結節性硬化症の1剖検例—特に母斑症と褐色脂肪出現との関連についての1新知見

AN AUTOPSY CASE OF TUBEROUS SCLEROSIS, WITH SPECIAL CONSIDERATION ON BROWN FAT PHANEROSIS IN PHACOMATOSIS

　結節性硬化症（以下Ts）は多彩な病理解剖学的所見を呈する疾患であり，脳・心・腎等に見られる病変についての研究は甚だ多い。併しながら，著者らが最近経験したTs症例において見出された褐色脂肪の出現という所見に関しては未だ内外の文献に報告はなく，しかもこの所見はTsのみならず，母斑症一般の病因論に関して示唆に富むものと考えられるので，本論文では脂肪所見に重点をおいて報告したいと思う。

A girl aged 7 years, with symptoms of men-tal retardation, epileptic seizures and ade-noma sebaceum, was clinically diagnosed as tuberous sclerosis. Her main autopsy find-ings were as following:

1) Ca. 20 upto walnut-sized firm nodular lesions of the cerebral cortex (appearance of monstrous glial and nerve cells). Many heterotopic lesions of the cerebral medulla. A few pea-sized tumors of the lateral ventricles (spongioblastomas). 2) A few pea-sized myo-cardial tumors (rhabdomyomas). 3) Ca. 20 upto pinhead-sized nodules of the kindneys (lipo-angio-myo-mesenchYmomas). 4) A hen's-egg-sized firm subcutaneous mass of the left parietal region (fibroma durum). 5) Adenoma sebaceum of the face. 6) Cystic degeneration of the ovarii. 7) Parenchyma-tous thymus (30g in weight) and hypertro-phied lymphatic apparatus. 8) Widely dis-tributed appearance of brown fat tissue in the depth of body.

The item 8 is a remarkable finding, which has never been described in the literature of tuberous sclerosis, and, therefore, this paperwas concerned with this matter especially.