Early cognitive impairment in a subset of patients with presumed low-grade glioma

We investigated the presence of cognitive impairment, in adults with presumed low-grade glioma at early stage of disease prior to major treatments, in relation to neurological symptoms and radiological characteristics of the tumour. Sixteen patients were evaluated. A subset of patients was identified with clearly impaired cognition. Patients with cognitive impairment often had large tumours in the left frontal lobe, were relatively young, and most of them were males. We conclude that cognitive dysfunction may be present already at early stage of disease, and that early identification of patients at risk is warranted.     

Physical activity in a total population of children and adolescents with cerebral palsy

The aims of this study were to describe the participation in physical activity of children with cerebral palsy (CP) at school and during leisure time and to identify characteristics associated with physical activity. The frequency of receiving physiotherapeutic interventions were described as a variable of interest. A total population of 364 children with verified CP aged 7–17 years living in the Skåne region in Sweden was studied using cross-sectional data from the CP follow-up programme (CPUP). Proportional odds ratios showed the most severe gross motor limitations Gross Motor Function Classification System Expanded and Revised (GMFCS-E&R) to be a characteristic for low participation in physical education at school (PE) and GMFCS-E&R level III to be a characteristic for low participation in regular physical leisure activity. The age group of 7–11 years and obesity were characteristics associated with high participation in PE, whereas thinness was associated with low participation in regular physical leisure time activities. The highest proportion of children receiving physiotherapeutic interventions was found in GMFCS-E&R level III, while mental retardation, especially if moderate or severe, proved to be an independent characteristic associated with low frequency of physiotherapeutic interventions. Gender and epilepsy did not influence the odds for participation in physical activities. Special considerations are needed when planning interventions for increased physical activity in children with CP, as the individual prerequisites differ, even among children with the same gross motor function level according to the GMFCS-E&R.     

Sources of Uncertainty and Their Association with Medical Decision Making: Exploring Mechanisms in Fanconi Anemia

Background

Effects of different sources of medical uncertainty on people’s health-related cognitions, emotions, and decision making have yet to be systematically examined.

Purpose

The aim of this study is to examine how uncertainties arising from different sources are associated with decision making regarding stem cell transplantation in Fanconi anemia, a rare, inherited bone marrow failure syndrome that typically presents during childhood.

Methods

Data were collected through a cross-sectional survey of 178 parents of 126 Fanconi anemia patients.

Results

Two distinct sources of uncertainty were associated with decision outcomes: probability was associated with a lower likelihood of choosing stem cell transplantation, and ambiguity due to conflicting expert opinions was associated with greater decision-making difficulty. Concern about transplantation may mediate these associations.

Conclusions

Different sources of uncertainty have different effects on Fanconi anemia treatment decisions, which may be mediated by parents’ emotional reactions. Further research is needed to elucidate these effects and help Fanconi anemia families cope with uncertainty.     

Skin Cancer and Quality of Life: Assessment With the Dermatology Life Quality Index

Background. Quality of life (QOL) has been identified as an important outcome in cancer research, yet the most common malignancy among humans, nonmelanoma skin cancer (NMSC), has been poorly studied in this regard.
Objective. To determine whether change occurred in the QOL of NMSC patients after surgery using a general, validated dermatology QOL instrument: the Dermatology Life Quality Index (DLQI).
Methods. A prospective study was conducted on 121 consecutive patients referred to a dermatologic Mohs surgery clinic with NMSC of the head and neck. QOL assessment was performed using the DLQI before (n=121) and after surgical treatment at 4 months (n=101).
Results. QOL scores demonstrated little handicap at initial diagnosis. The total DLQI scores showed little change over time, but an item analysis revealed that 2 of the 10 items demonstrated statistically significant change over time, with QOL improving after treatment—decreased painfulness/itchiness/soreness and less necessity to use concealing clothing.
Conclusions. General dermatology QOL instruments demonstrated minimal handicap at initial diagnosis and little change after treatment of NMSC. Although the associations were modest, improvement in some aspects of well-being after treatment of NMSC was demonstrated. A more disease-specific instrument may be necessary to study this disease process further.     

Imaging findings of familial Mediterranean fever

PURPOSE: The aim of this study was to study the imaging findings of familial Mediterranean fever (FMF). MATERIALS AND METHODS: We performed a retrospective review of the medical records and imaging studies of 38 patients with proven FMF, diagnosed between 1992 and 2002. RESULTS: The most common clinical manifestation was recurrent peritoneal attacks with abdominal pain (76.3%) and fever (42.1%). Abdominal imaging findings included ileus (n=12), splenomegaly (n=5), hepatomegaly (n=2), ascitis (n=2), focal peritonitis (n=2), mesenteric streaking (n=1), and enlarged mesenteric lymph node (n=1). One patient developed fatal peritoneal mesothelioma, and 13.1% of the patients developed amyloidosis with sonographic findings of renal parenchymal disease or cardiomyopathy. Arthritis was second in frequency, occurring in 34.2% of patients; radiographs were normal (n=4) or showed joint effusion and periarticular soft tissue swelling (n=4) due to synovitis. One patient developed seronegative destructive arthropathy. Skin lesions were noted in 23.6% of patients. Pleuritis was encountered in 13.1% and pericarditis in 5.2%. Polyarteritis nodosa (PAN) was present in two patients, multiple sclerosis in one, and autoimmune hemolytic anemia in one patient. CONCLUSION: FMF predominantly involves abdominal viscera but can affect other organs. The majority of patients have nonspecific imaging findings, and the radiologic diagnosis is rarely considered. Amyloidosis, mesothelioma, and destructive arthropathy are potential serious complications of FMF. PAN, multiple sclerosis, and autoimmune hemolytic anemia are probably rare associations or rather than coincident with FMF.     

Monitoring stroke progression: in vivo imaging of cortical perfusion,blood–brain barrier permeability and cellular damage in the rat photothrombosis model

Focal cerebral ischemia is among the main causes of death and disability worldwide. The ischemic core often progresses, invading the peri-ischemic brain; however, assessing the propensity of the peri-ischemic brain to undergo secondary damage, understanding the underlying mechanisms, and adjusting treatment accordingly remain clinically unmet challenges. A significant hallmark of the peri-ischemic brain is dysfunction of the blood–brain barrier (BBB), yet the role of disturbed vascular permeability in stroke progression is unclear. Here we describe a longitudinal in vivo fluorescence imaging approach for the evaluation of cortical perfusion, BBB dysfunction, free radical formation and cellular injury using the photothrombosis vascular occlusion model in male Sprague Dawley rats. Blood–brain barrier dysfunction propagated within the peri-ischemic brain in the first hours after photothrombosis and was associated with free radical formation and cellular injury. Inhibiting free radical signaling significantly reduced progressive cellular damage after photothrombosis, with no significant effect on blood flow and BBB permeability. Our approach allows a dynamic follow-up of cellular events and their response to therapeutics in the acutely injured cerebral cortex.     

Localized chrysiasis,aluminum salt deposition and dystrophic calcification a decade after gold injections

Localized chrysiasis is rare and can occur in two settings: after localized or traumatic implantation of elemental gold or gold salts or after localized laser or light therapy in someone who has been previously exposed to systemic gold therapy. We report a unique case of localized chrysiasis with associated aluminum salt deposition and sclerosing lipogranulomas because of previous injections of aurothioglucose (Solganal®). The unique histopathologic findings seen in this case have not been previously reported.