Biomedical subjects
Publications and source records attributed to E C Raps.
A man with plasma cell dyscrasia and bone marrow infiltration presented with rapidly progressive encephalopathy. Magnetic resonance imaging revealed multiple small areas of abnormal signal that correlated with the autopsy findings of thrombotic microangiopathy isolated to the central nervous system.
Giant aneurysm surgery continues to be a technically difficult task with high operative morbidity. Recent advances in cardiac surgery have fueled interest in the technique of deep hypothermic circulatory arrest for the treatment of giant and complex intracranial aneurysms. Fourteen patients with giant intracranial aneurysms operated on with the technique of deep hypothermic circulatory arrest are presented. All 14 aneurysms were successfully treated. There were 2 intraoperative strokes: 1 resulted in severe disability and 1 resulted in mild disability. No significant neurological complications were related to the technique of cardiopulmonary bypass with deep hypothermic circulatory arrest. This initial experience indicates that patients with giant and complex intracranial aneurysms might benefit from a surgical approach that included the use of deep hypothermic circulatory arrest.
Histiocytic infiltration of the optic chiasm is rare. We report a patient with a seizure disorder on anticonvulsant therapy, who developed systemic reactive histiocytosis. Treatment with splenectomy, corticosteroids, and anticonvulsant medication change resulted in clinical remission. The patient later developed recurrent lymphohistiocytosis restricted to the optic chiasm. Extensive re-evaluation yielded no evidence for infectious or malignant etiology. Radiation therapy and withdrawal of anticonvulsant therapy resulted in clinical remission. We conclude that both phenytoin and phenobarbital may trigger a lymphohistiocytic process and that infiltration of the visual pathway is possible.
We report a patient who developed an acute loss of vision in the left eye associated with proptosis, ophthalmoparesis, conjunctival injection, and chemosis. Funduscopy revealed optic disc swelling, and retinal whitening consistent with an ophthalmic artery occlusion. Angiography disclosed bilateral carotid dissections presumably resulting from head trauma 11 days earlier. An orbital ischemic syndrome may be a delayed manifestation of traumatic carotid dissection and precede cerebral hypoperfusion.
We report the simultaneous occurrence of biopsy-proven temporal arteritis in husband and wife. Serologic and viral studies were negative, including viral culture of the wife's temporal artery. The concurrent incidence of giant cell arteritis in a married couple would suggest a common exogenous exposure.
We report a patient with a pathologically proven pontine cavernous angioma and a horizontal one and a half syndrome. Following recurrent hemorrhages, the vascular malformation was removed from the dorsal pons using microsurgical techniques with partial resolution of the motility disturbance. This case demonstrates that vascular malformations of the brainstem parenchyma may be removed, thereby precluding further hemorrhage and facilitating the return of neurologic function.
Central nervous system infections with Listeria monocytogenes result in varied clinical syndromes ranging from meningitis to rhomboencephalitis. A case of Listeria meningitis complicated by symptomatic communicating hydrocephalus and hydrostatic cervical cord compression is presented which clinically and radiographically improved with aggressive ventricular drainage.
A 23-year-old woman with systemic lupus erythematosus had a severe hypersensitivity reaction to the drug ibuprofen. The reaction, documented by rechallenge during hospitalization, was characterized by marked hypotension, fever, rigors, conjunctivitis, meningitis, nausea, arthralgias, and leukopenia. In addition to illustrating the potential diagnostic confusion with infection or lupus flare, this case is of interest in that the reaction developed suddenly despite the patient's uneventful use of the drug for over two years. Collected cases are reviewed from the literature, and the common features are summarized.
The induction of nucleoside-specific nonresponsiveness was further studied in the autoimmune strain MRL/MP +/+ (MRL/n). Experiments were undertaken to determine (i) whether nucleoside-conjugated spleen cells are able to induce specific nonresponsiveness to T-dependent nucleoside antigens in MRL/n mice, and (ii) whether periodic treatment with nucleoside-conjugated spleen cells would retard the development of spontaneous anti-DNA antibodies and associated indicators of autoimmunity. The results show that nonresponsiveness to nucleoside antigens is inducable in male, but not in female, MRL/n mice. Nonresponsiveness in male MRL/n was transferable and mediated by T cells. Treatment of male MRL/n mice with nucleoside-conjugated spleen cells (NSC) appeared to attenuate the progress of autoimmune symptoms in experimental animals. These results are discussed in the context of recent studies exploring the etiology of autoantibody production and the loss of self-tolerance in murine models of autoimmunity.
The kinetic and the H-2 requirements for the induction of nucleoside-specific suppression were examined in several strains of mice; specifically, whether adenosine (A)-coupled spleen cells given intravenously suppress the primary response to adenosine-KLH. The adenosine system was chosen because C57Bl/6 mice were originally found to be resistant to immune suppression when challenged 5 days after treatment with adenosine-coupled spleen cells. (Raps et al. J. Immunol. 126, 1542, 1981.) It was determined (i) whether A-specific nonresponsiveness is inducible in strains other than C57Bl/6; (ii) whether changes in hapten density on the A-conjugated spleen cells could alter C57Bl/6s ability to become nonresponsive, and (iii) whether there are interstrain differences in the time required to induce A-specific suppressor T cells (Ts). The results show that there are H-2-associated differences in the time required to induce A-specific immune suppression. While A-spleen cells failed to suppress the A-specific response in C67Bl/10 (H-2b), they did induce unresponsiveness in B10.D2 (H-2d on C57Bl/10 background). A 2.5-fold increase in epitope density of adenosine on cells did not influence the kinetics of suppression. C67Bl/6 were resistant to suppression on Day 5, but like the CB6F1, susceptible to unresponsiveness 10 days after treatment. Nonresponsiveness was T-cell-mediated and transferable across IgH-V barriers. Suppression induced by Balb/c donor mice is transferable to Igh-incompatible CAL-20 mice. These results are discussed in the context of genetic restrictions which regulate suppressor T-cell interactions.
The fine specificity of immune suppression was further studied in a murine experimental model in which the induction and expression of suppression by individual nucleoside-bound spleen cells were examined. The experiments were undertaken: a) to determine whether all 4 nucleosides have the ability to elicit suppressor T cells; b) to examine the specificity of cross-suppression induced by individual nucleosides; and c) to establish that nucleoside-specific suppression is mediated by T cells bearing nucleoside-specific receptors. The results show that, of the 4 nucleosides studied, only guanosine and thymine riboside were capable of eliciting immune suppression. Thymine riboside-coupled spleen cells induced cross-suppression to all 4 nucleosides. Furthermore, the nonresponsive state generated by guanosine-spleen cells was found to be transferable and mediated by nucleoside-binding T cells. The ability of only select nucleosides to elicit immune suppression is discussed in the context of recent evidence demonstrating the selective activation of T cell subsets by intramolecular antigenic determinants.