EEG CHANGES DURING CAROTID ENDARTERECTOMY: DRUG EFFECT AND EMBOLISM

Clinical neurological status and the electroencephalogram (EEG)were monitored during right carotid endarterectomy under regionalanaesthesia in a patient who had suffered two prior cerebralinfarctions. No change in EEG or clinical status was seen withocclusion of the carotid artery, but generalized encephalopathicchanges were seen after a small dose of narcotic given beforecarotid occlusion. Bihemispheric changes in EEG and neurologicalstatus occurred upon release of carotid occlusion. This casedemonstrates three important points. First, EEG changes canbe seen with sedative or anaesthetic agents in patients whohave previously suffered brain injury. Second, temporary neuraldysfunction may be seen remote from the site of a localizedinjury to the brain—a phenomenon known as diaschisis.Finally, this report reiterates the observation that the riskof injury to the brain during carotid endarterectomy is notlimited to the period of possible ischaemia during occlusionof the carotid artery.     

Rapidly Progressive Type III Gaucher Disease: Deterioration Following Partial Splenectomy

ABSTRACT. Total splenectomy has been found to accelerate disease progression in Type III Gaucher disease, therefore partial splenic resection was performed in a 28 month old girl with rapidly progressive (non-Norrbottnian) Type III disease to alleviate the effects of hypersplenism. Surgery failed to arrest the disease process and splenomegaly recurred within three months. The erythrocyte glucosylceramide level increased post-operatively. Post-mortem studies revealed dense accumulation of Gaucher cells in spleen, liver, lungs, bone marrow and within the central nervous system. The glucosylceramide content of brain and liver was elevated to the range previously noted in splenectomised Norrbottnian patients. Thus, in rapidly progressive Type III Gaucher disease, neither total nor partial splenectomy alone provides a favourable outcome.     

Clinical and Biochemical Outcome of Marrow Transplantation for Gaucher Disease of the Norrbottnian Type

ABSTRACT. Three children, two girls and one boy, with Gaucher disease of the Norrbottnian type were treated by allogeneic bone marrow transplantation (BMT). Two of the donors were heterozygotes for Gaucher disease, and the third did not carry the gene. Engraftment was achieved in all three children but the boy became a chimera. The children have been followed from 1.5 to 6 years after transplantation. In each case the outcome was favourable, and in two children (patients 1 and 3) the BMT has been life saving. In all three cases a positive biochemical effect was apparent. Almost all biochemical parameters normalized within one year. The authors conclude that BMT is the treatment of choice in severe cases of Gaucher disease. BMT should be considered early in the course of disease and vigorous efforts made to find a suitable donor.     

Role of ultraviolet light and UV DNA in the induction of skin lesions in experimental animals

In vivo and in vitro animal experiments have been performed to clarify the role of ultraviolet light denatured DNA (UV DNA) and ultraviolet light (UVL) in the pathogenesis of the dermal lesions of human SLE. Rabbits immunized with UV DNA show deposition of immunoglobulin at the dermal-epidermal junction following exposure to UVL. We have also shown that UV DNA appears concomitantly with the antibody at the dermal-epidermal junction subsequent to the UVL exposure. Both n DNA and UV DNA have been shown to bind to the dermal-epidermal junction in vitro which could result in the persistence of these antigens at this site. These studies lend further support to the hypothesis that the release of UV DNA and its subsequent deposition at the dermal-epidermal junction may result in binding of both immunoglobulin and complement, leading to the development of histological lesions of SLE.