Biomedical subjects

W F Arts

Publications and source records attributed to W F Arts.

60 records · Page 4Linked to original sources

PubMed1978

Investigations on the inheritance of nemaline myopathy.

Extensive investigations on 11 patients with nemaline myopathy (six index patients, five relatives), their parents, and some healthy relatives were carried out. In one family, nemaline myopathy was inherited as an autosomal dominant trait. No linkage between the locus of nemaline myopathy and the locus of seven informative genetic markers (out of 25 investigated markers) was found. In two families an autosomal recessive inheritance could be demonstrated with certainty. In these families, both parents of each index patient had rods and an increased number of fibers with internal nuclei. In two other families, one or both parents of each index patient had an increased number of fibers with internal nuclei, also indicating the possibility of autosomal recessive inheritance in these cases. It can be concluded that there are two types of nemaline myopathy. However, these two diseases could not be separated on a clinical or histopathological basis.

Adolescent↗

PubMed1978

Common origin of rods, cores, miniature cores, and focal loss of cross-striations.

Twelve patients suffering from congenital neuromuscular disorders showed at least two of the following structural changes in their muscle biopsy specimens: rods, cores, miniature cores (M lesions), and focal loss of cross-strians (F lesions). There appeared to be no essential differences between M and F lesions except for the presence of vesicular nuclei in and around the latter. A striking finding was the presence of adjacent M lesions, F lesions, or both in two or several adjoining muscle fibers. Serial sections often showed a definite relationship of the lesions with blood vessels. The finding of rods cores, and M and F lesions, not only in one biopsy specimen but also in one muscle fiber, raised the intriguing possibility of common pathogenic mechanisms producing these lesions.

Adult↗

PubMed1977

Long-term effect of glucocorticosteroids on neuromuscular blocking in mice.

The effect of dexamethasone, given thrice weekly, on neuromuscular blocking by D-tubocurarine was studied in mice. Dexamethasone antagonised the effect of D-tubocurarine. This antagonism developed gradually during the first two weeks of treatment. The possible relationship between this finding and the beneficial effect of glucocorticosteroids in patients with myasthenia gravis is discussed.

Animals↗

PubMed1975

Effect of prednisolone on neuromuscular blocking in mice in vivo.

The working capacity of inbred female Swiss mice on the rotating rod was tested in an investigation of the effect of previously injected prednisolone, 1.2 mg, on the neuromuscular blocking effect of two different doses of d-tubocurarine and of low-dose hemicholinium-3. The effect of both drugs could be partially or wholly antagonized by prednisolone. The consequences of this finding are discussed in relation to the beneficial effect of prednisone in patients with myasthenia gravis.

Animals↗

PubMedDate not supplied

Follow-up of 146 children with epilepsy after withdrawal of antiepileptic therapy.

The relapse rate after discontinuation of antiepileptic drug treatment was investigated in 146 children with epilepsy, in whom medication was withdrawn according to a predesigned protocol, after a seizure-free period of at least 2 years and normalization of the EEG. The cumulative probability of remaining seizure-free in this series was 74.5%. Three-quarters of the relapses occurred during the withdrawal period and in the 2 years thereafter. From multivariate analysis, the factors indicating a significantly higher relapse risk were seizures with a known cause and female sex. In primary generalized epilepsy, no factor significantly increased the likelihood of a recurrence. In partial epilepsy, significant factors predictive of recurrence were the presence of a neurological deficit (focal neurological signs and/or mental retardation), female sex, a positive family history for epilepsy, and the number of drugs necessary for control of the seizures. The present results are compared with the available literature data. It is argued that using multivariate analysis after elimination of EEG variables uncovers significant clinical predictive factors that in other studies may have remained hidden. Finally, it is argued that statistical analysis may be used to enable the clinician to predict the likelihood of recurrence in individual children with a given set of relevant predictive factors.

Adolescent↗

PubMedDate not supplied

Basilar artery giant fusiform aneurysms caused by congenital defect of the internal elastic lamina and media.

Giant fusiform aneurysms of the basilar artery were found in a 6-year-old boy who subsequently died after rupture of the aneurysm, and in a 64-year-old man who showed signs of ischemia and compression of the brain stem. Autopsy disclosed strikingly similar abnormalities of the wall of the basilar artery, consisting of a defect of the internal elastic lamina and absence of the media. A congenital anomaly may play a role in the pathogenesis of this abnormality, in both young and some elderly patients.

Basilar Artery↗