伊藤 進
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Assistant Professor
Article types Original article
Language English
Peer review Non peer reviewed
Title Association between diffuse cerebral MRI lesions and the occurrence and intractableness of West syndrome in tuberous sclerosis complex.
Journal Formal name:Epilepsy & behavior : E&B
Abbreviation:Epilepsy Behav
ISSN code:15255069/15255050
Domestic / ForeginForegin
Publisher ELSEVER
Volume, Issue, Page 103(Pt A),pp.106535
Author and coauthor OKANISHI Tohru, FUJIMOTO Ayataka, KANAI Sotaro, SAKUMA Satoru, ITO Susumu, OKANARI Kazuo, NISHIMURA Mitsuyo, MASUI Takayuki, ENOKI Hideo
Publication date 2020/02
Summary 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