Mes: junio 2015

BACKGROUND: A reliable and valid questionnaire for screening restless legs syndrome (RLS) is essential for determining accurate estimates of disease frequency. In a 2002 NIH-sponsored workshop, experts suggested three mandatory questions for identifying RLS in epidemiologic studies. We evaluated the reliability and validity of this RLS-NIH questionnaire in a community-based sample and concurrently developed and evaluated the utility of an expanded screening questionnaire, the RLS-EXP. METHODS: The study was conducted at Kaiser Permanente of Northern California and the Stanford University Sleep Clinic. We evaluated test-retest reliability in a random sample of subjects with prior physician-assigned RLS (n=87), subjects with conditions frequently misclassified as RLS (n=31), and healthy subjects (n=9). Validity of both instruments was evaluated in a random sample of 32 subjects, and in-person examination by two RLS specialists was used as the gold standard. RESULTS: For the first three RLS-NIH questions, the kappa statistic for test-retest reliability ranged from 0.5 to 1.0, and sensitivity and specificity was 86% and 45%, respectively. For the subset of five questions on RLS-EXP that encompassed cardinal features for diagnosing RLS, kappas were 0.4-0.8, and sensitivity and specificity were 81% and 73%, respectively. CONCLUSIONS: Sensitivity of RLS-NIH is good; however, the specificity of the instrument is poor when examined in a sample that over-represents subjects with conditions that are commonly misclassified as RLS. Specificity can be improved by including separate questions on cardinal features, as used in the RLS-EXP, and by including a few questions that identify RLS mimics, thereby reducing false positives.

BACKGROUND: A 69 year-old woman presented to an interdisciplinary medical group with pathological gambling, and went on to develop disinhibition, loss of empathy, and perseverative, stereotyped and ritualistic behavior. An initial neuropsychological evaluation showed selective impairment on the Iowa Gambling Task similar to that of patients with behavioral variant frontotemporal dementia, despite normal performance on standard neuropsychological tasks. MRI scans showed frontal lobe atrophy, which was consistent with findings on hexamethylpropyleneamine oxime single photon emission CT (HMPAO-SPECT). INVESTIGATIONS: Physical examination, neuropsychiatric and neuropsychological assessments, MRI brain scan, HMPAO-SPECT. DIAGNOSIS: Behavioral variant frontotemporal dementia. MANAGEMENT: Pharmacological treatment with the selective serotonin reuptake inhibitor paroxetine for impulsive behavior and carbamazepine to stabilize mood. The patient and her family also received counseling to advise on behavioral and legal issues.

A large proportion of human social neuroscience research has focused on the issue of decision-making. Impaired decision-making is a symptomatic feature of a number of neurodegenerative diseases, but the nature of these decision-making deficits depends on the particular disease. Thus, examining the qualitative differences in decision-making impairments associated with different neurodegenerative diseases could provide valuable information regarding the underlying neural basis of decision-making. Nevertheless, few comparative reports of decision-making across patient groups exist. In this Review, we examine the neuroanatomical substrates of decision-making in relation to the neuropathological changes that occur in Alzheimer disease, frontotemporal dementia, Parkinson disease and Huntington disease. We then examine the main findings from studies of decision-making in these neurodegenerative diseases. Finally, we suggest a number of recommendations that future studies could adopt to aid our understanding of decision-making cognition.

Real life Executive Deficits in Patients with Focal Vascular Lesions Affecting the Cerebellum. Autores Manes F, Ruiz Villamil A, Ameriso S, Roca M, Torralva T.  Año 2009 Journal  Manes F, Ruiz Villamil A, Ameriso S, Roca M, Torralva T.  Volumen 283(1-2): 95-98 Abstract   Otra información    

Traditional cognitive tests may not be sensitive for the early detection of executive and social cognitive impairments in the behavioural variant of frontotemporal dementia. The aim of this study was to detect specific executive and social cognitive deficits in patients with early behavioural variant frontotemporal dementia using a battery of tests previously shown to be sensitive to frontal lobe dysfunction. Behavioural variant frontotemporal dementia patients and paired controls were assessed with a complete standard neuropsychological battery evaluating attention, memory, visuospatial abilities, language and executive functions. All participants were then assessed with our Executive and Social Cognition Battery, which included Theory of Mind tests (Mind in the Eyes, Faux Pas), the Hotel Task, Multiple Errands Task-hospital version and the Iowa Gambling Task for complex decision-making. Patients were divided into two groups according to their Addenbrooke’s Cognitive Examination scores, a measure of general cognitive status. Low Addenbrooke’s Cognitive Examination patients differed from controls on most tasks of the standard battery and the Executive and Social Cognition Battery. While high Addenbrooke’s Cognitive Examination patients did not differ from controls on most traditional neuropsychological tests, significant differences were found between this high-functioning behavioural variant of frontotemporal dementia group and controls on most measures of our Executive and Social Cognition Battery. Our results suggest that the Executive and Social Cognition Battery used in this study is more sensitive in detecting executive and social cognitive impairment deficits in early behavioural variant of frontotemporal dementia than the classical cognitive measures.

The aim of this investigation was to evaluate improvement of executive functions after shunt surgery in patients with early normal pressure hydrocephalus (NPH). Patients with NPH were assessed before and after shunt surgery with tests shown to be sensitive to damage to the prefrontal cortex (PFC). Significant differences were found between basal and follow-up performances on the Boston Naming Test, the backwards digits span, Part B of the Trail Making Test, and the number of words produced on the phonological fluency task. In conclusion, our study reveals that patients with NPH who respond positively to continuous slow lumbar cerebral spinal fluid drainage and receive a ventriculoperitoneal shunt implant, improve their performance on tasks of executive function. Due to the high demand for this form of mental processing in real-life complex scenarios, and based on the severe executive deficits present in both demented and non-demented NPH patients, we encourage the assessment of executive functions in this clinical group.

Pavlovian trace conditioning depends on the temporal gap between the conditioned and unconditioned stimuli. It requires, in mammals, functional medial temporal lobe structures and, in humans, explicit knowledge of the temporal contingency. It is therefore considered to be a plausible objective test to assess awareness without relying on explicit reports. We found that individuals with disorders of consciousness (DOCs), despite being unable to report awareness explicitly, were able to learn this procedure. Learning was specific and showed an anticipatory electromyographic response to the aversive conditioning stimulus, which was substantially stronger than to the control stimulus and was augmented as the aversive stimulus approached. The amount of learning correlated with the degree of cortical atrophy and was a good indicator of recovery. None of these effects were observed in control subjects under the effect of anesthesia (propofol). Our results suggest that individuals with DOCs might have partially preserved conscious processing, which cannot be mediated by explicit reports and is not detected by behavioral assessment.

OBJECTIVE: To evaluate whether vegetative state patients maintain circadian rhythms. RESEARCH DESIGN: An observational study of five single cases. METHODS AND PROCEDURES:Five chronic vegetative state patients underwent clinical and neurological evaluations and 2-week continuous temperature measurements. MAIN OUTCOMES AND RESULTS: The two patients with traumatic brain injury showed well-formed circadian temperature rhythms and had more reflexive behaviours and relatively low cortical and sub-cortical atrophy, whereas the three patients from anoxic-hypoxic origin demonstrated no cycles or rhythmic behaviour. CONCLUSIONS: The presence of periods of wakefulness does not imply preserved sleep-wake cycling capacity, nor preserved circadian rhythms and it should not be taken as a distinguishing feature for the definition of the vegetative state.

BACKGROUND: The main aim of this study was to compare a large population of patients with bipolar disorder (BD) types I and II strictly defined as euthymic with healthy controls on measures of decision making. An additional aim was to compare performance on a decision-making task between patients with and without a history of suicide attempt.MethodEighty-five euthymic patients with BD-I or BD-II and 34 healthy controls were included. All subjects completed tests to assess verbal memory, attention and executive functions, and a decision-making paradigm (the Iowa Gambling Task, IGT). RESULTS: Both groups of patients had worse performance than healthy controls on measures of verbal memory, attention and executive function. No significant differences were found between BD-I, BD-II and healthy controls on measures of decision making. By contrast, patients with a history of suicide attempt had lower performance in the IGT than patients without a history of suicide attempt. CONCLUSIONS: Patients with euthymic BD-I and BD-II had intact decision-making abilities, suggesting that this does not represent a reliable trait marker of the disorder. In addition, our results provide further evidence of an association between impairments in decision making and vulnerability to suicidal behavior.

BACKGROUND: Theory of mind (ToM) refers to the ability to infer others’ mental states, including intentions and feelings, and is considered to be a critical part of social cognition. Earlier studies in individuals with Parkinson disease (PD) have shown ToM deficits in the more advanced stages of the disease. There is currently no evidence of social cognition deficits in patients in the early stages of PD. METHODS: In this study, we compared patients with early PD (n=36) and a control group of healthy subjects (n=36). Patients were assessed with 2 ToM tasks designed to differentially detect subtle deficits in the affective and cognitive aspects of ToM. Patients were also assessed with a complete neuropsychologic battery which included classic executive tests aimed at investigating the relationship between ToM and executive functions. Performance of medicated (n=16) and unmedicated (n=20) patients was also compared. RESULTS: Our results are the first to indicate that ToM is affected in the early stages of PD. As has already been reported in more advanced stages of PD, such deficits seem to be related to the cognitive aspects of this domain. In our study, these deficits were not related with performance on executive functioning, depression, or medication usage. CONCLUSIONS: These results provide evidence for ToM impairments early in the course of PD. Recognition of ToM impairments in early PD is important, as these deficits may impact patients’ social interactions and quality of life.