Evolutionary systems therapy for paranoid personality disorder: A seven cases series.

Little is known about effective psychosocial treatments for paranoid personality disorder. This study explores the feasibility of a novel treatment, namely Evolutionary Systems Therapy, in supporting individuals diagnosed with paranoid personality disorder. Seven patients attended 10 months of individual therapy without receiving any psychopharmacological treatment. The primary outcome was the feasibility of the intervention, while the secondary outcomes were remission from the diagnosis and reliable changes in personality pathology and paranoid ideation. All recruited patients completed the intervention and did not report any adverse events. Six out of seven patients experienced remission from the diagnosis of paranoid personality disorder. All participants showed reliable changes in personality pathology and paranoid ideation, which were maintained at the 1-month follow-up. Further research is needed to confirm these encouraging results.

Conceptualizing a less paranoid schizophrenia.

Schizophrenia stands as one of the most studied and storied disorders in the history of clinical psychology; however, it remains a nexus of conflicting and competing conceptualizations. Patients endure great stigma, poor treatment outcomes, and condemnatory prognosis. Current conceptualizations suffer from unstable categorical borders, heterogeneity in presentation, outcome and etiology, and holes in etiological models. Taken in aggregate, research and clinical experience indicate that the class of psychopathologies oriented toward schizophrenia are best understood as spectra of phenomenological, cognitive, and behavioral modalities. These apparently taxonomic expressions are rooted in normal human personality traits as described in both psychodynamic and Five Factor personality models, and more accurately represent explicable distress reactions to biopsychosocial stress and trauma. Current categorical approaches are internally hampered by axiomatic bias and systemic inertia rooted in the foundational history of psychological inquiry; however, when such axioms are schematically decentralized, convergent cross-disciplinary evidence outlines a more robust explanatory construct. By reconceptualizing these disorders under a dimensional and cybernetic model, the aforementioned issues of instability and inaccuracy may be resolved, while simultaneously opening avenues for both early detection and intervention, as well as for more targeted and effective treatment approaches.

[Paranoid personality disorder]. / Personnalité paranoïaque.

PARANOID PERSONALITY DISORDER. The paranoid personality disorder fascinates and worries health professionals, who are sometimes victims of aggressive claims from their patients. Overestimation of oneself, psychorigidity, distrust and relational hyperesthesia characterize the paranoid type of personality disorder. This disorder is often associated with co-morbidities which mask it and promote decompensation towards a delusional disorder with the risk of heteroaggressive acts or towards a severe depressive disorder with suicidal risk. On the basis of regular monitoring and exhaustive assesment, management requires rigor and balance in the sharing of information, the use of psychotropic drugs or hospitalization, if necessary, in compulsory care.

PERSONNALITÉ PARANOÏAQUE. Le trouble de la personnalité paranoïaque fascine et inquiète les professionnels de santé, parfois victimes de revendications agressives de la part de patients dont ils ont la charge. La surestimation de soi, la psychorigidité, la méfiance et l'hyperesthésie relationnelle caractérisent le trouble de personnalité de type paranoïaque. Ce trouble est souvent associé à des comorbidités qui le masquent et favorisent une décompensation vers un trouble délirant, avec risque de passage à l'acte hétéro-agressif ou vers un trouble dépressif sévère avec risque suicidaire. Sur la base d'une évaluation régulière et exhaustive, la prise en charge nécessite rigueur et équilibre dans l'utilisation de médicaments psychotropes, le partage d'information à des tiers et le recours à l'hospitalisation, si nécessaire en soin sans consentement.

Connectome-based predictive modeling predicts paranoid ideation in young men with paranoid personality disorder: a resting-state functional magnetic resonance imaging study.

Paranoid personality disorder (PPD), a mental disorder that affects interpersonal relationships and work, is frequently neglected during diagnosis and evaluation at the individual-level. This preliminary study aimed to investigate whether connectome-based predictive modeling (CPM) can predict paranoia scores of young men with PPD using whole-brain resting-state functional connectivity (rs-FC). College students with paranoid tendencies were screened using paranoia scores ≥60 derived from the Minnesota Multiphasic Personality Inventory; 18 participants were ultimately diagnosed with PPD according to the Diagnostic and Statistical Manual of Mental Disorders and subsequently underwent resting-state functional magnetic resonance imaging. Whole-brain rs-FC was constructed, and the ability of this rs-FC to predict paranoia scores was evaluated using CPM. The significance of the models was assessed using permutation tests. The model constructed based on the negative prediction network involving the limbic system-temporal lobe was observed to have significant predictive ability for paranoia scores, whereas the model constructed using the positive and combined prediction network had no significant predictive ability. In conclusion, using CPM, whole-brain rs-FC predicted the paranoia score of patients with PPD. The limbic system-temporal lobe FC pattern is expected to become an important neurological marker for evaluating paranoid ideation.

A case study on a severe paranoid personality disorder client treated with metacognitive interpersonal therapy.

Paranoid personality disorder (PPD) is a severe condition, lacking specialized and empirically supported treatment. To provide the clinician with insights into how to treat this condition, we present a case study of a 61-year-old man with severe PPD who presented with ideas of persecution, emotionally charged hostility, and comorbid antisocial personality disorder. The client was treated with 6 months of Metacognitive Interpersonal Therapy, which included: creating a shared formulation of his paranoid attitudes; trying to change his inner self-image of self-as-inadequate and his interpersonal schemas where he saw the others as threatening. Guided imagery and rescripting techniques, coupled with behavioral experiments, were used to promote a change. At the end of the therapy the client reported a reliable change in general symptomatology and, specifically, in interpersonal sensitivity, hostility, and paranoid ideation; he could no longer be diagnosed as PPD and only some paranoid and antisocial characteristics remained.

Effects of interaction of NOS1AP gene polymorphisms and childhood abuse on paranoid personality disorder features among male violent offenders in China.

BACKGROUND: Paranoid Personality Disorder (PPD) results from a complex synergy between genetic and environmental factors. Childhood abuse is one of risk factors. Nitric Oxide Synthase 1 Adaptor Protein (NOS1AP) is a candidate gene of schizophrenia, which has similar pathophysiology to PPD. This study investigated the role of NOS1AP gene polymorphisms and a history of childhood abuse in predicting PPD features among male violent offenders in the Chinese Han population. METHOD: Four NOS1AP Single Nucleotide Polymorphisms (SNPs), rs4145621, rs3751284, rs348624 and rs6680461 were genotyped in a sample of 423 male prisoners. Participant evaluations included demographic information, measures of childhood abuse (Child Trauma Questionnaire, CTQ), and PPD features (Personality Diagnostic Questionnaire-4, PDQ-4). Participants were divided into a PPD group and non-PPD group assessed by PDQ-4. RESULTS: Regression analysis revealed that emotional abuse, NOS1AP SNPs rs348624 and rs4145621 predicted PPD features (P < 0.05) among prison samples. Significant interactions between childhood abuse history and NOS1AP SNPs rs3751284 and rs6680461 were also observed. Individuals carrying the C allele of rs3751284 were susceptible to PPD features when exposed to higher levels of emotional neglect (P < 0.05); Individuals with the G allele of rs6680461 were susceptible to PPD features when exposed to higher levels of emotional, physical and sexual abuse (P < 0.01). CONCLUSIONS: These results suggest that the interaction between childhood abuse and NOS1AP gene polymorphisms may have an influence on PPD features, at least in male violent offenders.

Coexistence between personality disorders and substance use disorder. Madrid study about prevalence of dual pathology.

INTRODUCTION: Personality disorders (PD) and substance use disorders (SUD) have a high prevalence and an important health and socioeconomic impact so, it is interesting to study the relationship between them. The objectives of the study are: to compare the prevalence of SUD between patients with and without diagnosis of PD, to analyze if any PD is related to the SUD, and if a specific PD is associated with a specific SUD. MATERIAL AND METHODS: Cross-sectional study in 837 patients from centers of attention to drug addiction and mental health in Madrid, Spain. The Mini International Neuropsychiatric Interview (MINI) and the Personality Diagnostic Questionnaire-4+ (PDQ4+) are used to detect mental disorder and PD, respectively. RESULTS: SUD is significantly higher in antisocial PD (p<0.01); sedative (p<0.01) and alcohol (p<0.05) use disorder in borderline PD; cocaine (p<0.05) and alcohol (p<0.01) use disorder in paranoid PD; and alcohol use disorder in histrionic PD (p<0.01). The SUD for cocaine is lower in obsessive- compulsive PD (p<0.05) and depressive PD (p<0.01). There is a positive correlation between the number of PD of a subject and the number of SUD that it presents. The risk of an alcohol [OR of 1,08 CI (1,01-1,16)] or sedatives [OR of 1,08 CI (1,001-1,17)] use disorders increases if an individual presents more than one type of PD. CONCLUSIONS: There is not differences of SUD prevalence between PD and not PD groups. We found an association between SUD and PD of cluster B (antisocial, borderline and histrionic) and also with paranoid PD. The SUD are more common among man with the exception of sedatives.

Schizotypal traits, neurocognition, and paternal age in unaffected first degree relatives of patients with familial or sporadic schizophrenia.

Studies comparing cognitive processes between familial and sporadic schizophrenia have yielded inconsistent findings. In this study we examined differences in neurocognition and schizotypal traits in unaffected relatives of schizophrenia-spectrum patients with either the familial (multiplex) or the sporadic (simplex) subtype of the disorder, taking paternal age at birth into consideration. Simplex (n = 65; SR), multiplex (n = 35; MR) relatives and controls (n = 114) were compared on several cognitive functions and schizotypal traits; between-group differences were evaluated with and without including paternal age in the analyses. SR and MR had higher negative and paranoid traits compared with controls, but paternal age abolished the differences between the SR and control groups. When taking into account schizotypal traits and participants' age, controls outperformed MR in strategy formation and set-shifting and SR in psychomotor speed, set-shifting and executive working memory. After including paternal age in the analyses, controls outperformed MR in strategy formation, working memory and executive working memory and both groups in psychomotor speed and set-shifting. These findings suggest that multiplex relatives present with a "riskier" personality and cognitive profile when considering the effects of paternal age. Nevertheless, simplex relatives are impaired in fundamental cognitive processes, thus highlighting the detrimental effects of paternal age on neurocognition.

[Paranoid personality disorder and criminal offense]. / Trouble de personnalité paranoïaque et infractions pénales.

BACKGROUND: Currently, all of the studies that focus on the relationship between paranoia and criminal offenses exclusively concern subjects suffering from a delusional paranoid disorder. However, subjects with single paranoid personality disorder, without any associated delusional disorder, are not uncommon in forensic practice. OBJECTIVES: This study aims to describe the offenses committed by subjects suffering from a single paranoid personality disorder and to compare them with the offenses committed by the subjects affected by a paranoid delusional disorder associated with paranoid personality disorder. Our initial hypothesis is that both populations have a comparable criminological profile. METHODS: Based on a 17 year-long experience carried out in the framework of a forensic assessment, we have selected all subjects presenting a paranoid personality disorder, whether single or associated with paranoid delusional disorder. The selected individuals were divided into two groups according to whether they presented paranoid delusional disorder or not. The offenses were grouped into criminal categories. The alpha risk was fixed at 1%. Data analysis is done by SAS software version 9.4. RESULTS: In a sample of 106 subjects presenting a paranoid personality disorder, including 4 women and 102 men, we found 79 subjects with a single paranoid personality and 27 with an associated paranoid delusional disorder. The average age at the time of the offense was 41 for those with single personality disorders and 49 for those with paranoid delusional disorders. Both groups had forensic antecedents (41%, 11/27 of paranoid delusional disorder and 51%, 40/79 of single paranoid personality disorder). Psychiatric history was more frequent in the paranoid delusional disorder group (59%, 16/27) than in the single paranoid personality disorder group (13%, 10/79). History of addiction was comparable in terms of alcohol abuse (26% in both groups) and other substances (7.5%, 2/27 of paranoid delusional disorder and 9%, 7/79 of single paranoid personality disorder). Comparison of the two groups highlighted significant differences in the type of criminal offenses committed (Fisher's exact test: P=0.0003, alpha risk <0.0001). The offenses committed by delusional authors essentially came down to verbal or physical violence, including homicide (44%, 12/27), and were usually focused on a designated persecutor. Sexual violence was rare. On the other hand, paranoid personality disorder was associated with a wider variety of offenses. Sexual offenses (including 28 rapes, 35%, 28/79) were thus almost as frequent as murder, and attempted murder (38%, 30/79). This diversity of committed offenses was found in their forensic antecedents. In these subjects, the logic of omnipotence may had over ruled the logic of revenge. CONCLUSION: We conducted a retrospective study on 106 subjects with paranoid personality disorder, including 27 subjects with associated paranoid delusional disorder. The comparison of the two groups demonstrated significant differences in offenses. Verbal and physical but non-sexual violence, committed in a delusional logic, was found among delusional subjects, while the forms of violence were more multiform in the single paranoid personality disorder group, frequently including sexual violence. This is, as far as we know, the first study describing the medico-legal acting-out of paranoid personalities. These results, which will need to be confirmed by future studies, point out the importance of the criminological risk that may be associated with paranoid personality disorder, without any associated delusional disorder.

Interpersonal sensitivity, bullying victimization and paranoid ideation among help-seeking adolescents and young adults.

AIM: The effects of a negative interpersonal experience, such as bullying victimization in childhood and adolescence, can be strong and long lasting. Bullying victimization is associated with paranoid ideation and suspiciousness. Few studies have focused on personality traits of victims of bullying. The aim of this study is to investigate whether a particular personality trait called interpersonal sensitivity may be related to suspiciousness in those who experienced bullying victimization. METHODS: The study sample consisted of 147 help-seeking adolescents (mean age 17 years) selected after a screening phase (Prodromal Questionnaire) and evaluated with the Structured Interview for Psychosis-risk Syndromes (SIPS). All participants were specifically asked if they had experienced either psychological bullying or physical bullying, and they completed the Interpersonal Sensitivity Measure (IPSM). RESULTS: Of the whole sample, 30 (20%) participants had experienced psychological bullying or physical bullying at least once in their life. Performing a multiple regression, bullying victimization was found to be an independent predictor of subtle paranoid ideation and suspiciousness. Interpersonal sensitivity was also found to be an independent predictor of subtle paranoid ideation; in particular, two IPSM subscales, fragile inner-self and separation anxiety, showed a significant correlation with subtle paranoid ideation. CONCLUSIONS: Our results confirmed that bullying victimization is a negative interpersonal experience associated with paranoid ideation and suspiciousness. However, being overly sensitive and having negative beliefs about the self as fragile and vulnerable to threat also lead to a tendency to attribute experiences as externally caused and, in turn, facilitate the formation and maintenance of paranoid ideation.

Clinical cut-off point for the Distrust dimension of the Dimensional Clinical Personality Inventory 2 (IDCP-2).

The Clinical Dimensional Personality Inventory 2 (IDCP-2) is a 206-item self-report tool developed for the assessment of 12 dimensions (divided into 47 factors) of personality pathology. One of the scales comprising the instrument, the Distrust scale, is intended to provide psychometric information on traits closely related to the Paranoid Personality Disorder (PPD). In the present research, we used the Item Response Theory and the Receiver Operating Characteristic curve analysis to establish a clinical meaningful cutoff for the Distrust scale. Participants were 1,679 adults, among outpatients diagnosed with PPD, outpatients diagnosed with other PDs, and adults from the community. The Wright map revealed that outpatients were located at the very high levels on the latent continuum of the Distrust scale, with a very large effect size for the mean difference between patients and non-patients. The ROC curve supported a cutoff at -1.00 score in theta standardization which yielded 0.87 of sensitivity and 0.54 of specificity. Findings from the present investigation suggest the IDCP-2 Distrust scale is useful as a screening tool of the core features of the PPD. We address potential clinical applications for the instrument and discuss limitations from the present study.

Longitudinal associations of temperament and character with paranoid ideation: A population-based study.

The aim of this study was to examine (a) the associations of temperament and character dimensions with paranoid ideation over a 15-year follow-up in the general population (b) the associations of explosive temperament and organized character profiles with paranoid ideation. 2137 subjects of the Young Finns Study completed the Temperament and Character Inventory and the Paranoid Ideation Scale of the Symptom Checklist-90 Revised in 1997, 2001, and 2012. Temperament dimensions of high novelty seeking, high harm avoidance, low reward dependence and explosive temperament profile were associated with the development of higher paranoid ideation. Regarding character, high self-directedness, high cooperativeness, and low self-transcendence and organized character profile were associated with lower paranoid ideation. These associations sustained after controlling for age, gender, and socioeconomic factors. However, the associations between temperament and paranoia mostly disappeared after taking character into account. Our study supported the hypothesis that personality dimensions contribute to the development of paranoid ideation. Temperament and character might combine a variety of single previously found risk factors into a more comprehensive framework for the developmental etiology of paranoia. Our findings provide evidence for psychotherapeutic interventions that support the self-regulation of temperamental vulnerabilities by internalizing mature concepts about the self and social relationships.

Gene expression study of mitochondrial complex I in schizophrenia and paranoid personality disorder.

OBJECTIVES: The aetiology and molecular mechanisms of schizophrenia (SCZ) and paranoid personality disorder (PPD) are not yet clarified. The present study aimed to assess the role of mitochondrial complex I and cell bioenergetic pathways in the aetiology and characteristics of SCZ and PPD. METHODS: mRNA levels of all genomic and mitochondrial genes which encode mitochondrial complex I subunits (44 genes) were assessed in blood in 634 SCZ, 340 PPD patients and 528 non-psychiatric subjects using quantitative real-time PCR, and associated comprehensive psychiatric, neurological and biochemical assessments. RESULTS: Significant expression changes of 18 genes in SCZ patients and 11 genes in PPD patients were detected in mitochondrial complex I. Most of these genes were novel candidate genes for SCZ and PPD. Several correlations between mRNA levels and severity of symptoms, drug response, deficits in attention, working memory, executive functions and brain activities were found. CONCLUSIONS: Deregulations of both core and supernumerary subunits of complex I are involved in the aetiology of SCZ and PPD. These deregulations have effects on brain activity as well as disorder characteristics.

[The concept of schizoidia in psychiatry : From schizoidia to schizotypy and cluster A personality disorders]. / Das Schizoidie-Konzept in der Psychiatrie : Von der Schizoidie über die Schizotypie zu den Cluster­A­Persönlichkeitsstörungen.

From a perspective of conceptual evolution schizoidia was initially considered to describe features both of the premorbid personality of schizophrenic patients and of the personalities of non-psychotic family members (Bleuler, Kahlbaum, Kraepelin). On a psychopatholocial level a close link to the complex basic symptom of autism was stressed. From the very beginnings of modern psychiatry schizoidia was discussed within a conceptual frame of schizophrenia spectrum disorders (Kretschmer, Hoch, Polatin). Approaches to operationalize these conceptual works laid the basis for the cluster A personalities in DSM-III. Due to the prominent concept of schizotypy (Kety, Rado, Meehl) three split up diagnostic categories of schizotypal, schizoid and paranoid personality disorders resulted. Cluster A personality disorders are frequent in community-based epidemiological studies. Health-care seeking behaviour due to primary personality-related problems, however, seems to be less paramount compared to cluster B and C personality disorders. Many family- and twin-based genetic studies convincingly stress a close link between schizotypal personality disorder and schizophrenia. This link is less pronounced for paranoid personality disorder, and even vanishingly low for schizoid personality disorder. From a perspective of schizophrenia spectrum disorders a vast amount of data from molecular genetic, neurobiological, neuropsychological and psychosocial research has impressingly confirmed this link for schizotypal personality disorder. Major research deficits, however, have to be noticed for paranoid and schizoid personality disorder.

Stability and Change in Personality Disorder Symptoms in 1-Year Follow-up of Depressed Adolescent Outpatients.

We investigated stability and change in personality disorder (PD) symptoms and whether depression severity, comorbid clinical psychiatric disorders, and social support predict changes in personality pathology among adolescent outpatients. The 1-year outcome of PD symptoms among consecutive adolescent psychiatric outpatients with depressive disorders (N = 189) was investigated with symptom count of depression, comorbid psychiatric disorders, and perceived social support as predictors. An overall decrease in PD symptoms in most PD categories was observed. Decreases in depression severity and in number of comorbid diagnoses correlated positively with decreases in PD symptoms of most PD categories. Social support from close friends predicted a decrease in schizotypal and narcissistic, whereas support from family predicted a decrease in paranoid symptoms. Our results suggest that among depressed adolescent outpatients, PD symptoms are relatively unstable, changes co-occuring with changes/improvement in overall psychopathology. Social support seems a possibly effective point for intervention efforts regarding positive outcome of PD symptoms.

Expression of schizophrenia-spectrum personality traits in daily life.

The present study examined the expression of the Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) schizotypal, schizoid, and paranoid personality disorder (PD) traits in daily life using experience sampling methodology in 206 nonclinically ascertained Spanish young adults oversampled for risk for schizophrenia-spectrum psychopathology. This study examined the overlap and differentiation of pathological personality traits in daily life settings, according to both diagnostic and multidimensional models. Daily life outcomes differentiated among schizophrenia-spectrum disorders. The assignment of Cluster A personality traits to positive, negative, paranoid, and disorganized dimensions provided an alternative to the traditional PD diagnoses. Positive, disorganized, and paranoid schizotypy were associated with elevated stress reactivity, whereas negative schizotypy was associated with diminished reactivity in daily life. The current diagnostic model is limited by the considerable overlap among the PD traits. Nonetheless, experience sampling methodology is sensitive enough to detect differences in day-to-day impairment and can be a powerful research tool for the examination of dynamic constructs such as personality pathology. (PsycINFO Database Record

The Quest for Greatness in the Race to Not Be Forgotten.

Details from a brief psychoanalytic treatment with a disturbed and desperate patient in acute emotional crisis are used to consider the internal dread some patients have regarding separation from the object and their phantasy of eternal damnation as a result of self-differentiation. One patient's loyalty to her object as the only way to prevent abandonment is examined in depth, but also considered as a severe psychic struggle found in other disturbed patients. The nature of this pathological loyalty has to do with the internalized and projected demand for perfection as the only currency for love and acceptance. Theoretical considerations are offered from a Kleinian perspective.

The relationship between anxiety disorders and dimensional representations of DSM-IV personality disorders: A co-twin control study.

BACKGROUND: There is substantial comorbidity between personality disorders (PDs) and anxiety disorders (ADs). Sharing of familial risk factors possibly explains the co-occurrence, but direct causal relationships between the disorders may also exist. METHODS: 2801 persons from 1391 twin pairs from the Norwegian Institute of Public Health Twin Panel were assessed for all DSM-IV PDs and ADs. Bivariate Poisson-regression analyses were performed to assess whether PDs predicted ADs at three different levels: All PDs combined, PDs combined within DSM-IV-clusters and each individual PD separately. Next, bivariate co-twin control analyses were executed within monozygotic (MZ) and dizygotic (DZ) twin pairs. A similar analytic strategy was employed in multivariate models including PDs as independent variables. RESULTS: PDs predicted ADs at all levels of analysis in bivariate regression models. Bivariate co-twin control analyses demonstrated an increased risk of ADs in all PDs combined, all PD-clusters and in schizotypal, paranoid, borderline, antisocial, avoidant and dependent PD. In the multivariate regression model, all PD-clusters and schizotypal, borderline, avoidant and obsessive-compulsive PD predicted ADs. Only borderline and avoidant PD predicted ADs in the multivariate co-twin control analysis. LIMITATIONS: Over-adjustment may explain the results from the multivariate analyses. The cross-sectional study design hampers causal inference. CONCLUSIONS: Comorbidity between ADs and PDs can be largely accounted for by shared familial risk factors. However, the results are also consistent with a direct causal relationship partly explaining the co-occurrence. Our results indicate specific environmental factors for comorbidity of ADs and borderline and avoidant PDs that are not shared with other PDs.

[A descriptive study of the neuropsychological and psychopathological profile in patients with type 1 myotonic dystrophy]. / Estudio descriptivo del perfil neuropsicologico y psicopatologico en pacientes con distrofia miotonica tipo 1.

INTRODUCTION: Type 1 myotonic dystrophy (MD-1) or Steinert disease is a multisystemic progressive disorder. Studies have shown cognitive deficits, depressive symptoms and a high incidence of anxiety personality traits that compromise both the functionality and the quality of life of these patients. AIM: To describe the cognitive and psychopathological profile of a sample of patients with the adult variant of MD-1. PATIENTS AND METHODS: A sample of 27 patients diagnosed with MD-1 was selected from those being followed up in the neurology service of the Complejo Hospitalario de Navarra. Eligibility criteria were age under 50 years and the absence of any other pathology or physical condition that prevented them from performing the psychological evaluation. A neuropsychological evaluation battery specifically designed for this kind of pathology was used, together with psychopathological and functionality measures. RESULTS: The neuropsychological evaluation revealed mainly deficits in visual-constructional and visuospatial skills, alternating attention and in other-reported dysexecutive signs and symptoms. The group of patients did not present any clinically significant symptoms of depression or anxiety, but did score high on obsession-compulsion, interpersonal sensitivity, paranoid ideation and psychoticism. The results point towards a deterioration in functionality. CONCLUSIONS: In the integral approach to MD-1, the characterisation and developmental monitoring of the cognitive, psychopathological and personality profile, as well as the level of functionality, all contribute to an improvement in the quality of life of these patients.

TITLE: Estudio descriptivo del perfil neuropsicologico y psicopatologico en pacientes con distrofia miotonica tipo 1.Introduccion. La distrofia miotonica tipo 1 (DM-1) o enfermedad de Steinert es un trastorno multisistemico y progresivo. Se han encontrado deficits cognitivos, clinica depresiva y alta incidencia de rasgos de personalidad ansiosos con afectacion tanto en la funcionalidad como en la calidad de vida de estos pacientes. Objetivo. Describir el perfil cognitivo y psicopatologico de una muestra de pacientes con la variante adulta de DM-1. Pacientes y metodos. Se selecciono una muestra de 27 pacientes con diagnostico de DM-1 en seguimiento en el Servicio de Neurologia del Complejo Hospitalario de Navarra. Los criterios de inclusion fueron tener menos de 50 anos y descartar cualquier otra patologia o condicion fisica que impidiese realizar la evaluacion psicologica. Se utilizo una bateria de evaluacion neuropsicologica especificamente disenada para este tipo de patologia, ademas de medidas de psicopatologia y funcionalidad. Resultados. La evaluacion neuropsicologica reflejo, principalmente, deficits en habilidades visuoconstructivas, visuoespaciales, atencion alternante y en sintomatologia disejecutiva heteroinformada. El grupo de pacientes no presento sintomatologia depresiva ni ansiosa clinicamente significativa, pero si puntuaciones elevadas en obsesion-compulsion, sensibilidad interpersonal, ideacion paranoide y psicoticismo. Los resultados orientaron hacia un deterioro en la funcionalidad. Conclusiones. En el abordaje integral de la DM-1, la caracterizacion y el seguimiento evolutivo del perfil cognitivo, psicopatologico y de personalidad, asi como del nivel de funcionalidad, contribuyen a la mejora de la calidad de vida de estos pacientes.