Humoral immune stimulation and antiepithelial antibodies in yersinia infection

Results consistent with a general humoral immune stimulation were found when 127 sera from 89 patients with Yersinia enterocolitica infection were studied. Significantly increased gammaglobulin concentrations and elevated isohemagglutinin titers were seen in these sera as compared to sera from normal blood donors and patients with streptococcal infection. Antinuclear and anti–smooth muscle antibodies were demonstrated in both yersinia and streptococcal infection. The prevalence of non–organ specific antiepithelial antibodies reacting with gastrointestinal and thyroid epithelial cells was significantly increased in yersinia infection.     

Long-term follow-up of functioning after spinal surgery in patients with Rett syndrome

In a prospective study, 23 consecutive girls with Rett syndrome and neuromuscular scoliosis were evaluated for functioning at a long-term follow-up. The patients had mostly improved, which was confirmed by their parents. Rett syndrome is associated with neuromuscular scoliosis and has a typically long C-shaped thoracolumbar kyphoscoliosis. Prospective long-term follow-up studies related to these patients’ total situation are sparse. Most studies focus on the Cobb angle of the scoliosis, whereas parents are mainly concerned about the girls’ continued functioning. Twenty-three patients with Rett syndrome and neuromuscular scoliosis were evaluated preoperatively from 1993 to 2002. At follow-up, 19 patients remained in the study. Three patients died (not due to surgery), and one patient could not participate because it was too far to travel. Mean follow-up time was 74 months (range 49–99 months). The assessments comprised the sitting balance, seating supports in wheelchair, weight distribution, time used for rest, care given, and angle of scoliosis. Follow-up questionnaires and two-open-ended questions about the positive and negative effects of surgery were sent to parents. Sitting balance, number of seating supports in wheelchair, weight distribution, time used for rest, and the Cobb angle had all improved after surgery. The parents assessed improvement in seating position, daily activities, time used for rest, and cosmetic appearance. We can conclude that the stabilized spine resulted in sufficient strength to keep the body upright with the possibility of looking around at the surroundings more easily. The girls got better seating position with less need for seating adaptations in the wheelchair and with reduced time needed for resting during the day. Finally we can conclude that the indication for surgery is to get a better posture which lead to less risk of pressure sores, and that un upright position lead to better possibility to easily breath with fewer episodes of pneumonia and a better general health as result. The evidence of positive surgical effects for girls with Rett syndrome is of great importance in indication for surgery in the decision-making process.

Prolyl oligopeptidase: a potential target for the treatment of cognitive disorders

Prolyl oligopeptidase (POP) is a ubiquitous post-proline cleaving enzyme that is highly expressed in brain. Current knowledge about the biochemical features of POP and the pharmacological action of its specific inhibitors has indicated that POP participates in several aspects of the central nervous system (CNS), including learning, memory and mood. Furthermore, a role has been suggested for POP in pathological processes such as eating and mood disorders, hypertension and cell-cycle disturbances, in addition to its proposed connection with the neurodegenerative processes which occur in Alzheimer's, Huntington's and Parkinson's diseases. The milestones responsible for the accelerated development of POP inhibitors include the discovery that these compounds reverse memory loss in animal models of drug- or lesion-induced amnesia and the observation that the expression of POP correlates with age. Today, several POP inhibitors have already been evaluated in preclinical trials as potential drugs for the treatment of natural memory deficits that occur with aging or the pathological memory loss characteristic of Alzheimer's disease. Thus, the results that are emerging from basic research on POP function will facilitate the fine-tuning of more efficient drugs to target this protease.     

Possible interpretation of subjective complaints in patients with spontaneous subarachnoid haemorrhage.

OBJECTIVE: To analyse factors related to subjective non-cognitive and cognitive complaints in patients with spontaneous subarachnoid haemorrhage. SUBJECTS: Twenty-seven patients with subarachnoid haemorrhage and 27 age-, sex- and education-matched healthy controls. METHODS: A battery of cognitive tests measuring visuo-spatial abilities, verbal abilities, and fine-motor skill, Brief Social Support Questionnaire, and Life Orientation Scale were individually presented to all participants. RESULTS: Cognitive complaints were related to low social support but not to cognitive performance. Complaints about headaches and dizziness were also related to decreased cognitive performance. Above-normal optimistic life-orientation was related to the absence of complaints in patients with subarachnoid haemorrhage. Healthy participants were best discriminated from patients with subarachnoid haemorrhage by less satisfactory social support system and decreased fine motor skills in the latter group. CONCLUSION: Change in social support network may be an important resource for increasing quality of life in patients with subarachnoid haemorrhage not only through help provided by supporters but also indirectly, through increasing subjective well-being. The absence of subjective complaints in patients with subarachnoid haemorrhage is not necessarily related to better objective condition but rather to inadequately optimistic life orientation.     

Preoperative evaluation of activity and function in patients with paralytic scoliosis

Preoperative evaluation of patients with paralytic scoliosis should take into account the consequences of surgery on the every day life of the patient. However, the parameters that are customarily used in these operations relate only to very narrow measures such as the angle of scoliosis or kyphosis. The aim of this study was to introduce a set of instruments appropriate for measuring both function and activities in paralytic scoliosis patients. The study took as its starting point the WHO International Classification of Impairments, Disabilities and Handicaps (ICIDH), in which an activity is described at the level of the individual and function at the level of the organ. A consecutive series of 100 paralytic scoliosis patients with 18 different diagnoses were evaluated preoperatively with a set of instruments that had been specially developed at Linköping hospital, in which the variables are classified according to the system used in the ICIDH. The set of instruments included general information and evaluation of activities and function – sitting, balance, weight distribution to sitting surface, angle of scoliosis, reaching, pain estimation, activities of daily living (ADL) Barthel and ADL Klein and Bell, care given, time spent resting, and seating supports). The results showed that patients with paralytic scoliosis constitute a heterogeneous group in activities and function. Even when the patients were grouped into four subgroups according to the Scoliosis Research Society Classification, they remained very heterogeneous. However, reaching, Klein and Bell Activities of Daily Living and pain could only evaluate patients who could understand verbal instructions. In those who could not, assessment relied more heavily on measures of function and level of dependence. It was concluded that the choice of assessment must be guided by the patient’s ability to understand verbal instructions irrespective of his/her disorder. It is important to use the three levels – impairments, disabilities and handicaps – in order to focus on the different outcomes in the two groups with respect to the patient’s total situation.     

The hypoparathyroidism of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy protective effect of male sex

In autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy, hypoparathyroidism (HP) is the most common endocrine component. It occurs in most (but not all) patients. Determinants of its occurrence are unknown, and there is no proof for its autoimmune nature. Recently, the Ca(2+)-sensing receptor (CaSR) was reported to be an autoantigen in HP. With our group of 90 patients, we aimed at identifying the determinants and pathomechanism of HP. For the determinants, we evaluated gender and the HLA class II. For the pathomechanism, we searched for parathyroid autoantibodies, including antibodies against CaSR and PTH. Also, we studied whether AIRE is expressed in the human parathyroid, because its absence could be a pathogenetic factor. We found a clear gender linkage with lower and later incidence in males. Of the 14 patients who had escaped HP, 13 were males. This was associated with adrenal failure, which was the first or only endocrinopathy in 47% of males vs. 7% of females. In contrast, we found no linkage to the HLA class II. By immunofluorescence, 19% of the patients had antibodies to parathyroid epithelia. By immunoblotting, these recognized several parathyroid proteins. No antibodies were observed against the CaSR or PTH. By RT-PCR, AIRE mRNA was not found in the parathyroid.