Narcolepsy: immunological aspects

Narcolepsy with cataplexy is a debilitating sleep disorder with an estimated prevalence of about 0.05%. Narcolepsy is caused by a selective loss of hypocretin (orexin) producing neurons in the perifornical hypothalamus. Based on the very strong association with the HLA subtype DQB1*0602, it is currently hypothesized narcolepsy is caused by an autoimmune-mediated process directed at the hypocretin neurons. So far however, studies focusing on general markers of (auto)immune activation, as well as humoral immunity against the hypocretin system have not yielded consistent results supporting this hypothesis.     

Long-term effect of perindopril on coronary atherosclerosis progression (from the perindopril's prospective effect on coronary atherosclerosis by angiography and intravascular ultrasound evaluation [PERSPECTIVE] study)

The multicenter EUROPA trial of 12,218 patients showed that perindopril decreased adverse clinical events in patients with established coronary heart disease. The PERSPECTIVE study, a substudy of the EUROPA trial, evaluated the effect of perindopril on coronary plaque progression as assessed by quantitative coronary angiography and intravascular ultrasound (IVUS). In total 244 patients (mean age 57 years, 81% men) were included. Evaluable paired quantitative coronary angiograms were obtained from 96 patients randomized to perindopril and from 98 patients to placebo. Concomitant treatment at baseline consisted of aspirin (90%), lipid-lowering agents (70%), and beta blockers (60%). The primary and secondary end point was the difference of minimum and mean lumen diameters (quantitative coronary angiography) or mean plaque cross-sectional area (IVUS) measured at baseline and 3-year follow-up between the perindopril and placebo groups. After a median follow-up of 3.0 years (range 1.9 to 4.1), no differences in change in quantitative coronary angiographic or IVUS measurements were detected between the perindopril and placebo groups (minimum and mean luminal diameters -0.07 +/- 0.4 vs -0.02 +/- 0.4 mm, p = 0.34; mean luminal diameter -0.05 +/- 0.2 vs -0.05 +/- 0.3 mm, p = 0.89; mean plaque cross-sectional area -0.18 +/- 1.2 vs -0.02 +/- 1.2 mm(2), p = 0.48). In conclusion, we found no progression in coronary artery disease by quantitative coronary angiography and IVUS with long-term administration of perindopril or placebo, possibly because most patients were on concomitant treatment with a statin.     

The hypothalamus in episodic brain disorders

Episodic brain disorders (EBD) form an intriguing group of neurological diseases in which at least some of the symptoms occur in attacks. The hypothalamus integrates many brain functions, including endocrine and autonomic control, and governs various body rhythms. It seems a likely site in which the initiation of attacks of EBD can be modulated. Indeed, the hypothalamus has a crucial role in EBD such as narcolepsy and cluster headache. The same may be true for migraine and depression. Here we summarise the evidence supporting an important role for the hypothalamus in the initiation of disease episodes in various EBD. Study of the various pathophysiological concepts of EBD within the context of the hypothalamus may prove a fruitful example of cross-fertilisation between various research areas.     

Older living kidney donors: surgical outcome and quality of life

BACKGROUND: Older living kidney donors remain controversial because of their physiological decline in glomerular filtration rate and their increased susceptibility of surgical complications. Little is known about the quality of life (QOL) of this elderly group. The purpose of this study is to examine surgical outcome and the QOL in older living donors. PATIENTS AND METHODS: All 105 consecutive living donors who underwent a laparoscopic donor nephrectomy between June 2002 and February 2006 were prospectively included in the study. Intra- and postoperative complications were measured. Quality of life was recorded preoperatively and at several endpoints postoperatively. Older donors were defined as 55 years and older. RESULTS: There were no significant differences in intra- and postoperative complication rates and 1-year graft survival rate between both groups. Elderly donors (n=34) had both a significant lower postoperative pain at rest at day 1 compared with the younger group (P=0.019) and a lower total pain score in the analysis for the whole follow-up period (P=0.002). Although small solitary significant differences in Short Form-36 Health Survey, Multidimensional Fatigue Inventory-20 and visual analogue scale measuring pain, between both groups were detected, in general QOL of older donors was not different than of younger donors. CONCLUSION: Although small solitary significant differences exist with respect to pain, social functioning and mental health older donors, in general, have similar surgical outcome and quality of life when compared with younger donors. There is no need to exclude older donors in screening programs for transplantation.     

Perilunate fracture-dislocations of the wrist: comparison of temporary screw versus K-wire fixation

PURPOSE: Temporary intercarpal screw fixation has been suggested as an alternative to temporary K-wire fixation in the treatment of perilunate wrist dislocations. We compared the 2 treatment methods in 2 retrospective cohorts with a null hypothesis that there would be no difference in final wrist motion. METHODS: Eighteen patients with surgically treated perilunate wrist dislocations (9 treated with intercarpal screws, 9 with intercarpal K-wires) were evaluated an average of 44 months after injury. The intercarpal screws were removed an average of 5 months and the K-wires an average of 3 months after the initial procedure. Complications included 3 pin track infections (1 with wrist sepsis), 2 scaphoid nonunions (screw fixation), and 2 patients with loss of reduction (K-wire fixation) treated with repeat surgery. RESULTS: Four patients (2 in each cohort) had wrist arthrodesis with poor results. Among the 14 remaining patients the final flexion arc was 97 degrees for patients treated with screw fixation compared with 73 degrees for patients treated with K-wires. The mean grip strength was 74% (screw fixation) and 67% (K-wire) that of the uninjured arm. According to the Mayo Modified Wrist Score, the functional result was excellent in 1 patient (screw), good in 2 patients (1 each group), fair in 6 patients (3 in each group), and poor in 9 patients (4 screws, 5 K-wire). Seven patients (2 screws, 5 K-wires) had grade 2 or 3 midcarpal arthritis according to the criteria of Knirk and Jupiter, but none had more than mild radiocarpal arthritis. CONCLUSIONS: The results of treatment with temporary screws are comparable to the results of treatment with temporary K-wires. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III.