De exilios y emociones

The purpose of this article is to test the potential of the methods and concepts of the history of emotions for a renewed and deeper studying of the exile. Focused on the cases of Américo Castro, Pedro Salinas and Fernando de los Rios, the article explores through their correspondence, the emotional mapping that they followed during their exile process. In the exiles, it is warned from the start suffering and emotional stress which led them to quickly locate their spaces for rest and shelter. Once they realized they were going to live as exiles, they built emotional communities in which they deployed, vented their emotions, but also placed their lives, their common experiences and expectations. The family, the professional circle and the references from both colleagues who remained in Spain and from many others (Spanish or not) who shared exile, are easily detected as constructed emotional communities. The exilic process was plenty of ups and downs and emotions, and to live and not just survive, the exiles were forced to design their own emotional reconstruction, their new lifes, not forgetting the old ones, but also filled with content and relief.El propósito de este artículo es probar las posibilidades que los métodos y los conceptos de la historia de las emociones plantean para un estudio renovado y profundo del exilio. Atendiendo a los ejemplos que ofrecen Américo Castro, Pedro Salinas y Fernando de los Ríos, se realiza un seguimiento, a través de sus epistolarios, de la cartografía emocional que los tres siguieron durante su proceso exílico. En los exiliados se advierte desde el comienzo sufrimiento y esfuerzo emocional, lo que les llevó enseguida a localizar sus espacios para el descanso y el refugio. Una vez que tomaron conciencia de que iban a vivir como exiliados, tocaba detectar y construir las comunidades emocionales con las que entablarían contacto para desplegar y desahogar sus emociones, pero también ubicar sus vidas, sus experiencias comunes y sus expectativas. La familia, el círculo profesional y el contacto y las referencias tanto de los colegas que se habían quedado en España como de otros tantos (españoles o no) que compartían exilo, se detectan con facilidad como esas comunidades emocionales construidas. Dilatado el proceso exílico ya plagado de vaivenes y emociones encontradas, y para vivir y no sólo sobrevivir, era obligado diseñar la propia reconstrucción emocional, una vida nueva, que no olvidaba la antigua, pero que se llenaba también de contenido y alivio

On exiles and emotions

The purpose of this article is to test the potential of the methods and concepts of the history of emotions for a renewed and deeper studying of the exile. Focused on the cases of Américo Castro, Pedro Salinas and Fernando de los Rios, the article explores through their correspondence, the emotional mapping that they followed during their exile process. In the exiles, it is warned from the start suffering and emotional stress which led them to quickly locate their spaces for rest and shelter. Once they realized they were going to live as exiles, they built emotional communities in which they deployed, vented their emotions, but also placed their lives, their common experiences and expectations. The family, the professional circle and the references from both colleagues who remained in Spain and from many others (Spanish or not) who shared exile, are easily detected as constructed emotional communities. The exilic process was plenty of ups and downs and emotions, and to live and not just survive, the exiles were forced to design their own emotional reconstruction, their new lifes, not forgetting the old ones, but also filled with content and relief

Clinical manifestations of intermediate allele carriers in Huntington disease

Objective: There is controversy about the clinical consequences of intermediate alleles (IAs) in Huntington disease (HD). The main objective of this study was to establish the clinical manifestations of IA carriers for a prospective, international, European HD registry. Methods: We assessed a cohort of participants at risk with <36 CAG repeats of the huntingtin (HTT) gene. Outcome measures were the Unified Huntington's Disease Rating Scale (UHDRS) motor, cognitive, and behavior domains, Total Functional Capacity (TFC), and quality of life (Short Form-36 [SF-36]). This cohort was subdivided into IA carriers (27-35 CAG) and controls (<27 CAG) and younger vs older participants. IA carriers and controls were compared for sociodemographic, environmental, and outcome measures. We used regression analysis to estimate the association of age and CAG repeats on the UHDRS scores. Results: Of 12,190 participants, 657 (5.38%) with <36 CAG repeats were identified: 76 IA carriers (11.56%) and 581 controls (88.44%). After correcting for multiple comparisons, at baseline, we found no significant differences between IA carriers and controls for total UHDRS motor, SF-36, behavioral, cognitive, or TFC scores. However, older participants with IAs had higher chorea scores compared to controls (p 0.001). Linear regression analysis showed that aging was the most contributing factor to increased UHDRS motor scores (p 0.002). On the other hand, 1-year follow-up data analysis showed IA carriers had greater cognitive decline compared to controls (p 0.002). Conclusions: Although aging worsened the UHDRS scores independently of the genetic status, IAs might confer a late-onset abnormal motor and cognitive phenotype. These results might have important implications for genetic counseling. ClinicalTrials.gov identifier: NCT01590589

Clinical and genetic characteristics of late-onset Huntington's disease

Background: The frequency of late-onset Huntington's disease (&gt;59 years) is assumed to be low and the clinical course milder. However, previous literature on late-onset disease is scarce and inconclusive. Objective: Our aim is to study clinical characteristics of late-onset compared to common-onset HD patients in a large cohort of HD patients from the Registry database. Methods: Participants with late- and common-onset (30–50 years)were compared for first clinical symptoms, disease progression, CAG repeat size and family history. Participants with a missing CAG repeat size, a repeat size of ≤35 or a UHDRS motor score of ≤5 were excluded. Results: Of 6007 eligible participants, 687 had late-onset (11.4%) and 3216 (53.5%) common-onset HD. Late-onset (n = 577) had significantly more gait and balance problems as first symptom compared to common-onset (n = 2408) (P &lt;.001). Overall motor and cognitive performance (P &lt;.001) were worse, however only disease motor progression was slower (coefficient, −0.58; SE 0.16; P &lt;.001) compared to the common-onset group. Repeat size was significantly lower in the late-onset (n = 40.8; SD 1.6) compared to common-onset (n = 44.4; SD 2.8) (P &lt;.001). Fewer late-onset patients (n = 451) had a positive family history compared to common-onset (n = 2940) (P &lt;.001). Conclusions: Late-onset patients present more frequently with gait and balance problems as first symptom, and disease progression is not milder compared to common-onset HD patients apart from motor progression. The family history is likely to be negative, which might make diagnosing HD more difficult in this population. However, the balance and gait problems might be helpful in diagnosing HD in elderly patients

Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study

Background: People with Huntington's disease (HD) have been observed to have lower rates of cancers. Objective: To investigate the relationship between age of onset of HD, CAG repeat length, and cancer diagnosis. Methods: Data were obtained from the European Huntington's disease network REGISTRY study for 6540 subjects. Population cancer incidence was ascertained from the GLOBOCAN database to obtain standardised incidence ratios of cancers in the REGISTRY subjects. Results: 173/6528 HD REGISTRY subjects had had a cancer diagnosis. The age-standardised incidence rate of all cancers in the REGISTRY HD population was 0.26 (CI 0.22-0.30). Individual cancers showed a lower age-standardised incidence rate compared with the control population with prostate and colorectal cancers showing the lowest rates. There was no effect of CAG length on the likelihood of cancer, but a cancer diagnosis within the last year was associated with a greatly increased rate of HD onset (Hazard Ratio 18.94, p < 0.001). Conclusions: Cancer is less common than expected in the HD population, confirming previous reports. However, this does not appear to be related to CAG length in HTT. A recent diagnosis of cancer increases the risk of HD onset at any age, likely due to increased investigation following a cancer diagnosis