脳脂質症の電子顕微鏡的検索

DOI Web Site

書誌事項

タイトル別名
  • ULTRASTRUCTURAL FINDINGS IN FOUR
  • ノウ シシツショウ ノ デンシ ケンビキョウテキ ケンサク フウニュウタイ ケ
  • AUTOPSY CASES OF CERBRAL LIPIDOSIS
  • 封入体形成についての考察

この論文をさがす

抄録

Case 1 was a 4-year-old boy with macular cherry red spots, myoclonus and pyramidal tract signs. The clinical diagnosis was Tay-Sachs disease. Tay-Sachs ganglioside was increased in his brain obtained at autopsy.<BR>Case 2 was a 7-year-old girl with onset at age of 3 years with epileptic seizures. Macular degeneration, pyramidal and cerebellar signs followed. The clinical diagnosis was late infantile form of amaurotic family idiocy. GM1-ganglioside was increased in the brain at autopsy.<BR>Case 3 was a 27-year-old man with macular degeneration, myoclonus, convulsive seizures, cerebellar and pyramidal signs. The clinical diagnosis was adult type of amaurotic family idiocy (Kufs disease). In the brain at autopsy, ganglioside showed a normal pattern.<BR>Case 4 was a 33-year-old man with spastic paraplegia and progressive dementia. Neuropathologically, atypical leucodystrophy was diagnosed by a mild increase of cerebroside-sulfuric ester. Aryl sulphatase A activity was 50% of control in the brain at autopsy.<BR>Electron microscopy showed in Case 1 that the neurons were swollen by membranous cytoplasmic bodies (MCB) in cytoplasm. There were MCB, zebra bodies and some other organelles in a limiting membrane. It seemed that these formed autophagolysosome, some of which were similar to the so-called curvilinear bodies (CLB).<BR>In Case 2, there were several MCB and CLB enclosed with a limiting membrane in neurons from the frontal lobe. filamentous or reticular substance filled the cavities of dilated cisternae of the rough endoplasmic reticulum (ER). Besides dilated ER, CLB, small MCB and zebra bodies were observed in neurons from the occipital lobe. In Purkinje cells, there were many vesicles, cored vesicles and small MCB near cisternae of rough ER grown in the cytoplasm. Some of them were enclosed with a limiting membrane, being suggestive of their transformation into CLB.<BR>In Case 3, neurons at ammon's horn were swollen with finger print-like patterns. Glial cells from this region and neurons from the occipital lobe contained multilamellar cytosomes (MLC) with a limiting membrane.<BR>In Case 4, there were large osmiophilic myelin figures surrounded by a limiting membrane among myelin sheaths in the white matter. Some glial cells contained myelin figures in cisternae of ER, some of which were changed into large inclusions with osmiophilic myelin figures.<BR>These inclusions were generally regarded as residual bodies, but in our cases the origin of inclusion bodies were considered to be swollen cisternae of ER, many vesicles, cored vesicles, small MCB near the ER and myelin figures in the cisternae of ER.

収録刊行物

詳細情報 詳細情報について

問題の指摘

ページトップへ