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 / Foregin | Foregin |
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 |