were obtained from medical records and clinicopathological conferences.

Results: Of the 34 patients initially reviewed, three were excluded because of a lack of detailed clinical data. Of the remaining 31 patients, 16 were men and 15 were women. The mean ages at onset and death were 63.3 ± 6.7 (51-79) years and 69.1 ± 6.9 (54-86), respectively. The median disease duration was 6.0 (2.5-12) years. The clinical phenotypes were as follows: progressive supranuclear palsy syndrome (PSPS; n = 20, 64.5 %), probable or possible corticobasal syndrome (n = 6, 19.4 %), frontal behavioral-spatial syndrome (n = 4, 12.9 %), nonfluent/agrammatic variant of primary progressive aphasia (n = 1, 3.2 %). Furthermore, 28 (90.3 %) patients exhibited dysphagia with a median latency of 3.5 (1.0-10.0) years, and 22 (71.0 %) patients who underwent tube feeding survived longer than those who did not (P = 0.013).

Conclusions: Compared with Western populations, a high prevalence of PSPS may be a clinical characteristic of Japanese patients with CBD. Additionally, dysphagia occurs in many patients with early latency and may shorten survival. Tube feeding contributes to the prolonged survival of patients with CBD.

Keywords: Corticobasal degeneration; Corticobasal syndrome; Dysphagia; Progressive supranuclear palsy syndrome; Tube feeding.