BRAIN and NERVE Shinkei Kenkyu no Shinpo Volume 67, Issue 4 (April 2015)

Criteria for the Diagnosis of Corticobasal Degeneration Takayoshi Shimohata 1 , Ikuko Aiba 2 , Masatoyo Nishizawa 1 1Department of Neurology, Brain Research Institute, Niigata University 2Department of Neurology, National Hospital Organization Higashinagoya National Hospital Keyword: 大脳皮質基底核変性症 , 大脳皮質基底核症候群 , Armstrong基準 , タウ , corticobasal degeneration , corticobasal syndrome , Armstrong's criteria , tau pp.513-523
Published Date 2015/4/1
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Corticobasal degeneration (CBD) is a distinct neurodegenerative disorder characterized by widespread neuronal and glial accumulation of abnormally phosphorylated tau protein. Patients with CBD often present with corticobasal syndrome (CBS) showing impairment of the motor system, cognition, or both. Several studies demonstrate that they may also present with progressive supranuclear palsy syndrome (PSPS), aphasia, Alzheimer disease-like dementia, or behavioral changes, suggesting that CBS is merely one of the presenting phenotypes of CBD. Accurate diagnosis is important for future clinical trials using drugs aimed at modifying the underlying tau pathology. Although previous CBD diagnostic criteria reflected only CBS, Armstrong et al. proposed new diagnostic criteria for CBD in 2013 (Armstrong's criteria). The new criteria include 4 CBD phenotypes, including CBS, frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and PSPS. These phenotypes were combined to create 2 sets of criteria: specific clinical research criteria for probable CBD (cr-CBD) and broader criteria for possible CBD that are more inclusive but have a higher probability of detecting other tau-based pathologies (p-CBD). However, two recent studies revealed that the sensitivity and specificity of these criteria were insufficient. Further refinement of the criteria is needed via biomarker research with prospective study designs.

(Received August 19, 2014; Accepted December 26, 2014: Published April 1, 2015)

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BRAIN and NERVE-神経研究の進歩
67巻4号 (2015年4月)
電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院