Cuidados paliativos en personas con demencia severa: reflexiones y desafíos / Palliative care of patients with severe dementia

Dementia is an important public health problem due to the complex challenges it poses during the progression of the disease and especially during its end-of-life stage. The aim of this article is to consider the issues that arise from the use of commonly administered therapies in patients with severe dementia. Scenarios where excessive or futile therapeutic interventions might occur are discussed. The article concludes that it is advisable to analyze the clinical criteria and ethical considerations related to the administration of those treatments in order to develop and disseminate better practice guidelines for health professionals.

Propiedades psicométricas y utilidad diagnóstica del Addenbrooke's Cognitive Examination-Revised (ACE-R) en una muestra de ancianos chilenos / Psychometric properties and diagnostic usefulness of the Addenbrooke's Cognitive Examination-revised in a Chilean elderly sample

Background: The Addenbrooke's Cognitive Examination - Revised (ACE-R) is a good alternative to the Mini Mental State Examination (MMSE) for assessing cognitive capacities in dementia. Aim: To estimate the psychometric properties and diagnostic utility of the Addenbrooke's Cognitive Examination-Revised (ACE-R) in a Chilean elderly sample. Material and Methods: ACE-R was adapted for the Chilean population (ACE-R-Ch) and then administered to 60 dementia patients, 22 mild cognitive impairment (MCI) patients and 45 control subjects in addition to the MMSE for assessing global cognitive efficiency. Caregivers of dementia patients and collateral sources of MCI patients and elderly subjects without dementia were interviewed with measures of dementia severity, functional status in activities of daily living and cognitive changes. Convergent validity, internal consistency reliability, cutoff points, sensitivity and specificity for ACE-R-Ch were estimated. Results: Regarding convergent validity, the ACE-R-Ch showed significant correlations (p < 0,001) with another cognitive measure (r = 0,952 with MMSE), a rating for dementia severity (Spearman rho=-0,822 with CDR), functional capacity assessments (r = -0,70 with ADLQ-Ch; r = -0,725 with PFAQ-Ch; and r = 0,650 with IADL Scale) and a measure of cognitive changes (r = -0,633 with AD8-Ch). In terms of reliability, the test had a Cronbach alpha coefficient of 0.918. The best cut-off point to distinguish cases of dementia from control subjects was a score of 76, which reached a sensitivity of 0.92 and a specificity of 0.93. Conclusions: The ACE-R-Ch showed acceptable psychometric properties, becoming a valid and reliable instrument to assess global cognitive efficiency or cognitive impairment. Its diagnostic utility to detect dementia patients also worked very well in a Chilean elderly sample.

Parálisis general sifilítica: presentación de 5 casos / Paretic neurosyphilis: report of five patients

We report five male patients, aged 35 to 63 years who suffered from paretic neurosyphilis. The clinical course was that of a subacute dementia with a frontal syndrome, with more apathy than euphoria. All were HIV negative and four were heterosexual. In all, the cerebrospinal fluid had a mononuclear pleocytosis and a positive VDRL. EEG was abnormal in the 3 cases in whom it was performed. One patient in whom a brain angiography was performed, had images of vasculitis. Treatment with 18-24 million units of penicillin per day during two weeks or more, was partially effective.

Descomponiendo el síndrome de déficit atencional en el adulto: hacia un entendimiento de su heterogeneidad pronóstica / Prognostic heterogeneity of attention deficit/hyperactivity disorder in adults

Attention Deficit/Hyperactivity Disorder (ADHD) is a clinical syndrome characterized by an onset in early life. More than 65% of patients persist with manifestations of ADHD in adulthood. These symptoms may interfere in activities of daily-living, interpersonal relationships and professional and academic achievement. Nevertheless, the observation of an important group of adults with ADHD who do not show significant difficulties in the areas mentioned before puts into evidence the prognostic heterogeneity of this disorder. One of the current, most accepted explanations is the Double-Pathway Model: two double-dissociated deficits (Executive Disorders and Delayed-Reward Processing impairments) are involved in the genesis of ADHD, which explains the existence of different behavioral phenotypes. Moreover, personality traits like tenacity or perseverance are associated with higher levels of achievement in adults. On these grounds, we propose the hypothesis that the neurobiological correlate of tenacity/perseverance is a preserved Delayed-Reward Processing capacity, although further studies are needed to verify this idea.

Jonathan Swift y su contribución científica en "Los viajes de Gulliver" / Jonathan Swift's scientific contribution on his "Gulliver's Travels"

Jonathan Swift is one of the most celebrated satirist writers in literature. His well-known "Gulliver's Travels", apartfrom being a serious human being criticism, contains many interesting ana unrecognized comments about medical and other scientific facts. Swift made what seems to be thefirst account of a dementing illness in the elderly; his description fits well with what we now know as Alzheimer's disease. He also described a condition now called synaesthesia, a very interestingphenomenon that may contribute to creativity. In thispaper, we review aspects ofhis Ufe and reveal these amazing descriptions as samples of Swift's extraordinary power of observation.

Síntomas psicológicos y conductuales asociados a demencias: actualización terapéutica / An update on the treatment of psychological and behavioral symptoms associated to dementia

Behavioral disturbances in dementias are relevant because they are very common, they worsen patients' medical and cognitive condition, increase the caregiver burden and accelerate the admission to nursing homes. The different behavioral disturbances in dementias can appear isolated, but in most cases, patients present a combination of disturbances producing one of two main syndromes: the psychotic or the affective syndrome. Proper handling of these behavioral disturbances requires a correct characterization of the syndrome, discarding medical or environmental causes and selecting the drug with the better effectiveness and safety profile for each patient. In spite of the important number of psychotropics available, there is not enough scientific evidence about their real effectiveness and security in patients with Alzheimer's disease. This article reviews recent advances in the treatment of most common and disruptive behavioral disturbances.

Córtex prefrontal y trastornos del comportamiento: modelos explicativos y métodos de evaluación / Prefrontal cortex and behavioral disorders: theoretical framework and neuropsychological evaluation

Los trastornos del córtex prefrontal (CPF) permiten explicar la sintomatología de importantes cuadros neurológicos y psiquiátricos, tales como las secuelas de traumatismos encéfalo-craneanos y las esquizofrenias. Sin embargo, y a pesar de la gran importancia de sus funciones, su estudio se ha visto dificultado por razones teóricas, experimentales y clínicas. Recientemente han surgido dos nuevos modelos que intentan explicar los mecanismos a la base del funcionamiento del CPF. Presentamos una revisión de las principales manifestaciones clínicas ante su disfunción, los modelos explicativos postulados tradicionalmente, además de las dos propuestas recientes de Koechlin y Mesulam, para finalizar con una revisión de los instrumentos más utilizados en el ámbito clínico para la evaluación de las funciones propias de esta región, denominadas funciones ejecutivas y de autorregulación del comportamiento.

Dysfunction of prefrontal cortex explains the symptoms of many neurological and psychiatric disorders, such as the consequences of cerebral trauma and schizophrenia. Although the importance of frontal lobe functions in complex cognition has long been recognized, systematic research efforts to specify the nature, organization and roles of these functions have been difficult for theoretical, experimental and clinical reasons. Recently, two new theoretical frameworks have been proposed to explain the role of prefrontal cortex. In this paper, we present a revision of the main clinical manifestations secondary to prefrontal dysfunction, the frameworks proposed by Mesulam and Koechlin to explain the role of prefrontal cortex in behavior and the principal instruments available to evaluate executive and strategic self-regulation functions.

Miopatía por déficit de maltasa ácida en el adulto / Myopathy due to acid maltase deficiency in an adult patient

We report a 46 years old male presenting with tetraparesis and severe respiratory involvement. He had moderately elevated serum creatine phosphokinase values and the electromyography showed myopathic alterations and irritative signs. In the muscle biopsy a vacuolar myopathy with increased collagen deposits was found. Circulating lymphocytes presented abnormal PAS positive granules in their cytoplasm