Satisfaction with quality of life varies with temperament types of patients with schizophrenia

We sought to explore the relationships of three temperament factors with domain-specific subjective quality of life (QOL) of patients with schizophrenia. Ninety patients with schizophrenia were evaluated using the Quality of Life Enjoyment and Life Satisfaction Questionnaire, the Tridimensional Personality Questionnaire, the Positive and Negative Syndromes Scale, the Distress Scale for Adverse Symptoms, the Insight and Treatment Attitudes Questionnaire, the Insight Self-Report Scale, and standardized questionnaires for self-reported emotional distress and stress process-related variables. Predictors of domain-specific QOL were identified using multiple regression techniques. Temperament factors explain 6% to 16% of variability in QOL domain scores among patients with schizophrenia after controlling for the remaining variables (emotional distress, social support, self-esteem, avoidance coping, age, side effects, and depression). We found that higher levels of novelty seeking are associated with better general QOL, physical health, and more positive subjective feelings, whereas higher levels of reward dependence are related to better satisfaction from social relationships. Higher levels of harm avoidance are associated with poorer satisfaction with general activities, and medication. Thus, temperament factors, as assessed by the Tridimensional Personality Questionnaire, substantially influence satisfaction with life quality in schizophrenia. Novelty seeking, reward dependence, and harm avoidance are associated with different domains of QOL.     

Treatment of patients with tardive dystonia with olanzapine

Tardive dystonia represents a complication of long-term use of neuroleptics and its treatment is often unsatisfactory. Atypical neuroleptics appear to improve tardive dystonia, and cases of tardive dystonia successfully managed with clozapine have been reported. The aim of this open-label video-blinded study was to evaluate the antidystonic efficacy of olanzapine, a new atypical neuroleptic with a low risk of agranulocytosis, in a group of four patients (one man and three women) with tardive cervical dystonia. They developed severe dystonia after several years of neuroleptic treatment. Extensive laboratory evaluations, as well as neurophysiologic and neuroradiologic investigations, were negative. Olanzapine was started at a dose of 5 mg/d and increased up to 7.5 mg/d. All patients were evaluated at baseline and after 2, 4, 8, and 12 weeks of treatment, using the Toronto Western Spasmodic Torticollis Rating Scale, and videotaped. At the end of the trial, the videotapes were reviewed and scored by a blind observer. A self-rating visual analog scale completed the disability evaluation.A moderate to marked improvement in dystonia was observed in all patients, and significant differences were observed in Toronto Western Spasmodic Torticollis Rating Scale scores and videotape ratings after 8 and 12 weeks of treatment compared with the basal values (p < 0.05). The average percentage of improvement in Toronto Western Spasmodic Torticollis Rating Scale score and visual analog scale was 26.4% and 42.6%, respectively. No serious side effects were reported at the maximum dosage reached (7.5 mg/d). This study warrants a larger controlled study to conclusively demonstrate the efficacy of olanzapine in tardive dystonia.     

Compliance with health regimens of adolescents with epilepsy

The purpose of this paper was to describe the compliance of adolescents with epilepsy and some factors connected to it. Altogether 300 individuals with epilepsy aged 13-17 years were randomly selected from the Finnish Social Insurance Institution's register. Every fifth person on the list was included in the sample. Seventy-seven per cent (n= 232) of the selected adolescents with epilepsy returned a questionnaire sent to them relating to compliance. The data were analysed using the SPSS software. Twenty-two per cent of the adolescents with epilepsy felt that they complied fully with their suggested health regimens, while 44% placed themselves in the category of "satisfactory compliance", and the remaining 34% reported poor compliance. Compliance with their recommended life-style was poorest, while the highest degree of compliance was recorded for medication. Background variables, such as the duration of the disease, exercise, smoking, alcohol-intake and the number of seizures, were statistically significantly related to compliance (P< 0.001). Good motivation, a strong sense of normality, experience of results, subjective outcome, energy and will-power, support from parents, physicians and nurses, and a positive attitude towards to the disease and its treatment, no threat to social and emotional well-being and no fears of complications and no fear of seizures explained good compliance (P< 0.001).     

血管内栓塞治疗未破裂脑动静脉畸形并发癫痫患者的预后分析

目的分析血管内栓塞治疗未破裂脑动静脉畸形(CAVM)并发癫痫患者的预后情况。方法选择2013年3月至2017年6月收治的符合诊断标准的CAVM并发癫痫发作患者49例为研究对象,分析血管内栓塞治疗后患者的临床症状、生活质量(QOLIE-31)改善情况。结果患者经血管内栓塞治疗后,QOLIE-31各项指标(除了药物影响)评分均明显提高,高于治疗前(P0.05);Spetzler-Martin分级与Engel分级的I~II级例数多于治疗前(P0.05),同时Spetzler-Martin分级I~II级生活质量评分(76.04±18.33)分明显高于III~V级的(65.65±16.76)分(P0.05);Engel分级I~II级的生活质量评分(75.25±17.78)分明显高于III~V级的(66.23±13.22)分(P0.05);血管内栓塞比例80%的生活质量总评分(78.37±18.87)分明显高于栓塞比例80%的(64.16±16.92)分(P0.05);术后患者的头疼症状中重度例数明显低于治疗前(P0.01);患者的NIHSS评分和MRS评分均明显低于治疗前,头疼症状的生活质量评分高于治疗前(均P0.05)。结论血管内栓塞能明显改善未破裂脑动静脉畸形并发癫痫患者的头疼症状、癫痫发作情况、神经功能缺损,提高血管内栓塞比例能够提高患者生活质量。     

Mortality of patients with parkinson's disease treated with levodopa

Summary The effect of levodopa on the mortality of patients with Parkinson's disease was investigated in 349 patients treated with levodopa or levodopa combined with a decarboxylase inhibitor during 1969–1975 inclusive. During the study period, 61 patients died. The expected mortality was 32.99 resulting in a ratio of actual to expected deaths of 1.85. The excess mortality was accounted for by patients with a severe disease at entry and especially, by the less favorable effect of levodopa treatment than in the living patients. In comparison with the prelevodopa era, the reduction of mortality and the increase of life expectancy of patients with Parkinson's disease during levodopa treatment possibly reflect the decrease of the early mortality due to Parkinson's disease.

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Zusammenfassung Die Auswirkung der Levodopa-Behandlung auf die Mortalität von Parkinson-Patienten wurde anhand einer Serie von 349 Fällen untersucht, welche in den Jahren 1969–1975 einerseits mit L-Dopa, andererseits mit L-Dopa zusammen mit Decarboxylasehemmern behandelt wurden. Während der Beobachtungsperiode verstarben 61 Patienten. Die erwartete Mortalität hätte 32,99 betragen müssen, was eine Relation von tatsächlicher zu erwarteter Mortalität von 1,85 ergibt. Für die höhere Mortalität waren Fälle verantwortlich mit schweren Krankheitserscheinungen bei Beginn der Therapie und im besondern auch mit einem geringeren Effekt der L-Dopa-Therapie als bei den überlebenden Patienten. Verglichen mit den Beobachtungen vor Einführung der L-Dopa-Therapie beruht wohl die Verminderung der Mortalität und die erhöhte Lebenserwartung von Parkinson-Patienten unter L-Dopa auf der Abnahme der Frühtodesfälle durch die Parkinson'sche Krankheit.

     

Factors associated with stigmatization of persons with mental illness

Stigmatization of individuals with mental illnesses is widespread and serves as a major barrier to treatment. In a survey of 116 undergraduates, the authors examined the impact of diagnosis, attitudes about treatment, and psychiatric terminology on stigma associated with mental illness. Stigmatization of schizophrenia was significantly higher than stigmatization of depression. More positive attitudes toward treatment were associated with significantly less stigma. However, psychiatric terminology had no impact on attitudes toward mental illness. Significantly less stigmatization of mental illness was found among females than among males. Reducing the stigmatization of mental illness continues to be an important goal for mental health professionals.     

Family studies of individuals with eyelid myoclonia with absences

Purpose: Eyelid myoclonia with absences (EM) is an uncommon type of absence seizure associated with a variety of epilepsy syndromes. The syndrome of epilepsy with EM (EMA) has been proposed to denote the onset of frequent EM induced by eye closure and photic stimulation beginning in childhood. The clinical genetics of EMA has not been well characterized, although a family history of seizures is not infrequent. Methods: Individuals with EMA were ascertained by referral and through the investigators’ clinical practices. All available family members were assessed for seizures using a validated seizure questionnaire. Electroclinical data were obtained on each proband and all affected family members; pedigrees were constructed. Families were analyzed for phenotypic patterns. Key Findings: Eighteen individuals with EMA were recruited. A history of seizures was found in 34 relatives in 15 (83%) of 18 families. In terms of epilepsy syndromes, 9 relatives from 7 of 15 families had febrile seizures. Two relatives had EMA. Classical genetic generalized epilepsy (GGE) syndromes were seen in five relatives: two generalized tonic–clonic seizures alone, two childhood absence epilepsy (CAE), and one juvenile myoclonic epilepsy (JME). Genetic epilepsy with febrile seizures plus (GEFS+) phenotypes occurred in 16 relatives. On review of the epilepsy syndromes within each family, seven families had a pattern consistent with GEFS+, whereas three families had classical GGE. Significance: The clinical genetics of EMA is suggestive of complex inheritance with shared genetic determinants overlapping with both classical GGE and GEFS+. The epilepsy syndromes in relatives of probands with EMA differ from those found in families of probands with CAE, supporting the concept that patients with EMA have a syndrome that is distinct from CAE. This presumably reflects different genetic components contributing to their genetic architecture.     

Collaborative design of accessible information with people with aphasia

ABSTRACT

Background: People with aphasia report preferences for specially formatted health information materials, but there is little evidence that modified materials result in improved comprehension. Potential explanations for this include language included not taking account of aphasic processing difficulties, topics unrelated to aphasia, lack of clarity regarding the use of images, and the lack of end-user involvement in the design. Additionally, no definitive criteria for production of accessible information have been identified.

Aims: The first aim of this study was to collaborate with people with aphasia in an iterative design process to develop and finalise accessible information materials. The second aim was to identify definitive criteria for use in the future production of information materials for people with aphasia.

Methods and procedure: Prototype materials were developed for the study, based on criteria identified from the existing research into aphasia-accessible information, and on the evidence base concerning language processing in aphasia. Fourteen people with aphasia took part in two rounds of consensus group meetings and viewed information about aphasia presented within the prototype materials. Consensus points were identified within the groups through discussion and through ratings using Likert scales. The set of consensus points and ratings were adapted into criteria for graphic designers to incorporate into subsequent designs of the materials, in order to generate a final version, and related criteria.

Outcomes and results: The group discussions and the ratings of materials led to the identification of an agreed layout within which to present information, and specific criteria for the following: information consisting of one proposition expressed via everyday words and canonical syntactic forms; one or two images relating directly to keywords; sans serif typography with keyword emphasis. Individual preferences with regard to image types were identified. Novel criteria were identified in the study, relating to layout, language, images and typography. These were added to the original set of criteria to form definitive criteria for use in the development of accessible aphasia materials.

Conclusions: This study successfully involved people with aphasia in the design process to produce novel materials, and related design criteria. The resulting materials and criteria differ from those previously proposed, by reflecting directly people with aphasia’s views and preferences, and by incorporating language and images suitable for people with aphasia, based on the existing research evidence and the outcomes of this study. The materials and criteria have the potential to improve people with aphasia’s understanding of health information.     

Initial therapy of patients with Wilson's disease with tetrathiomolybdate

Patients with Wilson's disease who present with acute neurological symptoms often become clinically worse when initially treated with penicillamine. Other available anticopper drug therapies do not appear to offer a solution to this treatment problem. We are developing and evaluating a new drug, ammonium tetrathiomolybdate for this purpose. Theoretically, tetrathiomolybdate has optimal properties, including an immediate blockade of copper absorption and the property of forming complexes with copper in the blood, rendering the copper nontoxic. In this article, we present results from six patients treated with tetrathiomolybdate for up to 8 weeks as initial therapy. None of the five patients who had presented with acute neurological symptoms worsened. Also presented are methods of assay, preliminary stability studies, and methods of evaluating therapeutic end points with respect to copper metabolism.     

Relapsing polychondritis with features of dementia with Lewy bodies

We describe a 72-year old man with clinical features suggestive of dementia with Lewy bodies (DLB) who proved neuropathologically to have degeneration induced by relapsing polychondritis (RP), an autoimmune inflammatory disorder of cartilaginous tissues. There was lymphocytic infiltration of the leptomeninges, perivascular cuffing, reactive astrocytosis, and activation of microglia in multiple brain areas all consistent with an immunologically mediated process. There was widespread neuronal loss within the hippocampus, entorhinal cortex, and amygdala as well as diffuse myelin pallor of cortical pathways. Elevated levels of complement proteins and endothelial markers of inflammation were observed, which are similar to previous reports in DLB. This study demonstrates that qualitatively similar inflammation-associated neurodegeneration is present in widespread regions of the brain in a RP case presenting clinically as DLB.Senior authors Arnold Starr and David H. Cribbs contributed equally to this project.Funding supported by ADRC P50 AG16573, NIA-AG-20241 P50 AG000658, and NINDS-NS50895 (DHC)     

Pathomechanism of dyskinesia associated with treatment of Parkinson disease with levodopa

The 5-HIAA/HVA ratio was determined in the cerebrospinal fluid in 5 patients with Parkinson's disease with L-DOPA preparations in which drug-induced dyskineses developed, in 5 patients treated with L-DOPA without dyskineses, and 10 controls. A decrease in the 5-HIAA/HVA ratio was observed in the group with dyskineses as compared with patients without dyskineses and in controls. The directions of further investigations on hte pathomechanism of drug-induced dyskineses are discussed.