HLA-DPB1 gene polymorphism and multiple sclerosis: a large case-control study in the southwest of France

The polymorphism at the HLA-DPB1 locus has been characterized in a large number of patients with multiple sclerosis (n = 112) and in healthy controls (n = 115). Both patients and controls lived in the southwest of France (in the Pyrénées Atlantiques) and had similar ethnic background. The typing procedure involved the selective amplification of the second exon of the DPB1 locus by polymerase chain reaction, followed by hybridization of the amplified DNA with 14 sequence-specific oligonucleotide probes. Individual alleles were identified by the pattern of hybridization of the different probes. The distribution of the DPB1 alleles was not significantly different in multiple sclerosis patients and controls (p = 0.11). This does not corroborate the reported association of multiple sclerosis with the primed lymphocyte typing (PLT)-defined DPw4 specificity and is not in favour of a role played by polymorphic residues of the DP molecule in susceptibility to multiple sclerosis.     

Quantification of perfusion fMRI using a numerical model of arterial spin labeling that accounts for dynamic transit time effects.

A new approach to modeling the signal observed in arterial spin labeling (ASL) experiments during changing perfusion conditions is presented in this article. The new model uses numerical methods to extend first-order kinetic principles to include the changes in arrival time of the arterial tag that occur during neuronal activation. Estimation of the perfusion function from the ASL signal using this model is also demonstrated. The estimation algorithm uses a roughness penalty as well as prior information. The approach is demonstrated in numerical simulations and human experiments. The approach presented here is particularly suitable for fast ASL acquisition schemes, such as turbo continuous ASL (Turbo-CASL), which allows subtraction pairs to be acquired in less than 3 s but is sensitive to arrival time changes. This modeling approach can also be extended to other acquisition schemes.     

Protein nutrition and amino acid metabolism after 4 weeks of total parenteral nutrition with a mixture of 14 dipeptides: serendipitous observations on effects of sepsis in baboons

We investigated parameters of nutrition, metabolism, and organ function after 4 wk of total parenteral nutrition (TPN) in baboons receiving either dipeptides or amino acids as the nitrogen source. The two groups showed no significant difference with respect to gain in body weight, nitrogen balance, plasma and muscle concentrations of amino acids, plasma concentrations of proteins, and leucine incorporation into muscle protein. All dipeptides were efficiently utilized as evidenced by trace concentrations of dipeptides in plasma and urine; they produced no deleterious effect on the function of liver, kidney, or immune system. Development of infection in several baboons increased urinary excretion of urea nitrogen but had no effect on urinary excretion of dipeptides and amino acids with the single exception of taurine, which was greatly increased. In conclusion, the data show long-term efficacy and safety of the dipeptide mixture as the sole nitrogen source for TPN.     

Extent of ictal origin in mesial temporal sclerosis patients monitored with subdural intracranial electrodes predicts outcome.

In patients with mesiotemporal sclerosis, posterior hippocampal involvement at the ictal onset is not associated with an excellent outcome. A study confirmed that ictal onset in the posterior parahippocampal gyrus is associated with a less favorable outcome compared with ictal onset in the anterior parahippocampal gyrus in patients with mesiobasal temporal lobe epilepsy who are undergoing foramen ovale recording. The authors hypothesized that involvement of the two medial contact points of posterior basal temporal subdural (SD) strip at the ictal onset, representing ictal onset in the posterior parahippocampal gyrus, may also adversely influence the surgical outcome. With this objective, the authors assessed the incidence of posterior basal temporal SD strip (the two medial contact points) involvement at the ictal onset in patients with mesiotemporal sclerosis and determined whether presence of this finding influenced surgical outcome. Thirty-six patients with mesiotemporal sclerosis underwent a single SD grid (lateral frontotemporal) and strips (three basal temporal and one orbitosubfrontal) monitoring. Based on the earliest involvement of basal temporal strips (the two medial contact points) during the seizure, patients were classified into (1) anterior and/or middle basal temporal, or (2) posterior basal temporal (with or without involvement of anterior and/or middle basal temporal) ictal onset groups. A temporal lobectomy with adequate resection of the ictal onset zone was performed in all patients. Surgical outcome was based on Engel's classification. Six of 36 (17%) patients were classified into the posterior basal temporal ictal onset group. Only two patients from the posterior basal temporal ictal onset group experienced a good outcome compared with 26 of 30 patients from anterior and/or middle basal temporal ictal onset group (P = 0.01). In patients with mesiotemporal sclerosis who were monitored with SD electrodes, involvement of the two medial contact points of posterior basal temporal strip at the ictal onset (representing ictal onset in the posterior parahippocampal gyrus) occurred in 17% of the patients. These patients might not experience an excellent surgical outcome despite including the ictal onset zone in resection. These findings may be useful in presurgical counseling of patients with mesiotemporal sclerosis who undergo intracranial SD monitoring.     

Morbidity and mortality in Cushing's disease: an epidemiological approach

OBJECTIVE Although Cushing's disease is a well documented clinical entity, there is no epidemiological information about it. The present study tries to obtain this information. DESIGN AND PATIENTS Forty-nine patients affected by Cushing's disease living in Vizcaya (Spain) between 1975 and 1992 were considered for an epidemiological study. RESULTS The prevalence of known cases at the end of 1992 was 39.1 per million inhabitants. The average incidence of newly diagnosed cases was 2 4 cases per million people per year. Cushing's disease was more frequent in women (n= 46) than in men (n= 3), with a ratio of 15:1. Diabetes mellitus and hypertension were observed in 38.7 and 55.1% of patients, respectively. Remission of Cushing's disease was achieved in 36 out of 41 patients (87.5%). In general, the mortality was higher than that expected for the control population (standardized mortality ratio, SMR 3.8, 95% confidence interval, CI 2.5–17.9, P<0.03). Concerning the cause of death, the SMR of vascular disease was 5 (95% CI 3.4–48.6, P< 0.05). Higher age, persistence of hypertension and abnormalities of glucose metabolism after treatment, were independent predictors of mortality (multivariate analyses, P<0.01). CONCLUSIONS Prevalence of Cushing's disease was 39.1 cases/million inhabitants and average incidence was 2.4 cases/million per year. Mortality was elevated, due to vascular disease, associated with higher age, persistence of hypertension and impaired glucose metabolism.     

Balloon occlusion of the internal carotid artery in 40 cases of giant intracavernous aneurysm: technical aspects,cerebral monitoring,and results

Summary We have studied the results of carotid occlusion in the treatment of giant intracavernous carotid artery (ICA) aneurysms in 40 patients. Clinical, angiographic, Doppler and cerebral blood flow (CBF) criteria for tolerance of occlusion are discussed. The patients had headaches (47.5%), cranial nerve compression (87.5%), decreased visual acuity (20%), ruptured aneurysm (15%) and 5% were asymptomatic. Balloon occlusion tests were performed under light sedation anaesthesia: a successful test required perfect clinical tolerance and adequate angiographic collateral circulation in arterial, parenchymatous, and venous phases. Additional criteria include xenon 133 CBF measurements, and transcranial Doppler sonography of the middle cerebral artery. According to these criteria, 5 patients did not tolerate test occlusion and required an extra-intracranial (EC-IC) bypass. Mean follow-up was 4.7 years. All patients were radiologically cured of their ancurysm, and in 35 the symptoms resolved, although 3 had persistent ocular motor nerve palsies, and in 4 visual defects were unchanged. Complications were 1 permanent and 3 transient neurological deficits. Balloon occlusion of the ICA is an effective, reliable form of treatment for intracavernous giant aneurysm and should replace surgical ligation of the cervical carotid artery. With CBF or Doppler monitoring, the risk of neurological deficit is diminished. EC-IC bypass prior to ICA occlusion is indicated if test occlusion is not tolerated.     

The fixed and dilated pupils of premature neonates

We examined on a weekly basis the pupils of 30 preterm infants. In relative darkness (less than 10 foot-candles [ft.-c.] of illumination), the pupils measured a mean of 4.7 mm in the youngest infants (26 weeks' postconceptional age) when the corneal diameter was 7.0 mm. The pupils became progressively smaller, reaching 3.4 mm at 29 weeks' postconceptional age (P less than .001). The pupils did not constrict to the stimulating light (600 ft.-c.) until a mean of 30.6 weeks' (+/- 1 week) postconceptional age. Mydriasis should not be considered indicative of a central nervous system disorder, and a pupil unresponsive to light should not be considered suggestive of blindness until a preterm infant reaches at least 32 weeks' postconceptional age.