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抄録 上矢状洞血栓症に頭蓋外の静脈血栓症が合併してくることは稀ならず経験されることである。著者らは最近,42歳の男性において,上矢状洞血栓症に頭蓋外の多発性動・静脈閉塞,多発性脂肪腫そして高脂血症を合併した1例を経験した。本症例においては高脂血症,すなわちLDL (low density lipoprotein)およびVLDL (very low density lipoprotein)の上昇による血液凝固性の亢進により,多発性の動脈系ならびに静脈系の閉塞が招来されたものと考えられた。一方,多発性脂肪腫は脂肪代謝異常が関与している可能性もあるものと思われた。
A 42-year-old man was admitted to the Saisei-kai Fukuoka General Hospital on March 22, 1985 with complaints of nausea, vomiting and headache which had continued for twelve days. On physical examinations, multiple subcutaneous nodules were noted in the chest, abdomen and extremities with dilated veins in the chest wall. Furthermore, the discoloration of the skin attributable to the venous occlusion was noted in the left foot. Neurological examinations failed to reveal any abnormalites except for moderate choked disc in bilateral ocular fundi. Spinal puncture revealed CSF pressure as high as 350 mmH2O with slight increase in cell numbers. CT demonstrated small intracerebral he-matomas bilaterally in occipital lobes with evi-dence of "empty delta" sign in the superior sagit-tal sinus. On angiographical findings, the superior sagittal sinus was not demonstrated in venous phase and the blood was drained mainly through the paravertebral plexus and the pterygoid plexus without any abnomalities in arterial and capillary phases. Serulological examination revealed a hypercoagulability and hyperlipidemia especially in LDL (Low Density Lipoprotein) and VLDL (Very Low Density Lipoprotein).
On etiological considerations, all of these ab-normalities seemed to be derived from the hyper-lipidemia. Furthermore, multiple symmetrical lipomatosis was defined as a "lipoprotein storage disease" in which laboratory examinations revealed hyperlipidemia especially in LDL and in some subfraction of HDL. Although not a typical case, there must be some possibilities that the lipomatosis of this case is secondary to disorder of lipid meta-bolisms. In conclusion, all of various pathological conditions observed in this case were considered to be due to the disorder of lipid metabolism.
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