Can cognitive and behavioural disorders differentiate frontal variant-frontotemporal dementia from Alzheimer's disease at early stages?

Frontal variant-Frontotemporal dementia (fvFTD) and Alzheimer's disease (AD) patients matched for severity of dementia at the Clinical Dementia Rating (CDR) received neuropsychological testing in order to explore if the dysexecutive disorder might characterise fvFTD at early stage, when AD is dominated by the episodic memory defect. We also determined if the behavioural syndrome was more severe in fvFTD than AD, and if specific patterns of behavioural symptoms could differentiate the two types of dementia, using the Neuropsychiatry Inventory (NPI). AD patients performed worse than fvFTD not only in memory but also in executive tasks. Apathy and eating disorders proved to be more severe or frequent in fvFTD even if the two groups did not differ in the total NPI score. CDR score significantly correlated with the NPI score in fvFTD and with the MMSE in AD. Our data confirm that the memory disorders may differentiate the two types of dementia; however, the dysexecutive syndrome is as severe, and even more severe in AD. The severity of the behavioural syndrome is comparable in the two groups but the nature of the behavioural disorders may vary to some extent. We conclude that AD dementia at early stage is a behavioural-cognitive syndrome, while in fvFTD the behavioural disorders appear when the cognitive deficit is still relatively mild.     

Continuity in childhood depression

It is only in recent years that childhood depression has received widespread attention from mental health specialists. Its status as a clinical syndrome of childhood remains unclear. Many controversies surround various facets of this condition, foremost among these are questions relating to the duration of depressive disorders, the likelihood of recurrence, and the long-term outcome of depressed children. In the course of addressing these issues, attention is devoted to the outcomes as well as the advantages and disadvantages of commonly used research designs (prospective, retrospective, and catch-up prospective), the level of diagnosis used (symptom, syndrome, disorder), the significance of the age of onset on severity, and specific areas in need of further research. Among the tentative conclusions, it is asserted that many depressive symptoms are transient, diminishing or disappearing with age and/or changing environmental circumstances, but that severe depressive disorders do persist, with periods of remission, at least into the early adult years.     

Episodic manifestation of hemiparkinson syndrome with severe dementia personality change and precursors of paranoid hallucination symptoms

A case of episodic manifestation of semiparalysis agitans is described, accompanied by severe demential personality change and precursory hallucinatory symptoms, which is made the basis for the discussion of aspects of mental disorders, notably dementia and symptoms resembling schizophrenia, in Parkinsonian patients. By way of allusion to Glass, a diagnosis including a very extensive, complex, symptomatology is made of a Parkinsonian syndrome.     

Deep dyslexia in the two languages of an Arabic/French bilingual patient.

We present a single case study of an Arabic/French bilingual patient, ZT, who, at the age of 32, suffered a cerebral vascular accident that resulted in a massive infarct in the left peri-sylvian region. ZT's reading displays the characteristics of the deep dyslexia syndrome in both languages, that is, production of semantic, visual, and morphological errors, and concreteness effect in reading aloud and impossibility of reading nonwords. In the first part of this paper, using a three-route model of reading, we account for the patient's performance by positing functional lesions, which affect the non-lexical, the semantic lexical and the non-semantic lexical routes of reading. Phonological priming observed in a cross-language visual lexical decision task indicates that implicit assembled phonological recoding is possible. The above lesions and implicit nonword reading characterize the output form of deep dyslexia. However, error distribution reveals dissociations across languages (e.g. the semantic error rate is higher in French whereas translations are more frequent in the Arabic testing) that cannot be accounted for within a three-route model. In the second part, extensions to Plaut and Shallice's connectionist model (Cognitive Neuropsychology, 10 (5) (1993) 377) are proposed to account for the translinguistic errors observed. ZT's error distribution is compared to that obtained by Plaut and Shallice after lesions had been applied at different locations through the 40-60 network. The overall syndrome of deep dyslexia found in both languages is explained as resulting from lesions along the direct (O-->I) and output (S-->Ip, Ip-->P) pathways of reading. Lesions along the output pathway mostly affecting S-->Ip connections in French and Ip-->P connections in Arabic account for discrepancies in ZT's error pattern across tasks and languages. This case study demonstrates the superiority of a connectionist approach for predicting the error pattern in deep dyslexia.     

BDNF serum levels in subjects developing or not post-traumatic stress disorder after trauma exposure

Post-traumatic stress disorder (PTSD) is a syndrome resulting from exposure to a severe traumatic event that poses threatened death or injury and produces intense fear and helplessness. The neural structures implicated in PTSD development belong to the limbic system, an important region for emotional processing. Brain-derived neurotrophic factor (BDNF) is a neurotrophin that serves as survival factor for selected populations of central nervous system (CNS) neurons and plays a role in the limbic system by regulating synaptic plasticity, memory processes and behavior. Impaired BDNF production in the brain can lead to a variety of CNS dysfunctions including symptoms associated with PTSD. However, so far fewer studies have investigated this neurotrophin in patients with PTSD. Furthermore, given the multiple role of BDNF in various CNS disorders, it cannot be excluded that traumatic events per se may influence neurotrophin levels, without a direct association to the PTSD syndrome.     

Abh?ngigkeitserkrankungen und Pers?nlichkeitsst?rungen

Substance use disorders and personality disorders often co-occur in clinical treatment. In this review the association between substance use disorders and personality disorders is described and discussed. Clinical and neurobiological data as well as psychodynamic concepts are included to focus on the therapeutic implications for patients with these comorbidities. The consumption of substances often begins as a kind of self-medication. Patients suffering from severe personality disorders need these substance effects to avoid severe destructive affects and to regulate their self-esteem. Patients with chronic substance dependence often show changes in brain structure. In the dependency stage of addiction clinical symptoms can make the diagnosis of a specific personality disorder very difficult. The treatments can be classified by the severity of substance use disorders and personality disorders. Substance misuse can be treated with psychotherapy. In substance dependence, specific forms of addiction therapy should be included. In patients with the frequent comorbidity of substance use disorders and severe personality disorders, disorder-specific treatment can be used with good results.     

Language phenotypes and intervention planning: bridging research and practice

This paper focuses on the communication and language phenotypes associated with three genetic disorders: Down syndrome, Williams syndrome, and fragile X syndrome. It is argued that there is empirical evidence that these disorders predispose children to specific profiles of strength and weakness in some areas of speech, language, and communication, and that intervention planning for children with each syndrome may take an approach informed by these profiles. Issues related to within-group variability, shared outcomes among syndromes, and the need for empirical validation for syndrome-specific recommendations are discussed.     

Comorbidity and the Treatment of Principal Anxiety Disorders in a Naturalistic Sample

This study examined the impact of comorbidity on treatment outcome and the effects of cognitive behavioral therapy (CBT) for anxiety and depressive disorders on comorbid disorders in a naturalistic sample of 150 patients presenting to an anxiety disorders clinic. The following results were observed across principal (i.e., most severe) diagnoses. Patients with comorbid anxiety and depressive disorders presented for treatment with higher severity of their principal disorder than patients without comorbidity. However, the presence of comorbidity did not predict dropout or poor treatment response, and patients demonstrated significant improvement in their principal disorders regardless of comorbidity. The frequency of clinically severe and subclinical (i.e., not severe enough to meet diagnostic criteria) comorbid conditions decreased significantly over the course of treatment. The implication of these findings for the classification and treatment of emotional disorders is discussed.     

Sexuality,Intimacy, and Recovery

Human sexuality is an inseparable part of the yearning for intimacy, merging, and touch, both for people in general and for those with severe mental disorders. The exploration and acceptance of sexuality and the yearning for intimacy are vital to the recovery process of people with severe mental disorders. Despite this importance, therapists appear to disregard sexual issues in therapy with people with severe mental disorders. This article discusses the sexuality difficulties of these patients resulting from their personal history and examines the difficulties of therapists and therapeutic institutions in dealing with these patients’ sexuality-related issues.     

Electroencephalographic findings in vertebro-basilar circulation disorders]

The method of electroencephalography was used for examining 86 patients with clinical signs of vertebrobasilar blood supply disorders and bulbopontine localization of ischemic phenomena. Light to moderate diffuse alterations were found which manifested themselves, on the one hand, in a reduction in tension with a tendency toward desynchronization of the alpha rhythm and, on the other, in interspersed theta waves, sometimes with temporal localization. The determination of the location of the vascular process depends upon both the clinical syndrome and the performance of function tests during the electroencephalographic examination. The disproportion between the excellent organization of the bioelectrical activity and the severe clinical picture is sufficient reason to consider vascular damages to the pontobulbar portion of the brain stem.     

Family Impact of Tourette's Syndrome

Tourette's syndrome (TS) is a neuropsychological disorder characterized by vocal and motor tics. TS is also associated with several behavior disorders such as Attention Deficit Hyperactivity Disorder, Oppositional Defiant Disorder, conduct disorder, and Obsessive-Compulsive Disorder. We examined the impact of Tourette's syndrome with and without comorbid psychiatric disorders on the family. TS complicated by comorbid disorders had a greater impact on the family than uncomplicated TS. Tourette's symptom severity was significantly correlated with the level of impact on the family and with the number of comorbid disorders. TS is a disorder with effects that extend beyond motor and vocal tics.     

Temporal coherence of criteria for four personality disorders

This study sought to investigate the coherence of changes observed in diagnostic criteria for borderline, schizotypal, obsessive-compulsive, and avoidant personality disorders. Five hundred, forty-nine patients were independently evaluated 2 years apart, and correlations of observed changes in each diagnostic criterion with changes in other criteria were examined to determine if there was within-syndrome consistency in these changes. The observed changes in criteria were consistent within syndrome (median alpha = 0.72 across 4 disorders), and reasonably specific to that syndrome relative to the other disorders. The results support the validity of these criterion sets as representing coherent syndromes.     

Bias and Millon clinical multiaxial inventory (MCMI-II) diagnosis

In the current study, the degree of bias is calculated for each of the personality disorder and clinical syndrome scales of the MCMI-II. In general, most of the MCMI-II scales are prone to only mild or moderate biases. However, the paranoid personality disorder, somatoform, bipolar: manic, thought disorder, and delusional disorder scales are prone to severe biases. When the MCMI-II is utilized to make diagnostic decisions, bipolar and schizophrenic disorders are apt to be grossly underestimated. The implications of bias in diagnostic prevalence rates are discussed and an equation is offered which provides for adjustments to be made when the percentage of positive MCMI-II test results are used to determine prevalence rates for clinical or research populations. The observation is made that imperfect sensitivity and specificity for the MCMI-II scales will result in inaccurate estimates of personality disorders and clinical syndromes when the MCMI-II is used to survey various populations.     

Fluency disorders in genetic syndromes

The characteristics of various genetic syndromes have included “stuttering” as a primary symptom associated with that syndrome. Specifically, Down syndrome, fragile X syndrome, Prader-Willi syndrome, Tourette syndrome, Neurofibromatosis type I, and Turner syndrome all list “stuttering” as a characteristic of that syndrome. An extensive review of these syndromes indicated clients diagnosed with these syndromes do show evidence of nonfluency patterns, but not all would be considered stuttering. Many of the syndromes are marked by degrees of mental retardation that probably contribute to a higher than average prevalence of stuttering, as well as a higher than average prevalence of other fluency disorders (when compared to the population at large).

An in-depth analysis of the available data indicates that some of these genetic syndromes show patterns of stuttering that may be indicative of only that syndrome (or similar syndromes) that can be differentially diagnosed from developmental stuttering. Among these patterns are the word-final nonfluencies noted in Prader-Willi syndrome; the presence of stuttering in the absence of secondary behaviors noted in Prader-Willi syndrome and; the presence of palilalia, word-final and word-medial nonfluencies, and word-medial and word-final nonfluencies in Tourette syndrome. Implications for future research are discussed in light of these findings.

Educational objectives: The reader will be able to: (1) describe the various different genetic syndromes that are associated with fluency disorders; (2) describe the types of nonfluencies that are associated with the major types of genetic syndromes that have fluency disorders; (3) describe the behaviors that may assist in differentially diagnosing different types of speech characteristics associated with various genetic syndromes.     

Rethinking the concepts of ‘local or global processors’: evidence from Williams syndrome,Down syndrome,and Autism Spectrum Disorders

Both Williams syndrome (WS) and Autism Spectrum Disorder (ASD) have been characterized as preferentially processing local information, whereas in Down syndrome (DS) the reported tendency is to process stimuli globally. We designed a cross‐syndrome, cross‐task comparison to reveal similarities and differences in local/global processing in these disorders. Our in‐depth study compared local/global processing across modalities (auditory‐verbal/visuo‐spatial) and levels of processing (high/low) in the three syndromes. Despite claims in the literature, participants with ASD or WS failed to show a consistent local processing bias, while those with DS failed to show a reliable global processing bias. Depending on the nature of the stimuli and the task, both local and global processing biases were evident in all three neurodevelopmental disorders. These findings indicate that individuals with neurodevelopmental disorders cannot simply be characterized as local or global processors.     

The Identity Consolidation Inventory (ICI): Development and Application of a Questionnaire for Assessing the Structuralization of Individual Identity

This paper describes the author's effort to develop a self-rating scale to measure the consolidation of identity in a given individual. It describes the construction of the 35-item Identity Consolidation Inventory (ICI) and, as an appendix to the paper, includes the final version of this questionnaire. The scale's reliability and validity are demonstrated via its administration to 238 normal controls, 80 individuals with Borderline Personality Disorder and 13 subjects with Dissociative Identity Disorder (“multiple personality”). The clinical and research usefulness of ICI lies in the realm of discerning the syndrome of identity diffusion that underlies all severe personality disorders.     

从中西医结合角度探讨代谢综合征

代谢综合征的发生、发展、变化错综复杂,中医的病因病机学说有很多包括脾胃功能失常、肝失疏泄、痰浊瘀血阻滞等。由于老年人抗病能力和修复能力低下,往往同时合并多种其他病变。所以,在辨证研究上需要一个标准化的、符合实际情况的、临床可行的分型。由于目前的中医临床研究的深度和广度还不足以产生一个辨证分型的方案,所以可分期对待,在分期的基础上进一步辨证论治。治疗的重点在于纠正代谢紊乱,改善和延缓合并症的发生及发展。中医学整体调整的理念和中药中的多种成分的多重作用,有利于多因素多系统病变的防治。中药可通过不同途径和环节改善胰岛素敏感性,在防治代谢综合征方面可能有着广阔的前景。     

Applying Cluster Analysis to Define a Typology of Chronic Fatigue Syndrome in a Medically-Evaluated,Random Community Sample

This study involved a randomly selected, medically-evaluated, community-based sample of 166 individuals with chronic fatigue. Participants diagnosed with chronic fatigue syndrome and medically-explained chronic fatigue reported significantly more severe fatigue following exertion than the idiopathic chronic fatigue group, and participants with medically-explained chronic fatigue also reported significantly more severe fatigue following exertion than the psychiatrically-explained chronic fatigue group. A cluster analysis was performed to define a typology of chronic fatigue symptomatology for participants diagnosed with chronic fatigue syndrome. Three clusters emerged. Cluster 1 contained only one participant with chronic fatigue syndrome and was characterized by relatively low post-exertional fatigue. Cluster 2 contained a small proportion of individuals with chronic fatigue syndrome and was characterized by most severe post-exertional fatigue and most improvement in fatigue following rest. Cluster 3 contained the highest proportion of individuals with chronic fatigue syndrome, and was characterized by high post-exertional fatigue and fatigue not alleviated by rest.     

Brain imaging in neurogenetic conditions: realizing the potential of behavioral neurogenetics research

Behavioral neurogenetics research is a new method of scientific inquiry that focuses on investigation of neurodevelopmental dysfunction associated with specific genetic conditions. This research method provides a powerful tool for scientific inquiry into human gene-brain-behavior linkages that complements more traditional research approaches. In particular, the use of specific genetic conditions as models of common behavioral and cognitive disorders occurring in the general population can reveal insights into neurodevelopmental pathways that might otherwise be obscured or diluted when investigating more heterogeneous, behaviorally defined subject groups. In this paper, we review five genetic conditions that commonly give rise to identifiable neurodevelopmental and neuropsychiatric disability in children: fragile X syndrome, velo-cardio-facial syndrome, Williams syndrome, Turner syndrome, and Klinefelter syndrome. While emphasis is placed on describing the brain morphology associated with these conditions as revealed by neuroimaging studies, we also include information pertaining to molecular genetic, postmortem, and neurobehavioral investigations to illustrate how behavioral neurogenetics research can contribute to an improved understanding of brain disorders in childhood.