[L-tryptophan and the eosinophilia-myalgia syndrome].
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Biomedical subjects
Publications and source records attributed to J J Prick.
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During the last decade nine adult patients were seen with an unilateral choreoathetosis or choreoballism of acute onset. In seven a hemodynamically significant lesion in a carotid artery has been detected; in four the artery was completely occluded. In one there was a subclavian steal syndrome, and in another the etiology remained unclear. Three patients had their dyskinesia at the same side as the carotid artery lesion, and four at the controlateral side.
The hypereosinophilic syndrome (HES) is a rare disease, characterized by a long lasting hypereosinophilia of the blood and the marrow and by the presence of varying internal symptoms and frequent neurological signs. These latter can be as well central: encephalomalacy, organic psycho-syndrome, as peripheral: polyneuropathy or mononeuropathia multiplex, autonomic neuropathy and in rare cases a polymyositis. The disease clinically resembles very to periarteriitis nodosa (PAN). However, the prognosis of HES is far better, especially when early recognized and adequately treated.
A chondroblastoma within the temporal bone in a 16-year-old boy is reported. The tumor recurred after intracapsular resection and curettage. After reoperation, radiotherapy was performed. The data from 137 cases of chondroblastoma are summarized.
3 siblings with symmetrical calcifications in the strio-pallido-dentate system are described. Parathyroid function was normal and there were no signs of central or peripheral myelinopathy. This is the 9th family reported with autosomal recessive idiopathic strio-pallido-dentate calcinosis and the first to be investigated by computerized tomography (CT). CT scans appeared to be superior to plain skull radiograms to assess the localization and the extent of the calcifications in vivo. The calcifications were the least extensive in the youngest and the most extensive in the eldest. It is suggested that the calcifying process is a progressive disorder. It seems to start in the dentate nuclei and pons, and subsequently extends to the basal ganglia and to the radiation of the corpus callosum.
The clinical symptomatology of patients suffering from pseudoxanthoma elasticum and the radiodiagnostic signs in four patients suffering from this disease have been reviewed. In these patients four groups of previously unpublished radiodiagnostic abnormalities were found. These are calcification of a number of ligaments; changes in the wall of brachiocephalic trunk, the right common, internal and external carotid arteries; fibro-osseous metaplasia of the long bones; dysplasia of the vertebrae. The authors believe that these changes are explicable by the general disorder of connective tissue and its derivatives which occurs in pseudoxanthoma elasticum.
32 patients with the chronic progressive form of the multiple sclerosis were treated with high doses of cyclophosphamide and prednisone during 20 days, to produce immunosuppression. The effect of treatment was measured by scoring three different Kurtzke scales just before treatment (1), just after treatment (II), and 3 months after treatment (III). The results indicate that in the treatment period I to II most patients improve, some dramatically. The improvement continues in 15 of 25 patients scored between 6 and 33 months after treatment. Better results were found in patients with shorter duration of their disease. More improvement is found as the IgG percentage of the spinal fluid at I is higher. The improvement is less when the initial condition (I) is more serious.
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