Electroneuromyographical and morphological findings in progressive myoclonus epilepsy (PME)

The purpose of this study was to assess the function of peripheral nerves and muscles and to describe morphological changes in muscle biopsies of patients with progressive myoclonus epilepsy. Electroneuromyographic studies were performed on 24 adult patients whose mental and motor skills were either little, moderately or severely impaired by the disease. In 5 patients a specimen of tibial anterior muscle was morphologically and histochemically investigated. The electrophysiological functions of the peripheral nerves and muscles showed gradual increasing abnormalities parallel to the severity of clinical deterioration. The muscle biopsies of 2 patients showed signs compatible with peripheral neuropathy. These findings suggested that progressive myoclonus epilepsy may be related to a systemic membrane disorder.     

Autofluorescence of the human diabetic lens in vivo.

We studied the in vivo measurements of the autofluorescence of the clear lens in 80 juvenile diabetics and 25 young healthy subjects. When the diabetes was of seven years' duration or more, no overlap with the control subjects was found. The pathologic autofluorescence of the diabetic lens showed a positive correlation with age and the duration of diabetes.     

L-tryptophan treatment and the episodic secretion of pituitary hormones and cortisol.

(1) l-Tryptophan, the natural precursor of brain serotonin, was administered to healthy subjects. Plasma free and total tryptophan, somatotropin (growth hormone, GH), follitropin (FSH), lutropin (LH), prolactin and cortisol were analysed after the oral administration of 2 g and 100 mg/kg of l-tryptophan or after 1·28 g of l-leucine, at 08:30 and 11:30. (2) Plasma levels of free and total tryptophan were markedly increased after the oral administration of l-tryptophan. (3) Plasma somatotropin levels were significantly elevated after l-tryptophan treatment at different times of day, but this elevation was not dose-dependent. (4) l-Tryptophan or l-leucine treatment did not affect the pulsatile secretion of follitropin and lutropin. (5) Plasma prolactin was not significantly elevated after the oral administration of l-tryptophan. (6) The morning decline of plasma cortisol was significant with or without l-tryptophan. However, no decline was noticed after l-tryptophan in the middle of day.     

The Need for Halothane Supplementation of N2 O-O2-Relaxant Anaesthesia in Chronic Alcoholics

The demand for intermittant halothane supplementation during N2O-O2-relaxant anaesthesia was studied in 25 alcohlics (annual consumption over 15 1 pure alcohol) scheduled for biliary or gastric surgery. The controls were 45 non-alcoholics and 43 patients with an annual consumption of between 1 to 15 1. Thiopental (3 mg/kg/min) was given for induction. After intubation, halothane supplementation was given in 0.5% concentration for 10-min periods. Standardized criteria for halothane supplementation were various motor and autonomic responses to painful stimuli. Muscular relaxation was kept fairly constant (roughly 90%), as assessed visually with the aid of a peripheral nerve stimulator. The total time for which halothane supplementation was given, expressed as a percentage of the total anaesthesia time, was used as an indication of the need for halothane supplementation. The need for thiopental for induction was not increased to a statistically significant extent in alcoholics, but signs of excitation did occur in 40% as compared with 11% in non-alcoholics (P less than 0.01). The demand for halothane supplementation was higher in alcoholics (47 +/- 4.8%, s.e. mean) than in non-alcoholics (33 +/- 2.3%). This difference, however, was partly due to the higher incidence of gastric surgery, which required more supplementation than biliary surgery. Analysis of the different criteria indicating the need for halothane supplementation revealed that an increase in blood pressure or heart rate was more common in non-alcoholics, whereas motor irritability, sweating and lacrimation were more frequent in alcoholics. Management of the anaesthetic posed no special difficulties in the alcoholics with an estimated mean annual consumption of 32 +/- 4 (s.e. mean) litres of absolute alcohol. Three patients (5% of the alcohol consumers) reported dreams or recollections, suggesting that this mode of halothane supplementation does not guarantee an adequate anaesthetic depth. The difficulties and biases associated with this type of analysis are discussed.     

Pituitary and gonadal function during the use of norgestrel-estradiol vaginal rings

Four women used polysiloxane contraceptive rings (CVR) impregnated with d-Norgestrel and estradiol for contraceptive purposes. The treatment was given in three-week cycles, leaving one treatment-free week between the cycles. Subjects were followed by blood sampling twice a week for three or four treatment cycles. Plasma concentrations of d-Norgestrel, estradiol, progesterone, and gonadotropins were determined by radioimmunoassay.The bleeding patterns were very acceptable. One subject experienced acne and weight gain, but no other side-effects were observed. Furthermore, no local irritation was found during the follow-up period of six or seven months.The individual variation in the mean plasma concentrations of d-norgestrel was between 3.2 and 1.1 ng/ml. Apart from one case, the highest individual levels were observed at the beginning of the first treatment cycle. Plasma estradiol was low during the treatment, but in some cases low post-insertion peaks were observed. No ovulatory progesterone values were found.Plasma LH was generally suppressed, but FSH was not. During the treatment-free week increasing concentrations of both LH and FSH were found, indicating an activation of pituitary function.     

Comorbidities and survival of patients with type 1 diabetes on renal replacement therapy

Aims/hypothesis  

Comorbidities are frequent among type 1 diabetes patients on renal replacement therapy, yet the effect of comorbidities on survival is unknown. Our aim was to estimate this effect.     

Allogeneic hematopoietic stem cell transplantation for chronic myeloid leukemia in Europe 2006: transplant activity, long-term data and current results. An analysis by the Chronic Leukemia Working Party of the European Group for Blood and Marrow Transplantation (EBMT)

The introduction of imatinib mesylate has changed attitudes towards hematopoietic stem cell transplantation (HSCT) for chronic myeloid leukemia (CML). Information on the current use and results of HSCT is warranted. Data from 592 teams in 42 European countries described their use of HSCT for CML from 1990 to 2004. Outcomes were analyzed for 13,416 patients, with a median age of 36 years (range 1-71 years); 60% were male. The analysis considered three time cohorts, 1980 to 1990, 1991 to 1999 and 2000 to 2003. Survival, transplant-related mortality and relapse incidence were assessed at 20 years for the first cohort and compared at 2 years between the three cohorts. The numbers of HSCT for CML increased from 540 allogeneic HSCT in 1990 to 1,396 HSCT in 1999 and declined to 802 in 2004. One third of all patients and half of those with a low risk were alive at 20 years. Survival at 2 years has improved from 53% to 61% in the most recent years due to a reduction in transplant-related mortality from 41% to 30% in all patients and from 31% to 17% in low-risk patients. Stage, donor type, time interval, age and donor-recipient sex combination remain the main risk factors; patients with a risk score of 0 or 1 have a survival probability of 80% at 2 years. HSCT remains an important treatment option for patients with CML. The data describe the current status of this option and the outcome a patient can expect today. They provide an objective basis for decision making.