[Temporal profile of mental symptoms in Creutzfeldt-Jakob syndrome (author's transl)].
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Biomedical subjects
Publications and source records attributed to M Suetsugu.
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Further experimental transmission of Creutzfeldt-Jakob disease (CJD) from three patients to mice and rats was carried out successfully. The clinical signs and pathologic features of spongiform encephalopathy transmitted to animals were much the same as in previous experiments, except that distribution of the lesions in the mice differed with each inoculated material taken from the patients. These observations suggest that multiplicity of CJD agents, as in the case of scrapie agents.
The number of spines along the apical dendrites of the pyramidal neurons in the hippocampal and cingulate gyri were counted on Golgi preparations of the brains of seven cases of Down's syndrome; they did not show neuropathological senile changes. The number of spines in the middle and distal segments of the apical dendrites of Down's syndrome group were significantly fewer than in those of the control group. The number of spines in aged noncharacteristic mentally retarded cases were not decreaed compared with the controls. The diminution of spines in Down's syndrome is considered to be rather specific in this condition and is not a common finding in other types of mental retardation. It might also be one of the early changes of dendritic degeneration related to senile plaque formation.
A young male patient with adrenoleukodystrophy progressed from the frontal to the parieto-occipital region was reported. The spreading pattern of the brain lesion in this patient was confirmed by a serial examination of Technetium scintigrams and the neuropathological findings. The high uptake area of the isotope in the scintigrams seemed to represent the active demyelinating and infiltrative-reactive process in the white matter. A chemical analysis of the frozen white matter tissue of the patient revealed the existence of long chain fatty acids mor than C 22 in cholesteryl esters and gangliosides. It is suggested by this case that the demyelination process can start in the frontal white matter also in adrenoleukodystrophy as well as in Schilder's disease.
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The correlation between the appearance of the characteristic electroencephalographic abnormality, cardinal clinical manifestations, and neuropathological features was studied in four cases of Creutzfeldt-Jakob syndrome consisting of subacute spongiform encephalopathy and classical Creutzfeldt-Jakob disease. Consideration is given to the differences in the electroencephalographic findings between these two subgroups of the syndrome and on the underlying pathological mechanism.
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BACKGROUND: The intima-media thickness (IMT) of the carotid artery, as determined by ultrasonography, is useful for reflecting the extent of subclinical atherosclerosis. We investigated the relationship between IMT and the serum concentrations of small low-density lipoprotein (LDL) in diabetic patients. METHODS: The study was conducted with 27 Type 2 diabetic patients (14 males and 13 females; mean age=62.6+/-8.3 years) and 12 age-matched healthy controls. The LDL subfraction was measured using a polyacrylamide gel electrophoresis method. Vascular endothelial growth factor (VEGF) and platelet-derived growth factor (PDGF) concentrations were measured by an enzyme immunoassay. The IMT was expressed as the maximum IMT (Max-IMT) and average IMT (Ave-IMT) of the carotid artery, measured by ultrasonography. RESULTS: Both the IMT and the small LDL concentrations were significantly increased in the diabetic patients compared with the healthy participants. The IMTs were significantly correlated with small LDL concentration and small LDL/total LDL more than LDL concentrations by multivariate analysis. The IMTs were not significantly correlated with the serum VEGF or PDGF concentrations. The patients with a larger IMT had a significantly higher prevalence of hypertension or ischemic heart disease than did the patients with a normal IMT. CONCLUSIONS: The increased small LDL concentrations and small LDL/total LDL, in addition to total LDL concentrations, in Type 2 diabetic patients are closely associated with increased IMT of the carotid artery.
In a 46-year-old Japanese man with chronic psychiatric and neurologic symptoms, autopsy revealed enlarged adrenal cortical cells with trilamellar cytoplasmic inclusions and demyelinating lesions, marked in the fronto-parietal lobes and in the cerebellar hemispheres and corresponding to the pathology of adrenoleukodystrophy (ALD). Unusual gray matter lesions were found in the thalamus, cerebellar cortex, inferior olive and pontine nucleus, and the latter three lesions resembled those seen in cases of olivopontocerebellar atrophy (OPCA). These lesions are attributed to secondary changes due to disruption of the thalamo-cortical, cerebro-ponto-cerebellar and olivo-cerebellar connections.