Living with young-onset dementia in the family - a mixed method study.

Background: Studies on disease-related obstructions experienced in everyday life of younger people with dementia (YOD ≤ 65 years) and their families are encouraged.Aim: To explore how the family carers experience six predefined topics that influence the everyday life and needs of persons with YOD.Method: A quantitative and a qualitative study including family carers of persons with young-onset Alzheimer's dementia (AD) and frontotemporal dementia (FTD). Seventy-four informants responded to the Camberwell Assessment of Needs in the Elderly (CANE) and individual interviews were conducted with 13 informants.Results: Family carers of persons with YOD reported few unmet needs in the CANE assessment. Needs related to behavior and close relationships were reported significantly more frequent (p < 0.1) in persons with FTD than in persons with AD. From the qualitative data, six main themes were emphasized: daily activities turned upside down, involuntary loss of previous social network, losing close relationship, but maintaining a friendship with the spouse, unpredictable behavior adds burdens to a changing life, health and life risks, and economic insecurity for future life and caring costs.Conclusion: Whilst family carers quantitatively reported unmet needs, the individual interviews reported several major difficulties in everyday life.

Young-onset Alzheimer dementia: a comparison of Brazilian and Norwegian carers' experiences and needs for assistance.

OBJECTIVES: Although dementia typically occurs in older people, it can also emerge in people aged younger than 65 years in the form of young-onset dementia, the most common type of which is Alzheimer's disease (AD). However, few studies have examined the needs of persons with young-onset AD (YO-AD) and their families, and cross-cultural research on the topic is even scarcer. In response, we investigated the situations, experiences and needs for assistance of carers of persons with YO-AD in Brazil and Norway. METHODS: As part of our qualitative study, we formed a convenience sample of Brazilian (n = 9; 7 women) and Norwegian carers (n = 11; 6 women) in 2014 and 2015, respectively, and analysed data in light of a modified version of grounded theory. RESULTS: Carers' narratives from both countries revealed five common themes in terms of how YO-AD affected carers' psychological and emotional well-being, physical well-being, professional and financial well-being, social lives and need for support services. CONCLUSIONS: The infrequent differences between carers of persons with YO-AD in Brazil and Norway indicate that carers' problems are highly similar regardless of cultural differences and public services provided. Copyright © 2017 John Wiley & Sons, Ltd.

Extracellular vesicles from oral squamous carcinoma cells display pro- and anti-angiogenic properties.

BACKGROUND: A new intercellular communication mode established by neoplastic cells and tumor microenvironment components is based on extracellular vesicles (EVs). However, the biological effects of the EVs released by tumor cells on angiogenesis are not completely understood. Here, we aimed to understand the biological effects of EVs isolated from two cell lines of oral squamous cell carcinoma (OSCC) (SCC15 and HSC3) on endothelial cell tubulogenesis. METHODS: OSCC-derived EVs were isolated with a polymer-based precipitation method, quantified using nanoparticle tracking analysis and verified for EV markers by dot blot. Functional assays were performed to assess the angiogenic potential of the OSCC-derived EVs. RESULTS: The results showed that EVs derived from both cell lines displayed typical spherical-shaped morphology and expressed the EV markers CD63 and Annexin II. Although the average particle concentration and size were quite similar, SCC15-derived EVs promoted a pronounced tubular formation associated with significant migration and apoptosis rates of the endothelial cells, whereas EVs derived from HSC3 cells inhibited significantly endothelial cell tubulogenesis and proliferation. CONCLUSION: The findings of this study reveal that EVs derived from different OSCC cell lines by a polymer-based precipitation method promote pro- or anti-angiogenic effects.

Apoptosis deregulation influences chemoresistance to azaguanine in human leukemic cell lines.

The involvement of apoptosis in the cytotoxicity mediated by nucleoside analogues, namely azaguanine, and its implication in resistance are not well understood. Using human T-cell acute lymphoblastic leukemia cell lines, sensitive (CEM cells) and resistant to azaguanine (CM3 cells), we observe a decrease in the expression of proapoptotic proteins in CM3 cells, which may be related to the resistance to cell death induced by azaguanine. On the other hand, CM3 cells lack cross resistance with other anticarcinogenic drugs, suggesting that azaguanine may be used alternatively in the presence of chemoresistance. A better knowledge of the apoptotic pathways involved in leukemic cell death resistance may contribute to the development of therapeutic strategies, aimed to prevent chemotherapy resistance.

Clinical characteristics of Guillain-Barré syndrome in a tropical country: a Brazilian experience.

OBJECTIVE: To analyze the clinical variants, outcomes, and prognosis of Guillain-Barré syndrome (GBS) in a Brazilian population. MATERIALS AND METHODS: Clinical and laboratory data of 149 cases of GBS diagnosed from 1994 to 2007 were analyzed. RESULTS: Acute inflammatory demyelinating polyneuropathy (AIDP) was the most frequent variant (81.8%) of GBS, followed by acute motor axonal neuropathy (AMAN) (14.7%) and acute motor and sensory axonal neuropathy (AMSAN) (3.3%). The incidence of GBS was 0.3/100,000 for the state of Rio Grande do Norte and cases occurred at a younger age. GBS was preceded by infections, with the axonal variant associated with episodes of diarrheas (P = 0.025). Proximal weakness was more frequent in AIDP, and distal weakness predominant in the axonal variant. Compared to 42.4% of cases with AIDP (P < 0.0001), 84.6% of cases with the axonal variant had nadir in <10 days. Individuals with the axonal variant took longer to recover deambulation (P < 0.0001). The mortality of GBS was 5.3%. CONCLUSION: A predominance of the AIDP variant was seen, and the incidence of the disease decreased with age. As expected, the distribution of weakness correlated with the clinical variants, and individuals with the axonal variant had a poorer prognosis.

Are adverse effects of antiepileptic drugs different in symptomatic partial and idiopathic generalized epilepsies? The Portuguese-Brazilian validation of the Liverpool Adverse Events Profile.

We report the results of administration of the Portuguese-Brazilian translation of the Liverpool Adverse Events Profile (LAEP) to 100 patients (mean age=34.5, SD=12.12; 56 females), 61 with symptomatic partial epilepsy (SPE) and 39 with idiopathic generalized epilepsy (IGE) (ILAE, 1989) who were on a stable antiepileptic drug (AED) regimen and being treated in a Brazilian tertiary epilepsy center. Carbamazepine was the most commonly used AED (43.0%), followed by valproic acid (32.0%). Two or more AEDs were used by 69.0% of patients. The mean LAEP score (19 questions) was 37.6 (SD=13.35). The most common adverse effects were sleepiness (35.0%), memory problems (35.0%), and difficulty in concentrating (25.0%). Higher LAEP scores were associated with polytherapy with three or more AEDs (P=0.005), female gender (P<0.001), older age (P<0.001), and uncontrolled seizures (P=0.045). The intraclass coefficient (test-retest reliability) for LAEP overall score was 0.848 (95% CI=0.782-0.895), with a range from 0.370 (unsteadiness) to 0.750 (memory problems). Cronbach's α coefficient (internal consistency) was 0.903. The LAEP was highly correlated with Quality of Life in Epilepsy-31 inventory (r=-0.804, P>0.001) and Hospital Anxiety and Depression Scale (Depression: r=0.637, P<0.001; Anxiety: r=0.621, P<0.001) dimensions. LAEP overall scores were similar in people with SPE and IGE and were not helpful in differentiating adverse effects in these two groups. Clinical variables that influenced global LAEP were seizure frequency (P=0.050) and generalized tonic-clonic seizures in the last month (P=0.031) in the IGE group, and polytherapy with three or more AEDs (P=0.003 and P=0.003) in both IGE and SPE groups.

SLO-2, a K+ channel with an unusual Cl- dependence.

The gating of different potassium channels depends on many diverse factors. We now report a unique example of a K+ channel with a Cl - dependence. The slo-2 gene was cloned from Caenorhabditis elegans and is widely expressed in both neurons and muscles; it was highly abundant, as suggested by its high representation in the C. elegans EST database. SLO-2, like its paralogue, SLO-1, was also dependent on Ca 2+. We show by site-directed mutagenesis that its requirements for both Cl- and Ca2+ are synergistic and associated with the same functional domain. SLO-2's dependence on Cl - implies that intracellular Cl- homeostasis may be important in regulating cellular excitability through this unusual K+ channel.

Blockade of dopamine D2 receptors disrupts intrahippocampal connectivity and enhances pain-related working memory deficits in neuropathic pain rats.

BACKGROUND: Dopamine (DA) is thought to be important to local hippocampal networks integrity during spatial working memory (sWM) processing. Chronic pain may contribute to deficient dopaminergic signalling, which may in turn affect cognition. However, the neural mechanisms that determine this impairment are poorly understood. Here, we evaluated whether the sWM impairment characteristic of animal models of chronic pain is dependent on DA D2 receptor (D2r) activity. METHODS: To address this issue, we implanted multichannel arrays of electrodes in the dorsal and ventral hippocampal CA1 field (dvCA1) of rats and recorded the neuronal activity during a classical delayed food-reinforced T-maze sWM task. Within-subject behavioural performance and patterns of dorsoventral neural activity were assessed before and after the onset of persistent neuropathic pain using the spared nerve injury (SNI) model. RESULTS: Our results show that the peripheral nerve lesion caused a disruption in sWM and hippocampus spike activity and that disruption was maximized by the systemic administration of the D2r antagonist raclopride. These deficits are strictly correlated with a selective disruption of hippocampal theta-oscillations. Particularly, we found a significant decrease in intrahippocampal CA1 field connectivity level. CONCLUSIONS: Together, these results suggest that disruption of the dopaminergic balance in the intrahippocampal networks may be important for the development of cognitive deficits experienced during painful conditions. SIGNIFICANCE: This study provides new insights into the role of D2r in the manifestation of pain-related sWM deficits. Our findings support that selective blockade of D2r produces a significant decrease in intrahippocampal connectivity mediated by theta-oscillations, and amplifies pain-related sWM deficits. These results suggest that further characterization of intrahippocampal dopaminergic modulation may be clinically relevant for the understanding of cognitive impairments that accompanies nociceptive stressful conditions.

Head and neck cancer among marijuana users: a meta-analysis of matched case-control studies.

OBJECTIVES: The scientific literature presents conflicting data on a possible causal relationship between marijuana users and the development of head and neck cancer. DESIGN: This study performed a systematic review with meta-analysis. Articles were selected from various electronic databases using keywords obtained from the Medical Subject Headings (MeSH). After reading by three reviewers and scoring of methodological quality, six articles (totaling nine case-control studies) were assessed with Comprehensive Meta-Analysis(®) software. The value of effect (odds ratio) was calculated, which represented the chance of developing head and neck cancer between individuals who had smoked marijuana in their lifetime in models controlled for age, gender, race, and tobacco consumption. RESULTS: Approximately 12.6% of cases and 14.3% of controls were marijuana users. The meta-analysis found no association between exposure and disease (OR=1.021; IC 95%=0.912-1.14; p=0.718). CONCLUSION: No association between lifetime marijuana use and the development of head and neck cancer was found. The different methods of collection/presentation of results in the selected articles prevented other analyzes from being conducted. Additional studies are needed to assess for long-term effects.

Antioxidant enzymes activities of Burkholderia spp. strains-oxidative responses to Ni toxicity.

Increased agriculture production associated with intense application of herbicides, pesticides, and fungicides leads to soil contamination worldwide. Nickel (Ni), due to its high mobility in soils and groundwater, constitutes one of the greatest problems in terms of environmental pollution. Metals, including Ni, in high concentrations are toxic to cells by imposing a condition of oxidative stress due to the induction of reactive oxygen species (ROS), which damage lipids, proteins, and DNA. This study aimed to characterize the Ni antioxidant response of two tolerant Burkholderia strains (one isolated from noncontaminated soil, SNMS32, and the other from contaminated soil, SCMS54), by measuring superoxide dismutase (SOD), catalase (CAT), glutathione reductase (GR), and glutathione S-transferase (GST) activities. Ni accumulation and bacterial growth in the presence of the metal were also analyzed. The results showed that both strains exhibited different trends of Ni accumulation and distinct antioxidant enzymes responses. The strain from contaminated soil (SCMS54) exhibited a higher Ni biosorption and exhibited an increase in SOD and GST activities after 5 and 12 h of Ni exposure. The analysis of SOD, CAT, and GR by nondenaturing PAGE revealed the appearance of an extra isoenzyme in strain SCMS54 for each enzyme. The results suggest that the strain SCMS54 isolated from contaminated soil present more plasticity with potential to be used in soil and water bioremediation.

Properties of Ca2+ currents in acutely dissociated neurons of the chick ciliary ganglion: inhibition by somatostatin-14 and somatostatin-28.

Whole-cell voltage-clamp recordings were made from acutely dissociated neurons obtained from the embryonic chick ciliary ganglion. Recording pipettes were filled with salines containing 120 mM CsCl or 120 mM tetraethylammonium-Cl. Application of depolarizing voltage commands evoked L-type Ca2+ currents and, at voltages positive to 0 mV, an unidentified cationic conductance. The unidentified cationic conductances made the Ca2+ currents appear to undergo voltage-dependent inactivation and made a large contribution to tail currents present during repolarizing voltage steps. Ca2+ Ca2+ currents showed little or no sign of inactivation and did not reverse at voltages up to +60 mV. Application of somatostatin-14 or somatostatin-28 produced a reversible inhibition of Ca2+ currents in virtually all cells, regardless of size. Somatostatin-28 (1-14) was inactive. The effects of somatostatin-14 and somatostatin-28 were attenuated by pretreatment with pertussis toxin, suggesting a role for G-proteins in mediating the response. Somatostatin-14 and somatostatin-28 had no effect on voltage-dependent K+ currents. The results suggest that somatostatin peptides modulate the motor output of the chick ciliary ganglion.

[Group consultation: a space for women]. / Consulta coletiva: o espaço da mulher.

This experience took place in a Medical Center, in Rio de Janeiro, in 1988. The group responsible for it included a social worker, a nutritionist, a nurse and a gynecologist. The aims were: to offer better health care; to discuss the importance, the results and the appropriate therapies of the Pap smear; to offer women an opportunity to meet and discuss their problems, experiences, desires, expectations; and to offer to the health professionals the opportunity to re-think their own practices. The methodology used was characterized by making appointments for a medical exam subsequent to a Pap smear; evaluation of each Pap smear result and its appropriate treatment, previous to the group consultation; group discussion about the Pap smear results; and about themes brought up by women clients. The main results were: an increase of 42.7% in the number of women who went for their first medical exam; an increase of ten minutes in the length of each medical exam; better screening for syphilis and gonorrhea, better quality of health care within the perspective of women as integral beings (greater appreciation of a woman s own experiences, be it in terms of her complaints and problems be it in her evaluation of the medical care system). This kind of consultation is of rather recent date and if it becomes more wide-spread it could result in an increase in the number and quality of the medical exam.

[Lobar cerebral hemorrhage. Clinical aspects, etiology and course. Review of 58 cases]. / Hemorragia cerebral lobar clínica, etiologia, evolução. Revisão de 58 casos.

Fifty eight patients with spontaneous lobar hemorrhage of a 264 patients series with intracerebral hemorrhage seen during a six years period are reviewed. Twenty nine had arterial hypertension, 9 had other etiologies, and in the remaining 20 cases no apparent etiology was detected. Headache was a prominent symptom and occurred in 56%. Seizures occurred in 12%. Coma was infrequent. Clinical findings on admission depended on the location of the hematoma. Lobar hemorrhage occurred most commonly in the parietal region. Mortality rate was 8.6%. Size of hematoma (larger than 4 cm) on CT and altered consciousness on admission correlated with poor outcome.

[Fluctuations in Guillain-Barré syndrome related with intravenous human immunoglobulin (triphasic course): case report]. / Síndrome de Guillain-Barré com flutuações relacionadas ao tratamento com imunoglobulina humana endovenosa. Curso trifásico. Relato de caso.

The authors report the case of a patient with severe Guillain-Barré syndrome (tetraplegic and on mechanical ventilation), that was treated with intravenous immunoglobulin (IVIg), 2 g/Kg. At first, there was clinical improvement, followed by clinical deterioration two weeks later. On the second course of IVIg there was, again, clinical improvement and then deterioration, 65 days after treatment. Finally, on the third course of treatment definitive recovery was achieved and no more relapses happened so far (three years after the treatment). The authors review the literature about fluctuations related to treatment with IVIg. Conclusions are that these patients should be closely observed during the first weeks after IVIg treatment, and that further studies are still necessary to elaborate alternative protocols on the prevention of these cases.

[Epidemiology of tegumentary American leishmaniasis and its relations with agriculture and prospecting, in a locality of the State of Bahia, Brazil]. / Epidemiologia da leishmaniose tegumentar americana e suas relações com a lavoura e o garimpo, em localidade do Estado da Bahia (Brasil).

This study was carried out in the city of Lençóis, State of Bahia, with the objective of verifying the association between leishmaniasis infection and occupation. A Montenegro test and a questionnaire including biological and socio-economic variables were applied to the study group. Sandflies were captured in and around dwellings. The higher-than-average prevalence of leishmaniasis observed among agricultural workers and prospectors is explained by the double exposure to the infection-both at home and at work.

[Sensory polyneuropathy and neural conduction block as an initial manifestation of polyarteritis nodosa]. / Polineuropatía sensitiva y bloqueo de conducción como primera manifestación de una poliarteritis nodosa.

Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis which affects middle calibre arteries and which has a tendency to form aneurysms. It may appear as one of a great variety of clinical forms depending on the vascular territory afflicted. Almost any organ may be involved although the lung is usually unaffected. Neurological manifestations are very frequent in this disease (global incidence being around 80%), especially at the peripheral nervous system level. Multiple mononeuritis is the most usual manifestation classically associated with this type of vasculitis, but it may also appear as distal sensorimotor polyneuropathy, this being its most infrequent form of presentation. The presence of nerve conduction blocks has also been recently described, usually associated with demyelinating processes. However peripheral nerve affectation in vasculitis has its origins in small ischaemic infarcts in the sensorimotor conduction fibres. It may well be that segmentary demyelination and axonal degeneration reflect different levels of ischaemic damage.

[Chronotropic and pressoric response in myocardiopathy in patients with heart failure during ergometric test. Correlation of spirometry variables with effort]. / Resposta cronotrópica e pressórica nas miocardiopatias em portadores de insuficiência cardíaca durante prova ergométrica. Correlação das variáveis espirométricas ao esforço.

PURPOSE: To correlate the variables heart rate (HR), blood pressure (BP) and double product (DP) during the ergometric test with the variables oxygen consumption (VO2) and pulmonary ventilation (VE) of spiroergometry. METHODS: A study was carried out with 40 male patients suffering from cardiomyopathy with heart failure (functional class II-IV of NYHA)-of ischemic (IS), Chagas' disease (CH) and idiopathic (ID) etiology. These three groups were compared to a group of 10 normal individuals (N). The 4 groups were evaluated under 4 different conditions: rest (RES), anaerobic threshold (LA), power peak of exercise (P) and in the fourth minute recovery (REC). The investigation was carried out with the data obtained through spiroergometry (using a treadmill and spiroergometric equipment specific for the effort), as well as data related to HR, BP, DP, VO2 and VE. RESULTS: There were significant differences observed in the ergometric evaluate of the HR, BP and DP responses in the IS, CH and ID groups as compared with the N group. There were significant difference observed in the spirometric evaluation to the VO2 and VE efforts in the IS, CH and ID groups as compared with the N group. CONCLUSION: The HR, BP and DP variables studies, obtained by means of classic ergometry, unaided by direct methodology (spiroergometry) enabled them to infer valuable data for the control and evaluation of cardiomyopathies with IC, taking into consideration the low chronotropic and pressoric responses in the various phases of evaluation during this study, corresponding to the concomitant low performance of O2 consumption and pulmonary ventilation.

Association between executive/attentional functions and caries in children with cerebral palsy.

The aim of the present study was to evaluate the existence of an association between attention/executive functions and the development of dental caries in individuals with cerebral palsy (CP). Seventy-six children with CP were selected from a physical rehabilitation center and a school serving children with disabilities. The control group was made up of 89 children without neurological impairment. Socioeconomic status, presence of teeth with cavities due to caries, degree of motor impairment and intellectual, executive and attentional functions were assessed. Mean age of participants was 8.9 years (SD=3.56). The CP group had a significantly lower performance (p<0.05, Mann-Whitney test) on the intelligence, attentional function and executive function tests in comparison to the control group. Controlling for the clinical diagnosis (CP or control group), motor impairment and intellectual function, the significant explanatory variables for the presence of teeth with cavities were performance on the Complex Rey figure test (OR=0.941) and the Digit Span subtest of the Wechsler Intelligence Scale for Children in backward order (OR=0.581). After controlling for intellectual function, clinical diagnosis and motor impairment, deficits in executive and attentional functions increased the odds of developing dental caries in children with cerebral palsy.

Respiratory muscle strength and quality of life in myotonic dystrophy patients.

INTRODUCTION: Studies on quality of life in myotonic dystrophy (MD) are scarce and the relationship between respiratory muscle strength and health-related quality of life (HRQoL) has yet to be determined. The present study aims to investigate respiratory muscle strength and HRQoL and their relationship in MD patients. METHODS: Twenty-three patients (13 men, aged 40 ± 16 years) with MD were evaluated for pulmonary function, maximal inspiratory and expiratory pressure (MIP and MEP, respectively), sniff nasal inspiratory pressure (SNIP) and HRQoL using the Short Form (SF-36) quality of life questionnaire. RESULTS: Respiratory muscle strength values were 71 ± 20 cmH2O (64 % predicted), 76 ± 32 cmH2O (70 % predicted), and 79 ± 28 cmH2O (80 % predicted) for MEP, MIP, and SNIP respectively. Significant differences were found in the SF-36 domains of physical functioning 58.7 ± 31.4 vs. 84.5 ± 23 (p < 0.01, 95 % CI = 1.6-39.9) and physical problems 43.4 ± 35.2 vs. 81.2 ± 34 (p < 0.001, 95 % CI = 19.4-6.1) when compared with the reference values. According to single linear regression analysis, MIP explains 29 % of the variance in physical functioning, 18 % of physical problems and 20 % of vitality. CONCLUSIONS: Individuals with MD have reduced expiratory muscle strength. HRQoL may be more impaired in some physical domains, which might be influenced by variations in inspiratory muscle strength.