First Year Of Illness Research Articles

Longitudinal evaluation of the early auditory gamma-band response and its modulation by attention in first-episode psychosis.

Executive control over low-level information processing is impaired proximal to psychosis onset with evidence of recovery over the first year of illness. However, previous studies demonstrating diminished perceptual modulation via attention are complicated by simultaneously impaired perceptual responses. The present study examined the early auditory gamma-band response (EAGBR), a marker of early cortical processing that appears preserved in first-episode psychosis (FEP), and its modulation by attention in a longitudinal FEP sample. Magnetoencephalography was recorded from 25 FEP and 32 healthy controls (HC) during active and passive listening conditions in an auditory oddball task at baseline and follow-up (4-12 months) sessions. EAGBR inter-trial phase coherence (ITPC) and evoked power were measured from responses to standard tones. Symptoms were assessed using the Positive and Negative Syndrome Scale (PANSS). There was no group difference in EAGBR power or ITPC. While EAGBR ITPC increased with attention in HC, this modulation was impaired among FEP. Diminished EAGBR modulation in FEP persisted at longitudinal follow-up. However, among FEP, recovery of EAGBR modulation was associated with reduced PANSS negative scores. FEP exhibit impaired executive control over the flow of information at the earliest stages of sensory processing within auditory cortex. In contrast to previous work, this deficit was observed despite an intact measure of sensory processing, mitigating potential confounds. Recovery of sensory gain modulation over time was associated with reductions in negative symptoms, highlighting a source of potential resiliency against some of the most debilitating and treatment refractory symptoms in early psychosis.

Predictors of self-reported word-finding difficulties in glioma patients – a longitudinal study

ABSTRACT Background Word-finding difficulties are a common self-reported concern in glioma patients known to negatively affect social participation and life satisfaction. Discrepancies between self-reported difficulties and performance on objective tests have been reported, but studies are seldom conducted longitudinally. Aims The aim of the present study was to examine the occurrence of self-reported word-finding difficulties before and during the first year after glioma surgery. In addition, we investigated whether self-reported word-finding difficulties were predicted by standardized language tests and psychological distress. Methods and procedures Twenty-three patients with gliomas (grade 1–3) were assessed pre-surgery, at six and twelve months follow-up. Self-reported word-finding difficulties were addressed with the item I am able to find the right word(s) to say what I mean, from the Functional Assessment of Cancer Therapy – Brain (FACT-Br). Confrontation naming was tested with the Boston Naming Test (BNT), word production with a semantic fluency test and word knowledge with a vocabulary test. Self-reported measures of psychological distress were assessed with the Hospital Anxiety and Depression Scale (HADS). Ordinal regression models were used to examine predictors of self-reported word-finding difficulties. Outcomes and results Word-finding difficulties were reported by 68% of the patients pre-surgery, increasing to 90% and 85% at the following assessments. Significant changes were observed in the magnitude of reported concerns between pre-surgery assessment and six months follow-up. Regression analyses demonstated that self-reported word-finding difficulties were predicted by psychological distress and vocabulary pre-surgery and vocabulary at six months follow-up, whereas confrontation naming and semantic fluency did not become significant in any of the assessments. Conclusion Our results indicate that self-reported word-finding difficulties occurred in a high percentage of glioma patients throughout the first year of illness. Patients reported increased difficulties after surgery that were not predicted by confrontation naming or semantic fluency but by vocabulary performance. The results imply further that psychological distress is a factor that should be taken into account. Self-reported function is an important supplement to objective testing and can provide indications about mental health status and the patients` perspective on language related challenges in everyday life.

Self-assessment of a breast care nursing model within a Breast Unit: learning process and keys to improving breast care.

To assess the adherence of a nursing care model in a multidisciplinary breast cancer unit in a tertiary hospital to the recommended competencies and quality indicators. Aligning the competencies of the breast care nurse with international recommendations for this role helps better fulfil patient needs, increases satisfaction and ensures continuity of care. Cohort study. Breast care nursing was assessed in all patients treated at the Functional Breast Unit from 1 July 2016 to 30 June 2017. Patients were followed for 1 year. Sociodemographic, clinical and pathological data, treatments performed and nursing interventions were collected. The strobe checklist has been used to report this study. We analysed nursing interventions carried out in 382 patients attended over 1 year in a multidisciplinary breast cancer unit. All patients with early disease had contact with the nurse at different times during their primary treatment. Only 58% of patients with advanced disease had contact with the nurse during their first year of illness. Moreover, first contact with the nurse was delayed by more than a week from diagnosis, the interval recommended by international guidelines. The nursing care model meets the core competencies defined for the breast care nurse in patients with early breast cancer, but the first visit should be organised earlier, and follow-up should extend beyond completion of primary treatment. This study evaluated the breast care nurse model in one breast cancer unit according to international guidelines. Nursing care adhered to most guideline requirements in patients with early breast cancer, but not in those with advanced disease. New models of care need to be developed for women with advanced breast cancer in order to achieve true patient-centred care. No contribution from the patient or the public because the data collected was entered into the clinical history by the health professionals of the Breast Unit as part of their usual clinical practice.

КЛИНИКО-ГЕНЕТИЧЕСКАЯ ХАРАКТЕРИСТИКА БОЛЬНЫХ С TRAPS (ПЕРИОДИЧЕСКИЙ СИНДРОМ, АССОЦИИРОВАННЫЙ С РЕЦЕПТОРОМ ФАКТОРА НЕКРОЗА ОПУХОЛИ) ПО ДАННЫМ ФЕДЕРАЛЬНОГО РЕВМАТОЛОГИЧЕСКОГО ЦЕНТРА

The tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a rare autosomal dominant disease associated with a mutation in the TNFRSF1A gene. It belongs to the group of monogenic autoinflammatory diseases (mAIDs) characterized by the repeated and prolonged episodes of fever, skin rashes, musculoskeletal, ophthalmic symptoms and an increase in the level of acute phase markers. The main targeted therapy is inhibitors of interleukin-1 (IL-1). The purpose of the study was to present the clinical and genetic characteristics of patients with TRAPS according to the data from the V.A. Nasonova Research Institute of Rheumatology (Moscow, Russia). The study included 28 patients from 2013 till 2021 with a clinical diagnosis of TRAPS from a selection of 162 (both pediatric and adult) patients with mAIDs: 17 females/11 males aged 2.5 to 65 years old (Me 9.0 y/o [7.9; 14.5]); 7 adults/21 children. All of them have underwent molecular genetic analysis for mutations in the TNFRSF1A gene. Results: the TRAPS was diagnosed in 28 (17.3%) among 162 patients with mAIDs. The age of onset ranged from 1 month till 28 years old (Me 4.0 y/o [1.0; 7.1]). Most patients fell ill prior to the age of 10 years old (89.3%), of which 8 (28.6%) before 1 year old, 7 (25%) from 1 to 5 years old, 1 (3.6%) from 5 to 10 years old, 10 (35.7%) from 10 to 18 years old and 2 (7.1%) from 21 to 28 years old. The duration of the disease and the delay in diagnosis at the time of inclusion ranged from 3 months till 59 years old. The diagnosis was made within the first year of illness in 4 (14.3%) of observed cases; within 1 to 10 years in 18 (64.3%); in 8 (28.6%) there was a delay in diagnosis of over 10 years; and in 4 (14.3%) the delay was more than 20 years of illness. The duration of attacks varied from 10 to 30 days, the intervals between the attacks ranged from 2 weeks till 6 months. The clinical manifestations were as follows: fever in 96.4% of cases, skin rashes in 71.4%, musculoskeletal symptoms in 18 (64.3%) cases, oligoarthritis in 9 (32.1%), polyarthritis in 2 (7.1% ), gastrointestinal manifestations in 18 (64.3%), lymphadenopathy of various groups in 10 (35.7%), ophthalmological manifestations in 8 (28.6%). The less often were the different nervous system manifestations, which accounted for 6% to 21% of cases, pharyngitis in 4 (14.3%) and stomatitis in 2 (7.1%). An increase in the sedimentation rate of erythrocytes was noted in 23 (82.1%), C-reactive protein in 20 (71.4%), leukocytosis in 11 (39.3%). The diagnosis in all patients was confirmed genetically. 10 patients had a low-penetrance mutation of р.Arg121Gln (р.Arg92Gln according to the old nomenclature). 5 familial cases were identified, the total number of sick family members was 12, from 2 to 4 in each family; 4 cases of amyloidosis (the all four were adults). Treatment: glucocorticoids in 19 (67.9%) cases, Cyclosporine A in 1, Colchicine in 5. 15 patients (53.6%) received genetically engineered biological preparations, the IL-1 (Canakinumab) was prescribed in 10 (35.7%) patients with the duration of admission from 1 to 9 years, Etanercept in 1, Adalimumab in 1 and Tocilizumab in 3. Conclusion: due to the similarity of clinical and laboratory symptoms with manifestations of rheumatic diseases, patients with TRAPS can be encountered in the rheumatologists’ practice. Most patients in the studied cohort had an early onset of the disease (prior to 10 years old), and the diagnosis delay of 10+ years was noted in 1/3 of patients. All patients had a specialized inflammatory phenotype coupled with genetic mutations. About half had required the appointment of biological drugs, IL-1 mainly. A complete response to the therapy in most patients and a good tolerability of treatment were noted, which allows the authors to consider this as the basis in the treatment and management of patients with TRAPS.

Financial Burden in Families of Children with West Syndrome.

ObjectiveTo evaluate the illness-related expenditure by families of children with West syndrome (WS) during the first year of illness and to explore the potential determinants of the financial drain.MethodsThis cross-sectional study was conducted at a tertiary care hospital between July 2018 and June 2020. Eighty-five children with WS who presented within one year from the onset of epileptic spasms were included. The details of the treatment costs (direct medical and nonmedical) incurred during the first year from the onset of epileptic spasms were noted from a parental interview and case record review. Unit cost was fixed for drugs and specific services. Total cost was estimated by multiplying the unit cost by the number of times a drug or service was availed. The determinants of the financial burden were also explored.ResultsThe median monthly per-capita income of the enrolled families (n = 85) was INR 3000 (Q1, Q3, 2000, 6000). The median cost of treatment over one year was INR 27035 (Q1, Q3, 17,894, 39,591). Median direct medical and nonmedical expenses amounted to INR 18802 (Q1, Q3, 12,179, 25,580) and INR 6550 (Q1, Q3, 3500, 15,000), respectively. Seven families had catastrophic healthcare expenditure. Parental education and choice of first-line treatment were important determinants driving healthcare expenses. The age at onset of epileptic spasms, etiology, treatment lag, the initial response to treatment, and relapse following initial response did not significantly influence the illness-related expenditure by the families.ConclusionWS imposes a substantial financial burden on the families and indirectly on the healthcare system.Supplementary InformationThe online version contains supplementary material available at 10.1007/s12098-021-03761-1.

Patients’ experiences of being diagnosed with multiple sclerosis and their support and guidance needs in the first year of illness

Aims: To describe patients’ experiences of being diagnosed with multiple sclerosis (MS) and their needs for support and guidance in the first year with the disease. Methods: A qualitative, semistructured interview study with five patients with MS was conducted. Data were transcribed and analyzed using interpretative phenomenological analysis. Results: Three themes emerged: Frightening to get the diagnosis, needs from family and network, and the importance of continuity in contacts with nurses. The participants struggled with thoughts about serious disabilities and stigmatization, although for some the disease moved more into the background after a period. The patients expressed a need for support and guidance from both families and nurses. They primarily used their families for support, and this was influenced by the family’s emotional involvement. Family members typically did not have the necessary experience to give appropriate guidance. The patients were aware of the impact the disease had on their families, and this sometimes stopped them from seeking support from them. The nurse should develop the contact with the individual patient to become familiar with the patient’s illness narrative and previous coping strategies so to better provide holistic care. Conclusion: Being diagnosed with MS gives rise to frightening thoughts about the future and the fear of stigmatization. Patients need support and guidance from both their families and the nurses at the MS clinic. Nurses should consider the family’s role and how to involve the resources available from family members. It is important that the nurses develop a close relationship with the patient. The possibility of contact nurses may be a useful solution for providing targeted support and guidance to patients diagnosed with multiple sclerosis.

A Novel Mutation in FGF23 Causing Severe Manifestation of Hyperphosphataemic Familial Tumoral Calcinosis

We present a 16-year girl, second issue of a consanguineous couple presented with painless symmetrical bony swellings around hips for 3 years. She lost 25 kg weight due to profound anorexia in the first year of illness and developed secondary amenorrhea. Last 7 months she had repeated generalized tonic clonic seizure due to hypocalcaemia. The bony swellings were located in both gluteal regions and were 30x35 cm2, hard and non tender. Mobility of hip joints were restricted. Except for severe wasting as a part of generalized cachexia other systemic examination was unremarkable. Investigations revealed anaemia (Hb-E trait), low albumin, low corrected calcium (during convulsions) otherwise normal, persistently high phosphate & vitamin-D level, normal iPTH & alkaline phosphatase (ALP), low serum ascorbic acid, normal ESR & CRP. X-ray & CT scan of pelvis showed large calcified mass in periarticular soft tissues. Biopsy confirmed presence of dystrophic calcification. Diagnosis of tumoral calcinosis was based on biochemical parameters, imaging and biopsy findings. A homozygous novel mutation in FGF homology domain: B-21 mutated from G to A at 232 in exon 2. As a result glutamic acid is replaced by lysine. Phosphate restricted diet and phosphate lowering agent improved biochemical paramaters. Surgical excision has been withheld. Contemporary manifestation of hFTC and Hb E trait; two disease of autosomal recessive inheritance in an individual makes it extremely rare. High index of suspicion is needed for early diagnosis and treatment.

Clozapine Titration for People in Early Psychosis: A Chart Review and Treatment Guideline.

The use of clozapine, particularly in young people, is often limited by early treatment-emergent adverse effects including drowsiness and lethargy. Concerns about adverse effects, medication adherence, and the need for blood monitoring often impede the use of clozapine in this population, leading to repeated trials of less effective medications. Current clozapine dosing recommendations are based on people further in the course of their illness and thus reflect different responsiveness and sensitivities to antipsychotic medication. As such, there is a need for evidence-based guidelines for titration and dosing of clozapine among people in early psychosis. We performed a chart review of 14 people treated with clozapine within our early psychosis team. Data regarding dose titration, response, time to discontinuation, symptom severity, weight gain, and other adverse effects were gathered at clozapine initiation, 3 months, and last available visit on clozapine. People treated with slow titration within their first year of psychosis onset achieved sustained response at very low maintenance doses (mean dose = 81 mg/d, mean duration of treatment = 200 weeks) compared with slow titration with longer duration of illness (mean dose = 350 mg/d, mean duration of treatment = 68 weeks) or standard dose titration in early psychosis (mean dose = 112 mg/d, mean duration of treatment = 38 weeks). The most common adverse effects in all groups were weight gain and sedation, with the groups requiring higher mean doses reporting a broader range of adverse effects. There was no apparent difference in the clinical global impression for severity or improvement between the slow titration and standard titration groups in people with early psychosis. These observations are synthesized into a proposed treatment guideline for use of clozapine among people in early psychosis. We describe development of a slow titration approach to initiating clozapine among people in early psychosis. This approach resulted in clinical response at remarkably low maintenance doses of clozapine among people within their first year of illness, but not in those with longer duration of symptoms. Slow titration also led to good tolerability and acceptance of clozapine treatment for some patients.

F80. COGNITIVE TRAJECTORIES OVER 6 YEARS IN FIRST-EPISODE SCHIZOPHRENIA AND HEALTHY CONTROLS – A PROSPECTIVE LONGITUDINAL MULTI-ASSESSMENT STUDY

BackgroundPatients with first-episode schizophrenia (FES) have consistently showed impaired cognitive functioning compared to healthy controls across a broad array of cognitive domains. After psychosis onset the cognitive performance in FES seems to remain stable or even improve over time. Many earlier studies, however, did not include healthy control groups which made it unclear whether cognitive changes were due to genuine improvements or other arbitrary factors. Thus, the development of individual cognitive domains over time is not yet fully examined.MethodsThe present study has a multi-assessment design, and includes data from eight follow-ups over six years. For the patient group, assessments were conducted yearly, apart from the first year where assessments were conducted every six months. Healthy controls were assessed at baseline, after two years and after six years. A total of 28 FES-patients and 28 healthy controls participated in the study, with 79 % of patients retained at the 6-year follow-up. Cognition was assessed with MATRICS Consensus Cognitive Battery. Data were analyzed with linear multilevel models.ResultsFES-patients scored lower than the control group across all cognitive domains at baseline. Over six years, the cognitive trajectories of visual learning seem to remain stable for both groups, while FES-patients showed slight improvements in attention (β = 1.34, SE = .18, p < .001), verbal learning (β = .65, SE = .29, p < .031), processing speed (β = .69, SE = .35, p < .051), reasoning/ problem solving (β = 1.68, SE = .27 p < .001), working memory (β = .89, SE = .27, p < .002) and social cognition (β = .93, SE = .30, p < .003). Most of these cognitive trajectories start to improve within the first year of illness and continues throughout the six year period. The improvement in processing speed (β = .18, SE = .48, p > .05), verbal learning (β = .56, SE = .59, p > .05) and social cognition (β = .82, SE = .59, p > .05) seem to be larger for FES-patients compared to controls, but these differences were not significant. The patient group’s improvement in reasoning/ problem solving (β = 1.31, SE = .51, p < .05) was significantly larger that the control group, but they showed smaller improvement in working memory (β = -1.03, SE = .51, p < .05).DiscussionOur results show that improvements are already discernable after 6 months following illness outbreak. There are different trajectories for different cognitive domains. Moreover, two cognitive domain trajectories were significantly different between control group and FES-patients. This points to the importance of assessing cognitive development over many years with multiple assessments when exploring cognitive impairments in schizophrenia. From a clinical perspective, this may speak in favor of a targeted rehabilitation of different cognitive domains.

Alterations of complex mismatch negativity (cMMN) elicited by a two-tone pattern paradigm in early-phase psychosis

The mismatch negativity (MMN) is a component of the event-related potential (ERP) elicited by a change in auditory stimulation (e.g., the occurrence of a deviant sound that violates the rules or regularities of the preceding stimulus sequence) regardless of whether one is attending to the change or not. As such, the MMN provides a useful index of pre-attentive cognition. While decreases in MMN amplitude are robustly observed in chronic schizophrenia, these deficits are less consistently present at the early phase of the illness. The current study utilizes a two-tone pattern paradigm that requires more complex computations than typical oddball stimulus presentations, which may be more appropriate for elucidating MMN deficits in an early phase psychosis (EP) sample. The stimuli were a standard sequence consisting of two alternating tones with different tonal frequencies (eg. ABABAB…), with MMN-eliciting pattern violations created by repetitions of either the A or the B tone. EEG recordings of 15 EP participants and 12 healthy controls (HCs) were collected. While no between-group differences were observed, MMN amplitudes in the EP group were correlated with positive and negative psychosis symptom scores. Follow-up analysis stratifying EP participants according to illness duration showed a reduced MMN amplitude in EP participants with a longer (2+ years) duration of illness, but not in EP participants who were within the first year of illness. These findings suggest a two-tone pattern paradigm may be useful in characterizing MMN-indexed cortical impairment later in the early phase of the illness, but not at first episode.

Self-harm and suicide attempts in Schizophrenia.

The risk of suicide among persons with schizophrenia (SZ) is higher than in general population, with multiple contributory factors. We assessed the prevalence of risk of deliberate self-harm and suicide attempts, along with associated socio-demographic and clinical factors in a group of SZ outpatients (n=61) as part of a larger study on overall schizophrenia-associated risks. To investigate factors associated with risk of deliberate self-harm and suicide among persons with schizophrenia. Out of 270 SZ participants evaluated for various risks using Ram Manohar Lohia Risk Assessment Interview (RML-RAI), 61 reported risk of self-harm including suicide attempt/s. The factors associated with this risk were further evaluated on clinical details and Diagnostic Interview for Genetic Studies. Risk of reported self-harm was 22.59%. Among them, 10% had attempted suicide at least once. Current age and past month Global Assessment of Functioning score from DIGS (GAF) were significantly correlated with suicide attempt. Attempters had significantly lower current GAF score, indicating poorer functioning. Among 27 attempters, 9 attempted at the onset of illness while 6 others attempted suicide within one year. Most common method of attempt was ingestion of insecticides or overdose of medication, followed by hanging or jumping from height. In our hospital-based sample of suicide attempters, 10% had attempted suicide, among them over 55% within first year of illness. Attempters were significantly older than non-attempters and suffered from significantly more severe illness than non-attempters. Positive symptoms were significantly associated with attempt, irrespective of time at which suicide was attempted.

Outcome and Trends in Treatment of Systemic Juvenile Idiopathic Arthritis in the German National Pediatric Rheumatologic Database, 2000-2013.

To investigate the clinical presentation and medical treatment of patients with systemic juvenile idiopathic arthritis (JIA) during the first year of illness. Our study focused on 3-year outcomes in a subsample of patients who were followed up longitudinally. From 2000 to 2013, 597 patients with systemic JIA and a disease duration of ≤12 months were recorded in the National Pediatric Rheumatologic Database. Among those patients, 3-year outcome data were available for 133. These data included the clinical Juvenile Arthritis Disease Activity Score in 10 joints (JADAS-10) and the physician's global assessment score (on a numerical rating scale), as well as assessment of joint involvement, growth retardation, and patient-reported outcomes. The median clinical JADAS-10 declined significantly, from 7 in 2000 to 2 in 2013, while the proportion of patients with inactive disease increased from 19% in 2000 to 41% in 2013. The rate of treatment with systemic glucocorticoids and disease-modifying antirheumatic drugs (DMARDs) remained stable from 2000 to 2013. By 2013, the proportion of patients with systemic JIA who were treated with biologic DMARDs had increased to 20%. At 3-year follow-up, 72% of patients with systemic JIA had inactive disease, and 77% had no functional limitations. Growth retardation was associated with persistently high disease activity and continuing treatment with systemic glucocorticoids. At the 3-year follow-up, one-third of patients were still being treated with systemic glucocorticoids. The proportion of patients with inactive disease has increased over the past decade. Possible explanations may include improved access to specialized care, additional treatment options, and earlier or faster step-up treatment. However, challenges in the management of systemic JIA remain, as ∼30% of patients continue to present with ongoing active disease.

Suicide in the Early Stage of Schizophrenia.

Suicide is a relevant leading cause of death among patients affected by schizophrenia. Even if suicidal ideation may be present in different stages of disease, some differences have been described between the risk of suicide in patients experiencing first episode of psychosis and those with long-term schizophrenia. It is particularly higher during the first year of illness and reaches a steady decline over the following years. Suicidal ideation and attempts may also be common among subjects with subthreshold psychotic experiences. Factors associated with the risk of suicide in the early phase of schizophrenia are previous suicidal attempts and social aspects: the lack of social support and stable relationships, social drift after the first episode, and social impairment. Also, several psychotic symptoms (suspiciousness, paranoid delusions, mental disintegration and agitation, negative symptoms, depression and hopelessness, and command hallucinations) and substance abuse are associated with higher risk of suicide. It has been described that perfectionism and good levels of insight among individuals who have recently developed psychotic symptoms are significantly associated with higher numbers of suicidal attempts. Moreover, recent evidences show that prefrontal cortex-based circuit dysfunction may be related to suicide in the early stage of schizophrenia. This narrative review summarizes available evidences on suicide in the early stage of schizophrenia and deals with issues to be further studied and discussed.

Emergency Conditions in the Patients with Myasthenia Gravis. Clinical Case of Complicated Course of Generalized Myasthenia Gravis after Thymthymomectomy

Myasthenic crisis may be defined as respiratory failure or delayed extubation after surgical treatment for more than 24 hours resulting from myasthenic weakness. Myasthenic crisis results from weakness of upper airway muscles leading to obstruction and aspiration, weakness of respiratory muscles leading to reduced tidal volumes, or weakness of both muscle groups. About 1/5 of patients with myasthenia gravis experience crisis, usually within the first year of illness. Over the last four decades, prognosis from myasthenic crisis has dramatically improved from a mortality rate of 75 % to the current rate of less than 5 %. Myasthenic crisis should not be fatal, as long as patients receive timely respiratory support and appropriate immunotherapy to reduce myasthenic weakness of the respiratory muscles. In the last years there are more reports about the development of urgent conditions in patients with myasthenia gravis in the early postoperative period after thymectomy. Long-term follow-up monitoring for the study effects of thymectomy in patients with myasthenia gravis, conducted in the department of thoracic surgery of State Institution «Institute of General and Emergency Surgery named after V.T. Zaitsev of National Academy of Medical Sciences of Ukraine» showed that the incidence of early postoperative myasthenic crisis amounted 9.2–12.3 % of cases and was associated with the degree of compensation of bulbar and respiratory disorders before the surgery. This reflects the importance of an adequate selection of the dose of anticholinesterase drugs in the preoperative stage for effective compensation neurosomatic disorders and favorable postoperative prognosis. As an example, the complicated course of generalized myasthenia gravis after thymthymomectomy was described.

Natural history of muscle cramps in amyotrophic lateral sclerosis.

Muscle cramping is a common symptom in amyotrophic lateral sclerosis (ALS) that lacks efficacious treatment. The natural history of this symptom is unknown, which hampers efforts to design optimal clinical trials. We surveyed early stage ALS patients about their experience with cramps each month by phone for up to 21 months. Cramps developed in 95% of patients over the course of their disease. The number of cramps experienced by an individual varied widely from month-to-month and trended lower after the first year of illness (P = 0.26). Those with limb-onset and age >60 years had more cramps than bulbar-onset (P < 0.0001) and younger patients (P < 0.0001). The high variability of the number of cramps experienced suggests that clinical trials will need to use crossover designs or large numbers of participants, even when the treatment effect is substantial.

AB0727 Bone Mineral Density Depending on the Activity of Ankylosing Spondylitis in Men

Backgroundinfringement of a metabolism of bone tissue caused by inflammation, increases the risk of developing osteoporosis (OP) and can be considered as an indicator of “seriousness” of the disease and...

The myasthenic patient in crisis: an update of the management in Neurointensive Care Unit

Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular transmission leading to generalized or localized muscle weakness due most frequently to the presence of autoantibodies against acetylcholine receptors in the postsynaptic motor end-plate. Myasthenic crisis (MC) is a complication of MG characterized by worsening muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation. It also includes postsurgical patients, in whom exacerbation of muscle weakness from MG causes a delay in extubation. MC is a very important, serious, and reversible neurological emergency that affects 20-30% of the myasthenic patients, usually within the first year of illness and maybe the debut form of the disease. Most patients have a predisposing factor that triggers the crisis, generally an infection of the respiratory tract. Immunoglobulins, plasma exchange, and steroids are the cornerstones of immunotherapy. Today with the modern neurocritical care, mortality rate of MC is less than 5%.

Advances in the understanding and challenges facing the management of first-episode schizophrenia

Symptoms of schizophrenia typically first appear in adolescence, a time of transition involving biological, social and emotional changes. Schizophrenia and related psychotic illnesses can significantly impair educational and social development; consequently, early identification and treatment is paramount and can affect long-term outcome. Singh (2010) states that early intervention is cost effective and does ameliorate the course of early psychosis, referring specifically to specialist early intervention teams, and their impact on longterm disease outcome and prognosis. Much research is now available in this area, and the papers selected for this supplement focus on intervention at this critical stage, related side effects as well as mortality risk factors associated with schizophrenia. The stress–vulnerability model for schizophrenia suggests that the onset and course of schizophrenia may be determined by an underlying vulnerability, with clinical manifestation being modulated by environmental stressors. It has long been recognized, since the days of Kraepelin (1913), that people with schizophrenia are more likely to be obese and to suffer from metabolic problems such as diabetes mellitus. Leonard et al. review evidence concerning a disordered immune system in schizophrenia and postulate that the relationship between the therapeutic benefits of antipsychotics, the pathology of schizophrenia and these metabolic syndromes are linked to chronic, low-grade inflammation. As maternal infections have been implicated as a causative factor, the resulting inflammatory changes could contribute to neurodevelopmental difficulties, and a sensitized immune system that activates in adolescence. Leonard et al. also discuss the important role played by pro-inflammatory cytokines which modulate neuronal action, differentiation and survival during neurodevelopment, and more specifically influence activity and survival of neurons that utilize neurotransmitters such as serotonin, glutamate and dopamine. Interleukin-6 is particularly important in schizophrenia as it could augment dopaminergic function which, in turn, would affect psychotic symptoms. In addition to the dopaminergic system, some pro-inflammatory cytokines enhance the activity of the glutamatergic system, via the tryptophan–kynurenine pathway, and this might contribute to the cognitive decline sometimes seen in schizophrenia. The inflammation hypothesis of schizophrenia raises the possibility of using anti-inflammatory drugs, such as COX-2 inhibitors, as adjuncts to traditional antipsychotic medication. Van Haren et al. find further evidence to suggest that schizophrenia is a progressive brain disease, and that the most significant decline in grey matter volume occurs in the critical first year of illness. Indeed, there appears to be a linear correlation of grey matter loss with age in schizophrenia, as opposed to the exponential decline seen in healthy individuals. Van Haren et al. also suggest there is a link between the extent of volume loss and severity of symptoms and social functioning, but do acknowledge other studies refute this theory. Although antipsychotic medication remains a confounding factor for grey matter loss, studies in first-degree relatives and at-risk individuals show that cumulative antipsychotic intake can only explain some of these brain volume changes. This review highlights the need for further large studies that follow patients over a long period of time, to further clarify the relationship between brain volume change, antipsychotic intake and outcome. Indeed, the potential to attenuate brain volume changes could potentially improve long-term outcome, which again highlights the need for early intervention and effective treatment in this morbid disease. The advent of ‘atypical’, or second generation antipsychotics, engendered a therapeutic optimism in the treatment of first-episode patients (NICE, 2002). Taylor et al. reviewed the effectiveness of antipsychotic medications in first episode schizophrenia and found traditional randomized controlled trials (RCTs), whilst being necessary, lacked generalizability to real-world clinical scenarios. A need for long-term observational studies to complement the RCTs was recognized, and several high-profile studies have now been published, often using discontinuation rate of antipsychotic medication as the primary outcome. The rationale behind this is that a

Plasma antioxidant capacity is reduced in Asperger syndrome

Recent evidence suggests that children with autism have impaired detoxification capacity and may suffer from chronic oxidative stress. To our knowledge, there has been no study focusing on oxidative metabolism specifically in Asperger syndrome (a milder form of autism) or comparing this metabolism with other psychiatric disorders. In this study, total antioxidant status (TAOS), non-enzymatic (glutathione and homocysteine) and enzymatic (catalase, superoxide dismutase, and glutathione peroxidase) antioxidants, and lipid peroxidation were measured in plasma or erythrocyte lysates in a group of adolescent patients with Asperger syndrome, a group of adolescents with a first episode of psychosis, and a group of healthy controls at baseline and at 8–12 weeks. TAOS was also analyzed at 1 year. TAOS was reduced in Asperger individuals compared with healthy controls and psychosis patients, after covarying by age and antipsychotic treatment. This reduced antioxidant capacity did not depend on any of the individual antioxidant variables measured. Psychosis patients had increased homocysteine levels in plasma and decreased copper and ceruloplasmin at baseline. In conclusion, Asperger patients seem to have chronic low detoxifying capacity. No impaired detoxifying capacity was found in the first-episode psychosis group in the first year of illness.

Religious coping strategies in patients diagnosed with breast cancer in the UK

The use of religious/spiritual coping strategies may be particularly prevalent when dealing with the stress of a cancer diagnosis. There has, however, been very little research conducted on this topic outside the USA. Existing measures of coping largely ignore the complexity of religious/spiritual coping and its potential to be adaptive as well as maladaptive. The aim of this study was to examine the prevalence of various religious coping strategies in a UK cancer sample. A longitudinal design assessed religious coping strategies in patients newly diagnosed with breast cancer at the time of surgery and at 3 and 12 months post surgery. We recruited 202 patients of which, at 12 months, 160 remained. A non-religious coping measure was included for comparison. The use of religious coping strategies was overall common; up to 73% of patients used positive religious coping to some degree at surgery and up to 53% experienced various religious/spiritual struggles. The use of some religious coping strategies showed differing patterns of change across time while others remained stable. Using religious/spiritual resources in the coping process during the early stages of breast cancer appears common in the UK. Patients may benefit from having their spiritual needs addressed as experiencing some form of religious/spiritual struggle may serve as a barrier to illness adjustment. Health-care professionals should also be aware that some religious coping strategies may be more prevalent at different times during the first year of illness.