Home About us Contact | |||
Long-term Course (long-term + course)
Selected AbstractsLong-term course and mutational spectrum of spatacsin -linked spastic paraplegiaANNALS OF NEUROLOGY, Issue 6 2007Ute Hehr MD Objective Hereditary spastic paraplegias (HSPs) comprise a heterogeneous group of neurodegenerative disorders resulting in progressive spasticity of the lower limbs. One form of autosomal recessive hereditary spastic paraplegia (ARHSP) with thin corpus callosum (TCC) was linked to chromosomal region 15q13-21 (SPG11) and associated with mutations in the spatacsin gene. We assessed the long-term course and the mutational spectrum of spatacsin -associated ARHSP with TCC. Methods Neurological examination, cerebral magnetic resonance imaging (MRI), 18fluorodeoxyglucose positron emission tomography (PET), nerve biopsy, linkage and mutation analysis are presented. Results Spastic paraplegia in patients with spatacsin mutations (n = 20) developed during the second decade of life. The Spastic Paraplegia Rating Scale (SPRS) showed severely compromised walking between the second and third decades of life (mean SPRS score, >30). Impaired cognitive function was associated with severe atrophy of the frontoparietal cortex, TCC, and bilateral periventricular white matter lesions. Progressive cortical and thalamic hypometabolism in the 18fluorodeoxyglucose PET was observed. Sural nerve biopsy showed a loss of unmyelinated nerve fibers and accumulation of intraaxonal pleomorphic membranous material. Mutational analysis of spatacsin demonstrated six novel and one previously reported frameshift mutation and two novel nonsense mutations. Furthermore, we report the first two splice mutations to be associated with SPG11. Interpretation We demonstrate that not only frameshift and nonsense mutations but also splice mutations result in SPG11. Mutations are distributed throughout the spatacsin gene and emerge as major cause for ARHSP with TCC associated with severe motor and cognitive impairment. The clinical phenotype and the ultrastructural analysis suggest a disturbed axonal transport of long projecting neurons. Ann Neurol 2007 [source] The phenomenology of bipolar disorder: what drives the high rate of medical burden and determines long-term prognosis?DEPRESSION AND ANXIETY, Issue 1 2009Isabella Soreca M.D. Abstract Bipolar disorder (BD) has been classically described as one of episodic mood disturbances. New evidence suggests that a chronic course and multisystem involvement is the rule, rather than the exception, and that together with disturbances of circadian rhythms, mood instability, cognitive impairment, a high rate of medical burden is often observed. The current diagnostic approach for BD neither describes the multisystem involvement that the recent literature has highlighted nor points toward potential predictors of long- term outcome. In light of the new evidence that the long-term course of BD is associated with a high prevalence of psychiatric comorbidity and an increased mortality from medical disease, we propose a multidimensional approach that includes several symptom domains, namely affective instability, circadian rhythm dysregulation, and cognitive and executive dysfunction, presenting in various combinations that give shape to each individual presentation, and offers potential indicators of overall long-term prognosis. Depression and Anxiety, 2009. © 2008 Wiley-Liss, Inc. [source] The heterogeneity of causes and courses of attention-deficit/hyperactivity disorderACTA PSYCHIATRICA SCANDINAVICA, Issue 5 2009H-C. Steinhausen Objective:, Attention-deficit / Hyperactivity Disorder (ADHD) is a frequent mental disorder with onset in childhood and persistence into adulthood in a sizeable number of people. Despite a rather simple clinical definition, ADHD has many facets because of frequent co-morbid disorders and varying impact on psychosocial functioning. Thus, there is considerable heterogeneity in various domains. Method:, A review of recent research findings in: i) selected domains of aetiology reflecting the role of genes, brain structures and functioning and the interplay of causal factors and ii) clinical heterogeneity in terms of co-morbidities, gender effects, courses and outcomes. Results:, Molecular genetic studies have identified a number of candidate genes which have a small effect on behavioural variation in ADHD. In the most recent Genome Scan Meta Analysis of seven ADHD linkage studies, genome-wide significant linkage was identified on chromosome 16. The volume of both the total brain and various regions including the prefrontal cortex, the caudate nucleus and the vermis of the cerebellum is smaller in ADHD. Functional MRI has documented a specific deficit of frontostriatal networks in ADHD. Integrative aetiological models have to take the interaction of gene and environment on various dysfunctions into account. Clinical heterogeneity results from frequent associations with various co-morbidities, the impact of the disorder on psychosocial functioning, and gender effects. Partly, these effects are evident also in the course and outcome of ADHD. Conclusion:, ADHD is a chronic mental disorder with a complex aetiology. So far, various neurobiological factors have been identified that need to be studied further to better understand their interaction with environmental factors. The clinical presentation and the long-term course of ADHD are manifold. [source] How many well vs. unwell days can you expect over 10 years, once you become depressed?ACTA PSYCHIATRICA SCANDINAVICA, Issue 4 2009T. A. Furukawa Objective:, Prognostic studies of major depression have mainly focused on episode remission and relapse, and only a limited number of studies have examined long-term course of depressive symptomatology at threshold and subthreshold levels. Method:, The Group for Longitudinal Affective Disorders Study has conducted prospective serial assessments of a cohort of heretofore untreated major depressive episodes for 10 years under naturalistic conditions. Results:, Of the 94 patients in the cohort, the follow-up rate was 70% of the 11 280 person-months. Around 77% of the follow-up months were spent in euthymia, 16% in subthreshold depression and 7% in major depression. Duration of the index episode before reaching recovery was the only significant predictor of the ensuing well time. Conclusion:, On average, patients with major depression starting treatment today may expect to spend three quarters of the next decade in euthymia but the remaining one quarter in subthrehold or threshold depression. [source] Five-year follow-up during antipsychotic treatment: efficacy, safety, functional and social outcomeACTA PSYCHIATRICA SCANDINAVICA, Issue 2007E. Lindström Objective:, Explore the long-term course of schizophrenia and related disorders. Method:, Naturalistic study of 225 patients initially treated with risperidone (monotherapy or in combination with other psychotropic drugs) over 5 years. Results:, Stable symptomatology and side effects were observed. Clinician GAF scores were 55,61, but patients' self-ratings were higher. Clinician and patient CGI scores were at the same level. Annual in-patient days decreased but days in sheltered accommodations increased still more. Only 12% of the patients studied or worked full-time. One in four had no social contacts except with staff. Eight patients died during the 5 years. Conclusion:, The findings underline the chronicity and seriousness of psychotic disorders in terms of social outcome and, indirectly, the low quality of life of this group of persons. Patients were generally well aware of their illness and able to sort out symptoms from drug side effects. This opens for more active involvement of patients in monitoring their own treatment. [source] A follow-up study of juvenile obsessive,compulsive disorder from IndiaACTA PSYCHIATRICA SCANDINAVICA, Issue 6 2003Y. C. Janardhan Reddy Objective:, To study the long-term course and outcome of juvenile obsessive,compulsive disorder (OCD). Method:, Two to 9-year follow-up of largely self-referred, drug-naïve subjects (n = 58) by employing catch-up longitudinal design. Results:, The mean follow-up period was 5 years. Nearly three-fourth of the sample was adequately treated with medications. Only 21% of the subjects had clinical OCD at follow-up and 48% were in true remission (no OCD and not on treatment). Earlier age-at-onset was associated with better course and outcome. Conclusion:, Juvenile OCD has favorable outcome. Our findings are applicable to psychiatric hospital settings in India and perhaps to the general psychiatric settings in the Western countries. Whether the better outcome in this sample is the result of differing clinical characteristics or because of true cross-cultural variation in the course needs further exploration. It is speculated that early onset OCD could be a subtype of juvenile OCD with better outcome. [source] University of Michigan Addiction Research Center (UMARC): development, evolution, and directionADDICTION, Issue 6 2010Robert A. Zucker ABSTRACT A historical summary is provided of the evolution of the University of Michigan Addiction Research Center (UMARC) since its origins in 1988. Begun as an National Institutes of Health (NIH) research center within a Department of Psychiatry and focused solely upon alcohol and aging, early work emphasized treatment efficacy, differential outcome studies and characterization of the neurophysiological and behavioral manifestations of chronic alcoholism. Over the last 15 years, UMARC has extended its research focus along a number of dimensions: its developmental reach has been extended etiologically by studies of risk early in the life span, and by way of work on earlier screening and the development of early, brief treatment interventions. The addiction focus has expanded to include other drugs of abuse. Levels of analysis have also broadened, with work on the molecular genetics and brain neurophysiology underlying addictive processes, on one hand, and examination of the role of the social environment in long-term course of disorder on the other hand. Activities have been facilitated by several research training programs and by collaborative relationships with other universities around the United States and in Poland. Since 2002, a program for research infrastructure development and collaboration has been ongoing, initially with Poland and more recently with Ukraine, Latvia and Slovakia. A blueprint for the future includes expanded characterization of the neurobiology and genetics of addictive processes, the developmental environment, as well as programmatic work to address the public health implications of our ability to identify risk for disorder very early in life. [source] Are there empirically supported and clinically useful subtypes of alcohol dependence?ADDICTION, Issue 2006Victor M. Hesselbrock ABSTRACT Aims This paper provides an overview of several multidimensional empirically derived typologies of alcohol use disorders that have been derived primarily for research purposes in relation to their clinical utility. Methods Studies using multivariate statistical methods for identifying homogeneous groups of subjects were selected for inclusion. Theoretically based typologies were not included in this review. Results While formal diagnostic criteria typically identify separate categories of alcohol abuse and dependence, several studies using different statistical methods consistently suggest as many as four homogeneous types of alcoholism: a chronic/severe type, a depressed/anxious type, a mildly affected type and an antisocial type. Conclusions Even though the longitudinal outcomes of few empirically derived subtypes have been examined, alcoholism typologies remain a viable and potentially valuable tool for investigating etiological pathways, the effectiveness of treatments and the long-term course of alcohol use disorders. [source] Positioning biologic agents in the treatment of Crohn's disease,INFLAMMATORY BOWEL DISEASES, Issue 10 2009Stephen B. Hanauer MD Abstract One decade after the emergence of biologic therapy for Crohn's disease (CD), our treatment algorithms are beginning to change. Once reserved for patients with refractory disease, disease unresponsive to conventional therapies, or those requiring multiple courses of corticosteroids, there is increasing evidence that early, aggressive interventions with immunosuppressants or biologic therapies targeting tumor necrosis factor-, or ,-4 integrins can alter the natural history of CD by reducing the transmural complications of structuring and fistulization and the nearly inevitable requisite for surgical resections. More recent trials are beginning to suggest that intervention with combination therapy for selected patients with a poor prognosis may modify the long-term course of CD. Selection of patients with features predicting a complex or progressive course and early, combined intervention is now possible. Future studies are still needed to best identify predictors of response to individual agents with differing mechanisms of action, as well as to optimize the risk-benefit of long-term maintenance therapy. (Inflamm Bowel Dis 2009) [source] Twelve-year course and outcome predictors of anorexia nervosaINTERNATIONAL JOURNAL OF EATING DISORDERS, Issue 2 2006Dipl-Psych, Manfred M. Fichter MD Abstract Objective The current study presents the long-term course of anorexia nervosa (AN) over 12 years in a large sample of 103 patients diagnosed according to criteria in the 4th ed. of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV). Method Assessments were made at the beginning of therapy, at the end of therapy, at the 2-year follow-up, at the 6-year follow-up, and at the 12-year follow-up. Self-rating and an expert-rating interview data were obtained. Results The participation rate at the 12-year follow-up was 88% of those alive. There was substantial improvement during therapy, a moderate (in many instances nonsignificant) decline during the first 2 years posttreatment, and further improvement from 3 to 12 years posttreatment. Based on a global 12-year outcome score, 27.5% had a good outcome, 25.3% an intermediate outcome, 39.6% had a poor outcome, and 7 (7.7%) were deceased. At the 12-year follow-up 19.0% had AN, 9.5% had bulimia nervosa-purging type (BN-P), 19.0% were classified as eating disorder not otherwise specified (EDNOS). A total of 52.4% showed no major DSM-IV eating disorder and 0% had binge eating disorder (BED). Systematic,strictly empirically based,model building resulted in a parsimonious model including four predictors of unfavorable 12-year outcome explaining 45% of the variance, that is, sexual problems, impulsivity, long duration of inpatient treatment, and long duration of an eating disorder. Conclusion Mortality was high and symptomatic recovery protracted. Impulsivity, symptom severity, and chronicity were the important factors for predicting the 12-year outcome. © 2005 by Wiley Periodicals, Inc. [source] Screening for depression and anxiety: correlates of non-response and cohort attrition in the Netherlands Study of Depression and Anxiety (NESDA)INTERNATIONAL JOURNAL OF METHODS IN PSYCHIATRIC RESEARCH, Issue 4 2009Willem Jan Van Der Veen Abstract A major problem in the analysis of attrition of cohorts in studies on mental health problems is that data on those who do not participate at the outset of a study are largely unavailable. It is not known how underlying psychopathology affects the first stages of screening where non-response and selectivity are usually highest. This article presents results of one of the centres of the Netherlands Study of Depression and Anxiety (NESDA), a longitudinal study aimed at describing the long-term course and consequences of depression and anxiety disorders. The aim is to describe the different ways of attrition during the first NESDA-wave in a cohort of patients aged 18,65 years of the Registration Network Groningen and to analyse whether attrition is related to gender, age and psychopathology as recorded in general practice. The attrition of the study cohort (n = 8475) was highest during the first stages, eventually leading to a population of 169 patients only who participated in the full NESDA-programme. Probabilities of transition from one stage of the screening process to the next were regressed on selected background variables using binary logistic regression. Correlates of participation were being female and being older (>40). Psychopathology was an important variable in the formation of the initial sample cohort, but only had a weak influence on patient response to the screening questionnaire. Study design factors had a stronger impact on the changing composition of the cohort at each screening stage compared to patient factors. Copyright © 2009 John Wiley & Sons, Ltd. [source] Rosacea and its management: an overviewJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 3 2005AK Gupta ABSTRACT Background, Rosacea is a chronic inflammatory disorder that affects 10% of the population. The prevalence of rosacea is highest among fair-skinned individuals, particularly those of Celtic and northern European descent. Since a cure for rosacea does not yet exist, management and treatment regimens are designed to suppress the inflammatory lesions, erythema, and to a lesser extent, the telangiectasia involved with rosacea. Objectives, This review outlines the treatment options that are available to patients with rosacea. Methods, Published literature involving the treatment or management of rosacea was examined and summarized. Results, Patients who find that they blush and flush frequently, or have a family history of rosacea are advised to avoid the physiological and environmental stimuli that can cause increased facial redness. Topical agents such as metronidazole, azelaic acid cream or sulfur preparations are effective in managing rosacea. Patients who have progressed to erythematotelangiectatic and papulopustular rosacea may benefit from the use of an oral antibiotic, such as tetracycline, and in severe or recalcitrant cases, isotretinoin to bring the rosacea flare-up under control. Treatment with a topical agent, such as metronidazole, may help maintain remission. Patients with ocular involvement may benefit from a long-term course of an antibiotic and the use of metronidazole gel. A surgical alternative, laser therapy, is recommended for the treatment of telangiectasias and rhinophyma. Patients with distraught feelings due to their rosacea may consider cosmetic camouflage to cover the signs of rosacea. Conclusions, With the wide variety of oral and topical agents available for the effective management of rosacea, patients no longer need to feel self-conscious because of their disorder. [source] Long-term clinical course of patients with acute polymorphic psychotic disorder without symptoms of schizophreniaPSYCHIATRY AND CLINICAL NEUROSCIENCES, Issue 4 2006TAKAAKI ABE md Abstract, Few studies have investigated the long-term course of patients with acute polymorphic psychotic disorder, despite the clinical relevance. The present study focused on the frequency of acute polymorphic psychotic disorder without symptoms of schizophrenia (F23.0) and the prognosis of patients with the disorder over a long period. A total of 388 inpatients with nonaffective psychosis were examined and cases with F23.0 were retrospectively investigated regarding clinical course over 12 years with reference to clinical charts. A total of 16 cases were diagnosed with F23.0 according to first episode. After 12 years, five cases were rediagnosed with schizophrenia, and one case with borderline-type emotionally unstable personality disorder, although the diagnosis of F23.0 was not changed in 10 older patients. Of the four cases with >2 repeated episodes of F23.0, the same clinical picture as in the first episode was observed in three, while one case showed continuous residual symptoms. F23.0 does not represent a homogeneous clinical entity. In some cases, diagnosis changes to schizophrenia, but others display repeated episodes of F23.0. [source] Long-term course and mutational spectrum of spatacsin -linked spastic paraplegiaANNALS OF NEUROLOGY, Issue 6 2007Ute Hehr MD Objective Hereditary spastic paraplegias (HSPs) comprise a heterogeneous group of neurodegenerative disorders resulting in progressive spasticity of the lower limbs. One form of autosomal recessive hereditary spastic paraplegia (ARHSP) with thin corpus callosum (TCC) was linked to chromosomal region 15q13-21 (SPG11) and associated with mutations in the spatacsin gene. We assessed the long-term course and the mutational spectrum of spatacsin -associated ARHSP with TCC. Methods Neurological examination, cerebral magnetic resonance imaging (MRI), 18fluorodeoxyglucose positron emission tomography (PET), nerve biopsy, linkage and mutation analysis are presented. Results Spastic paraplegia in patients with spatacsin mutations (n = 20) developed during the second decade of life. The Spastic Paraplegia Rating Scale (SPRS) showed severely compromised walking between the second and third decades of life (mean SPRS score, >30). Impaired cognitive function was associated with severe atrophy of the frontoparietal cortex, TCC, and bilateral periventricular white matter lesions. Progressive cortical and thalamic hypometabolism in the 18fluorodeoxyglucose PET was observed. Sural nerve biopsy showed a loss of unmyelinated nerve fibers and accumulation of intraaxonal pleomorphic membranous material. Mutational analysis of spatacsin demonstrated six novel and one previously reported frameshift mutation and two novel nonsense mutations. Furthermore, we report the first two splice mutations to be associated with SPG11. Interpretation We demonstrate that not only frameshift and nonsense mutations but also splice mutations result in SPG11. Mutations are distributed throughout the spatacsin gene and emerge as major cause for ARHSP with TCC associated with severe motor and cognitive impairment. The clinical phenotype and the ultrastructural analysis suggest a disturbed axonal transport of long projecting neurons. Ann Neurol 2007 [source] Cardiac troponin T Arg92Trp mutation and progression from hypertrophic to dilated cardiomyopathyCLINICAL CARDIOLOGY, Issue 5 2001Noboru Fujino M.D. Abstract Background: Mutations in the cardiac troponin T gene causing familial hypertrophic cardiomyopathy (HCM) are associated with a very poor prognosis but only mild hypertrophy. To date, the serial morphologic changes in patients with HCM linked to cardiac troponin T gene mutations have not been reported. Hypothesis: The aim of this study was to determine the long-term course of patients with familial HCM caused by the cardiac troponin T gene mutation, Arg92Trp. Results: The Arg92Trp missense mutation was present in 10 individuals from two unrelated pedigrees. They exhibited different cardiac morphologies: three had dilated cardiomyopathy-like features, five had asymmetric septal hypertrophy with normal left ventricular systolic function, one had electrocardiographic abnormalities without hypertrophy, and one had the disease-causing mutation but did not fulfill the clinical criteria for the disease. The mean maximum wall thickness was 14.1 ± 6.0 mm. The three patients with dilated cardiomyopathy-like features had progressive left ventricular dilation. Three individuals underwent right ventricular endomyocardial biopsy. There was a modest degree of myocardial hypertrophy (myocyte diameter: 18.9 ± 5.2 m,m), and minimal myocardial disarray and mild fibrosis were noted. Conclusion: The Arg92Trp substitution in the cardiac troponin T gene shows a high degree of penetrance, moderate hypertrophy, and early progression to dilated cardiomyopathy in Japanese patients. Early identification of individuals with this mutation may provide the opportunity to evaluate the efficacy of early therapeutic interventions. [source] Optimizing open live-donor nephrectomy , long-term donor outcomeCLINICAL TRANSPLANTATION, Issue 3 2004M Schostak Abstract:, Introduction:, The technique of laparoscopic or retroperitoneoscopic donor nephrectomy has been increasingly propagated in recent years. The central advantage is supposed to be a reduction of perioperative discomfort. However, there have not been many reports describing the subjective feeling associated with an open donor nephrectomy, particularly with respect to the pain level in the perioperative and long-term course. This retrospective study examines the perioperative pain and morbidity and long-term outcome of living kidney donors from 35 yr of experience at the University Hospital Benjamin Franklin of the Free University of Berlin. Methods:, A total of 102 living kidney donors were asked to fill out a questionnaire. Five epidemiological questions were posed and the rest dealt mainly with lasting subjective and objective surgical impairments. There were also questions relating to the perioperative pain level (VAS/NAS-Score). In addition, basic information was obtained regarding the donor's current health status (physical examination, serum creatinine; sometimes also ultrasound, protein IU, blood pressure), and/or examinations were performed. Results:, The mean age at the time of donation was 45.5 and 55% were women. Donor nephrectomies were left-sided in 78 cases and right-sided in 24. There was a total complication rate of 53%, but serious complications only occurred in two cases (1.9%). A total of 53 donors could be reached. Although 41.5% felt they had a lasting impairment, somatic sequelae like respiratory, abdominal or scar problems were rare, affecting a maximum of only four patients in each case. Fifteen patients reported neurological problems such as sensory disturbances. The mean serum creatinine was 89.9 ,mol/L in female and 114.2 ,mol/L in male donors. Microalbuminuria was found in 22.6% of the donors, hypertension in 35.8%. Persistent pain was reported by 20.7%, its occurrence being permanent in two of the donors and very frequent in one. All the others rarely have pain. The median perioperative VAS/NAS score was 8 on the first day after surgery, 5 after 1 wk and 1 after 1 month. The analgesia was rated as good or very good by 71%. Everyday life was managed as well as before surgery after 2,4 wk by the highest percentage (42%) of patients, but working capacity was only regained after 1,3 months by a comparable percentage (44%). Forty-six percent had a very good and 33% a good feeling after the kidney donation. The relationship to the recipient had intensified in most cases. Ninety-one percent would again decide in favor of a donation. Conclusion:, Donor nephrectomy in an open technique is a safe and reliable procedure with low morbidity. After a median post-operative period of 7 yr, however, 42% of the donors still report general impairment due to the intervention, although concrete somatic problems were only detected in a few cases. Nearly all these patients underwent surgery in a full flank position. Wound-healing impairments were also significantly more frequent with this surgical technique. This positioning should thus be avoided. The post-operative pain level was relatively high, but a marked improvement was achieved in the course of the observation period by optimizing analgesic management. [source] Prevention and treatment of systemic glucocorticoid side effectsINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 3 2010Siamak Moghadam-Kia MD Background, Systemic glucocorticoids are used in dermatologic practice for various diseases including connective tissue disorders, bullous diseases, and many other dermatologic conditions. Patients with these diseases are at times treated with long-term courses of glucocorticoids, which place them at increased risk for glucocorticoid-induced side effects. Therefore, dermatologists must be knowledgeable of risks related to glucocorticoid use and be familiar with guidelines to manage them. Objective, To provide an update of recent advances in the prevention and treatment of major glucocorticoid-induced side effects. Methods, Review of the literature Results Data regarding the prevention and treatment of glucocorticoid-induced side effects are presented. Conclusion, This review should help dermatologists optimally manage and prevent glucocorticoid-induced side effects. [source] |