摘要:

This paper is a histochemical and ultrastructural study of a case of mucopolysaccharidosis with certain clinical and morphological peculiarities. The patient was a 3-year-old girl with a congenital cataract of the right eye; the onset at the age of one was marked by epileptic seizures, accentuated hypotonia, and progressive psychic degradation. Optical microscopy of a cerebral biopsy specimen revealed ballooning of the neurons, swollen dendrites, vacuolized cytoplasm, and excentric nucleus. PAS staining showed an intensely positive material in the grey matter. Ultrastructural aspects were the presence of lamellar bodies, granulo-membrano-vacuolar bodies, low electron-dense vacuoles, and a special aspect of membranous body conglomerates with a peculiar orientation of the membranes. The presence of nuclear bodies in the astrocytes is described. Listing of this case among the existing classification types is discussed.

ISSN:0014-3022    

DOI:10.1159/000114292

出版商:S. Karger AG    

年代:1973

数据来源: Karger

摘要:

The content and composition of cholesteryl esters in the apparently normal cerebral white matter of multiple sclerosis (MS) were investigated. In spite of only negligibly increased contents, the MS cases showed significantly altered cholesteryl ester composition, i. e. decreased proportions of saturated and monounsaturated cholesteryl esters and increased proportions of the polyunsaturated ones. It is concluded that this abnormality might be explained by the presence of small demyelinating lesions escaped identification during isolation of the apparently normal white matter.

ISSN:0014-3022    

DOI:10.1159/000114293

出版商:S. Karger AG    

年代:1973

数据来源: Karger

摘要:

In our studies, we tried to decide whether several enzymes of cortical grey and subcortical white matter of rabbit brain might be influenced differently by triethyltin (TET) and by other organometallic compounds. We performed neurochemical studies on rough mitochondrial and microsomal fractions. Despite differences in distribution and in specific activities, the inhibition patterns of the ATPases, nucleotidases and phosphodiesterases of grey and white matter in vitro were similar. Mitochondrial Mg2+-ATPase was inhibited more strongly than Na+,K+-ATPase. The activity of 3’,5’-cyclic AMP-phosphodiesterase was inhibited as strongly as the Na+,K+-ATPase activity. The activity of myelin-specific 2’,3’-cyclic AMP-phosphodiesterase was not influenced whereas the 5’-nucleotidase activity was stimulated by the organometallic compounds. The content of TET in the white matter could not be related with the TET edema and was somewhat lower than in the grey matter. The results obtained point towards a general conformational change in membrane structures by organometallic compounds, which might result after in vivo administration with TET in brain edema in the whi

ISSN:0014-3022    

DOI:10.1159/000114294

出版商:S. Karger AG    

年代:1973

数据来源: Karger

摘要:

Four cases who developed muscular atrophy 44–71 years after affection with acute poliomyelitis are reported. This disorder distinguishes itself from the classical progressive spinal muscular atrophy through its peculiar asymmetrical involvement and its benign course. A review of the literature shows that the cases reported do not form a homogenous group, because some cases have been shown to involve primarily the anterior spinal nerve roots while other cases involve the anterior horn cell

ISSN:0014-3022    

DOI:10.1159/000114295

出版商:S. Karger AG    

年代:1973

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000114296

出版商:S. Karger AG    

年代:1973

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000114297

出版商:S. Karger AG    

年代:1973

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000114298

出版商:S. Karger AG    

年代:1973

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000221040

出版商:S. Karger AG    

年代:1901

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000221041

出版商:S. Karger AG    

年代:1901

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000221042

出版商:S. Karger AG    

年代:1901

数据来源: Karger