[Diagnostic problems in a case of adrenoleukodystrophy].
A case of adrenoleukodystrophy is reported in which the diagnosis was possible only after electron microscopic examination in view of absence of clinical signs of adrenocortical failure.
Biomedical subjects
Publications and source records attributed to M Wender.
A case of adrenoleukodystrophy is reported in which the diagnosis was possible only after electron microscopic examination in view of absence of clinical signs of adrenocortical failure.
A group of 16 patients with clinically certain multiple sclerosis with a remitting course were subjected to alternating treatment with medium doses of prednisone (Encorton, Polfa) and Decaris (Richter). The cerebrospinal fluid and plasma were studied before the beginning of the treatment and after one course of alternating treatment, determining the levels of IgG and albumins, and the IgG index which is an indicator of IgG synthesis within the cerebrospinal fluid spaces. It was observed that this treatment led to a reduction of IgG synthesis but the effect of the treatment was less pronounced than that of large doses of prednisone found in the previous study.
A group of 18 patients with clinically certain diagnosis of multiple sclerosis were treated alternate with prednisone and Decaris (levamisole). The results were compared with those in a control group of 18 cases with similar initial clinical parameters treated exclusively with prednisone during exacerbations of the disease. Alternating treatment with the mild immunosuppressant, prednisone, and with the immunomodulating drug Decaris caused a statistically significant reduction in the number of exacerbations of multiple sclerosis, however, the effect of this treatment on the natural history of the disease could not have been established in view of the short period of follow-up.
Ten cases of the late-childhood form of neuronal ceroidolipofuscinosis are presented stressing particularly the great uniformity of the clinical pattern in various cases. Epileptic fits were the presenting sign, followed by progressive dementia, motor disturbances and visual disturbances. The appearance of these clinical signs associated with diffuse, high-grade dysrhythmia in EEG and progressing cerebral atrophy in CT images are indications to rectal mucosa biopsy or biopsy examination of the appendix, skin or conjunctiva. Histochemical and ultrastructural examinations of biopsy material make possible intravital diagnosis of neuronal ceroidolipofuscinosis.
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In five provinces of Western Poland the incidence and prevalence of multiple sclerosis was analyzed in patients living at the place of birth and in patients who had changed their place of residence in these provinces. The results showed that the occurrence of multiple sclerosis in geographic foci is more evident if all patients living in a given area are considered, both those born there and those who had come from other localities.
The incidence of malignant neoplasma was determined in patients with multiple sclerosis in epidemiological studies in 717 patients. No significant difference was found from the general population aged over 15 years. This seems to suggest that immunosuppression resulting from corticotherapy has no effect increasing the risk of malignant neoplasm development in multiple sclerosis.
A trial was made of finding a correlation between the incidence and prevalence of multiple sclerosis and large bowel carcinoma in several microregions (communities and areas of the former counties). The following conclusions have been reached. No correlation was found between the incidence of multiple sclerosis and large bowel carcinoma in these studied areas. A positive correlation was demonstrated between the prevalence of multiple sclerosis and large bowel carcinoma in the areas corresponding to former counties but not in communities. Since the latter index is of much lower importance for the estimation of the frequency of malignant neoplasms there is no reason to assume the existence of one or several common aetiologic factors of multiple sclerosis and large bowel carcinoma determining local differences in the frequency of these diseases.
In West Poland with population on the day of the evaluation of 3,097 thousand the incidence of primary neoplasms of the nervous system was calculated in 5 large administration units (provinces) and in 224 smaller units (communities). The testing of the hypothesis of the equal distribution in the communities demonstrated differences, and in 10 of them this incidence was significantly higher above the mean. The main conclusion of the study is that nervous system neoplasms, 90% of which were located intracranially in our material may occur in foci or clusters of clinical as well as statistical significance.
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