イトウ　ススム   伊藤　進    所属   医学部 医学科（東京女子医科大学病院）    職種   助教
論文種別	原著
言語種別	英語
査読の有無	査読なし
表題	Association between diffuse cerebral MRI lesions and the occurrence and intractableness of West syndrome in tuberous sclerosis complex.
掲載誌名	正式名：Epilepsy & behavior : E&B 略　 称：Epilepsy Behav ISSNコード：15255069/15255050
掲載区分	国外
出版社	ELSEVER
巻・号・頁	103(Pt A),pp.106535
著者・共著者	OKANISHI Tohru, FUJIMOTO Ayataka, KANAI Sotaro, SAKUMA Satoru, ITO Susumu, OKANARI Kazuo, NISHIMURA Mitsuyo, MASUI Takayuki, ENOKI Hideo
発行年月	2020/02
概要	OBJECTIVE:We aimed to clarify the association between magnetic resonance imaging (MRI)-lesion patterns, including cortices and white matters, and the development, occurrence, and intractableness of West syndrome in patients with tuberous sclerosis complex (TSC), using visual analysis.METHODS:We collected data for 44 patients with TSC who had undergone brain MRI and developmental evaluation after the ages of 2 and 3 years, respectively. Fluid-attenuated inversion recovery (FLAIR) and T1-weighted images were used to analyze the number of cyst-like tubers, the number of cyst-like subcortical lesions, and the presence of diffuse lesions involving the cortices and white matter.RESULTS:Developmental delays were observed in 28 patients. Nineteen patients had a history of West syndrome. Cyst-like tubers (range: 1-10), cyst-like subcortical lesions (range: 1-4), and diffuse lesions (range: 1-6 areas) were observed in 15, 9, and 14 patients, respectively. In the univariate analyses, all MRI findings were associated with development and/or history of West syndrome. However, in the multivariate analyses, only the diffuse lesion was associated with severe development (p = 0.003) and history of West syndrome (p = 0.012). In the subanalysis of patients with West syndrome, the diffuse lesions were also associated with pharmacological intractableness. Patients with diffuse lesions had a history of West syndrome with sensitivity of 68% and specificity of 96%. Patients with two or more areas of diffuse lesions had history of pharmacologically intractable West syndrome with sensitivity of 89% and specificity of 91%.CONCLUSIONS:Diffuse lesions may help to predict the poor neurological outcomes in patients with TSC.
DOI	10.1016/j.yebeh.2019.106535
PMID	31645317