BRAIN and NERVE Shinkei Kenkyu no Shinpo Volume 67, Issue 10 (October 2015)

A Clinical and Radiological Comparison of Adult- and Childhood-onset Tuberous Sclerosis Complex Shinichi Takeshima 1 , Naoyuki Hara 1,3 , Takahiro Himeno 1,4 , Satoshi Kubo 1,5 , Kazuhiro Takamatsu 1 , Hiromitsu Kobayashi 2 , Akio Tanaka 2 , Masaru Kuriyama 1 1Department of Neurology, Brain Attack Center Ota Memorial Hospital 2Department of Radiology, Brain Attack Center Ota Memorial Hospital 3Department of Neurology, Hiroshima City Hiroshima Citizens Hospital 4Department of Neurology, Oita Red Cross Hospital 5Hiroshima University Clinical Neuroscience and Therapeutics Keyword: 成人結節性硬化症 , 3主徴 , 脳画像 , 皮質結節 , 脳室上衣下結節 , adult-onset tuberous sclerosis complex , trias , brain CT/MRI , cortical tuber , subependymal nodule pp.1255-1260
Published Date 2015/10/1
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In this study, we assessed 11 patients who were definitively diagnosed with tuberous sclerosis complex (TSC) based on the accepted clinical diagnostic criteria (Roach, 1998). We compared the clinical and radiological findings of six adult-onset TSC cases (group A) and five child-onset TSC cases (group B). The prevalence rates of generalized tonic-clonic convulsions at onset, mental retardation, facial angiofibroma, and epilepsy were lower in group A than in B group. The number of cortical tubers and subependymal nodules on brain magnetic resonance (MR) and computed tomography (CT) images were also lower in group A. The number of cerebral white matter radial migration lines was similar in both groups. Cortical tubers were most frequently observed in the frontal lobe in both groups. The number of cortical tubers and subependymal nodules did not correlate with the presence of epilepsy or mental retardation. Extra-brain lesions in lung, kidney, and bone were recognized in both groups, and no specific lesions were noted in group A.

(Received May 11, 2015; Accepted June 22, 2015; Published October 1, 2015)

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BRAIN and NERVE-神経研究の進歩
67巻10号 (2015年10月)
電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院