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 172,036 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aim of this study is to investigate the changes of the pupil's light reflex (PLR) and mobility in Parkinson's disease (PD) patients with and without cognitive disorder. Twenty two (22) patients (ten males, twelve females, mean age: 72.7+/-7.3 years) with identified PD entered the study. The patients were examined with the Mini Mental State Examination (MMSE), the Wechsler II Memory Scale (WMS II) and the Hamilton Depression Scale (HAM-D17). Eleven (11) patients (five males, six females, mean age: 72.09+/-7.06 years) were free of any cognitive deficits and eleven (11) patients (five males, six females, mean age: 73.36+/-7.55 years) had cognitive disorder according to the aforementioned scales. None of the patients satisfied the DSM-IV-TR criteria for depression or anxiety disorder. The patients underwent a pupillometric study in both eyes with single flash stimuli of 24.6 candelas/m(2) intensity and 20 ms duration. The pupillometric parameters that were studied were: Latency for the onset of Constriction (T1), Baseline Pupil Radius (R1), Minimum Pupil Radius after the pupil reaction to light (R2), Amplitude (AMP, R1-R2), Time for maximum Miosis (T2), Maximum Constriction Velocity (VCmax) and Maximum Constriction Acceleration (ACmax). The pupillometric findings of each group were compared to those of an age and sex matched group of eleven healthy subjects. Furthermore, a comparison between the findings of the two groups was conducted. ACmax and VCmax were significantly lower in patients without (PD) and with coexisting cognitive impairment (PDC) compared to normal subjects (NC) (p<0.001). Patients with cognitive impairment (PDC) had significantly lower levels of ACmax, VCmax and AMP than patients without cognitive deficits (PD). Cognitive impairment in PD, which mainly reflects a central cholinergic deficit, may be a crucial pathogenetic factor for the decrease in the aforementioned pupillometric parameters. VCmax and ACmax can be considered as the most sensitive indicators of this central cholinergic deficiency.
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PMID:Pupillometric findings in patients with Parkinson's disease and cognitive disorder. 1904 1

We report an interesting case of a patient with Williams syndrome who presented with moderate supravalvar aortic stenosis and bilateral pulmonary artery stenosis at one week of age. The supravalvar aortic stenosis became severe by the age of one month with severe depression of left ventricular function. The patient had a difficult postoperative course, developed an acquired aortic arch hypoplasia and required multiple interventions during the first two months of life with an excellent outcome. The management of this difficult patient is discussed with focus on the importance of close follow-up, early diagnosis and early surgical intervention in improving the outcome in this difficult group of patients.
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PMID:Management of supravalvar aortic stenosis and severely depressed left ventricular function in a neonate with Williams syndrome. 1923 91

Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system very heterogeneous in its characteristics. In contrast to the well known sensitive/motor deficits, the cognitive dysfunction has only been analyzed in the last few decades. Attention, executive function, and memory were assessed in 28 patients with recurrent-remittent MS (RRMS) (duration, median 7 years; EDSS median 2) by means of a specific neuropsychological battery. Depression (BDI), anxiety (STAI) and fatigue (FSS) were also assessed. Twenty-five of these patients were selected for statistical study because they presented deficits in some cognitive areas. Twenty-four percent of the patients displayed memory deficits and 80% showed attention and executive function deficits related to prefrontal lobe function. No global memory difficulties were found, except for immediate visual memory of complex elements (immediate recall of the Rey figure), although the visual reproduction I subtest of the WMS-R was unaffected. In RRMS patients with a relatively short duration and low level of incapacity, cognitive impairments mainly affected prefrontal functions. The difficulties in immediate visual memory of complex elements could also be explained by a failure in these areas, due to the alteration of the organization and strategic use of the material to be encoded.
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PMID:Executive function and memory in patients with relapsing-remitting multiple sclerosis. 1962 22

Although many researchers have investigated emotional and behavioral difficulties in individuals with Williams syndrome, few have used standardized diagnostic assessments. We examined mental health problems in 92 adults with Williams syndrome using the Psychiatric Assessment Schedule for Adults with Developmental Disabilities-PAS-ADD (Moss, Goldberg, et al., 1996). Factors potentially associated with mental health problems were also explored. The PAS-ADD identified mental health problems in 24% of the sample. The most common were anxiety (16.5%) and specific phobias (12%). Other diagnoses included depression, agoraphobia, and social phobia. No association was found between the presence of mental health problems and either individual (e.g., age, IQ, language level) or external (life events) variables.
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PMID:Mental health problems in adults with Williams syndrome. 2002 56

Williams-Beuren syndrome (WBS) is a genetically determined neurodevelopmental disorder caused by a heterozygous deletion of 26-28 genes on chromosome band 7q11.23. During the past few years, researchers and clinicians have significantly contributed to define the phenotype of the syndrome, including its cognitive and behavioral aspects. However, it is not well known yet whether the psychological problems are specific to the syndrome or secondary to the intellectual disability (ID). The aim of our study was to better define the psychopathological profile of WBS and whether or not it is related with IQ or anxiety symptoms. Twenty-five subjects (12 girls, 13 boys) with a diagnosis of WBS were compared to 27 boys with Fragile X Syndrome and to 24 boys with ID of non-specific etiology using the Child Behavior Checklist. Anxiety, depression and attention problems were the main behavioral problems found in WBS with no gender differences. Significant differences between cohorts were observed in somatic complaints, delinquent behavior, aggressive behavior, and externalizing problems. Some associations between IQ and anxiety items were found. The findings are discussed in terms of behavioral phenotypes, genetic implications and ID.
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PMID:Behavioral features of Williams Beuren syndrome compared to Fragile X syndrome and subjects with intellectual disability without defined etiology. 2121 40

This is a preliminary report on the safety and efficacy of 1.5 ATA hyperbaric oxygen therapy (HBOT) in military subjects with chronic blast-induced mild to moderate traumatic brain injury (TBI)/post-concussion syndrome (PCS) and post-traumatic stress disorder (PTSD). Sixteen military subjects received 40 1.5 ATA/60 min HBOT sessions in 30 days. Symptoms, physical and neurological exams, SPECT brain imaging, and neuropsychological and psychological testing were completed before and within 1 week after treatment. Subjects experienced reversible middle ear barotrauma (5), transient deterioration in symptoms (4), and reversible bronchospasm (1); one subject withdrew. Post-treatment testing demonstrated significant improvement in: symptoms, neurological exam, full-scale IQ (+14.8 points; p<0.001), WMS IV Delayed Memory (p=0.026), WMS-IV Working Memory (p=0.003), Stroop Test (p<0.001), TOVA Impulsivity (p=0.041), TOVA Variability (p=0.045), Grooved Pegboard (p=0.028), PCS symptoms (Rivermead PCSQ: p=0.0002), PTSD symptoms (PCL-M: p<0.001), depression (PHQ-9: p<0.001), anxiety (GAD-7: p=0.007), quality of life (MPQoL: p=0.003), and self-report of percent of normal (p<0.001), SPECT coefficient of variation in all white matter and some gray matter ROIs after the first HBOT, and in half of white matter ROIs after 40 HBOT sessions, and SPECT statistical parametric mapping analysis (diffuse improvements in regional cerebral blood flow after 1 and 40 HBOT sessions). Forty 1.5 ATA HBOT sessions in 1 month was safe in a military cohort with chronic blast-induced PCS and PTSD. Significant improvements occurred in symptoms, abnormal physical exam findings, cognitive testing, and quality-of-life measurements, with concomitant significant improvements in SPECT.
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PMID:A phase I study of low-pressure hyperbaric oxygen therapy for blast-induced post-concussion syndrome and post-traumatic stress disorder. 2256 91

Prior research suggests that individuals with Williams syndrome (WS) have a disposition towards anxiety. Information regarding this is typically derived from parents and carers. The perspectives of the individuals with WS are rarely included in research of this nature. We examined the mental health of 19 adults with WS using explicit (psychiatric interview) and implicit (modified Stroop task) measures and compared informant (parents/carers) and respondent (adults with WS) reports of psychiatric symptoms. Informants and respondents both reported more symptoms of anxiety (n=7-9) than depression (n=2). Strong positive correlations were found between informant and respondent reports of symptoms of mental health problems. Compared to informants, respondents reported significantly more symptoms overall and somewhat more symptoms of anxiety. Results from the Stroop task indicated that the adults with WS were more vigilant to anxiety-related words than to depression-related words. The adults with WS provided reliable information regarding their mental health, thus providing further evidence that anxiety is part of the behavioural phenotype of the syndrome.
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PMID:Examining reports of mental health in adults with Williams syndrome. 2209 59

Symptom validity tests (SVTs) are commonly used to assess effort in neuropsychological evaluations. However, no empirical research or official guidelines exist about how clinicians should proceed if a patient produces a non-valid SVT result. The purpose of this study was to examine whether confronting patients immediately after scoring in a non-valid range on a SVT would have an impact on subsequent symptom validity and memory tests performance. Archival patient data for 507 adults with clinically definite multiple sclerosis (MS) (ages 18-76) were examined. All patients completed the Victoria Symptom Validity Test (VSVT), the Wechsler Memory Scale, 3rd edition (WMS III), and the Beck Depression Inventory, 2nd edition (BDI II). Although the majority (89%) of patients produced valid VSVT scores (the Valid group), 56 patients produced non-valid VSVT scores. Due to a change in clinical procedure, 28 of the 56 were confronted regarding their non-valid VSVT performances and were asked to complete the test a second time (the CONF group), while the remaining 28 proceeded with testing as usual following a non-valid score (the N-CONF group). Results showed that 68% of the CONF group produced valid VSVT scores on re-administration, as well as memory performances that were comparable to those of the Valid group. In contrast the N-CONF group produced memory scores that were significantly below the Valid group. This is the first study to provide empirical support for the effectiveness of intervention when patients exhibit inadequate effort on SVTs in clinical, non-forensic settings.
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PMID:Confronting patients about insufficient effort: the impact on subsequent symptom validity and memory performance. 2306 72

Sleep and related maternal beliefs were assessed in a narrow age range of 18 children with Williams syndrome (WS) and 18 typically developing (TD) children. WS is a rare genetic disorder characterised by a complex physical, cognitive and behavioural phenotype. High prevalence of sleep difficulties in older children and adults with WS have been reported. Parents completed 6 questionnaires: the Brief Infant Sleep Questionnaire, Infant Sleep Vignettes Interpretation Scale, Pittsburgh Sleep Quality Index of Parents, Child Behaviour Checklist, MacArthur Communicative Development Inventory for Infants - Words and Gestures, and the Major (ICD-10) Depression Inventory. Compared to TD children, those with WS had shorter night sleep, more night wakings and wakefulness according to parental report. Regression analyses revealed that a proportion of the variance in language development scores in WS children could be explained by night sleep duration. Compared to control parents, the mothers of the WS group were more likely to describe their child's sleep as problematic and had higher rates of involvement with child sleep, yet they had a lesser tendency to interpret sleep problems as signs of distress and a greater tendency to emphasise limit setting. Approximately half of both groups of mothers experienced poor sleep quality. This was also related to maternal mood, and night wakefulness in the children with WS. This is the first study to quantify sleep difficulties in young children with WS in a narrow age range using maternal report. The possible negative effects on maternal sleep and mood, and the link between night sleep and language development in young children with WS, requires further detailed investigation.
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PMID:Sleep problems and language development in toddlers with Williams syndrome. 2402 9

Monocyte chemoattractant protein-1 (MCP-1) and its receptor CC chemokine receptor-2 (CCR2) play important roles in neuroinflammation and they have been shown to be involved in Parkinson's disease (PD) pathogenesis. In addition, several studies have suggested a role for the MCP-1 and CCR2 genotypes in cognitive impairment and depression, which are common non-motor symptoms in PD patients. In this study, a cohort of 521 PD patients and 556 cases of healthy controls were recruited to investigate the association between the MCP-1 2518A/G (rs1064211) and CCR2 V64I (rs1799864) gene polymorphisms and PD risk in the Chinese population. We also analyze the influence of these genotypes on the cognitive function and depression in PD patients by comparing Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), Wechsler Adult Intelligence Scale-Chinese Revision (WAIS-RC), Wechsler Memory Scale-Chinese Revision (WMS-RC) and Hamilton Depression Rating Scale (HAMD) ratings in 217 PD patients. Our results showed no significant differences in the genotype frequency between the PD group and the control group (P > 0.05). In addition, we also failed to find an influence of the MCP-1 and CCR2 genotypes on MMSE scores, MoCA scores, WAIS-RC scores, WMS-RC scores and HAMD scores in PD patients (P > 0.05). The MCP-1 and CCR2 gene polymorphisms may not be genetic risk factors for PD in the Han Chinese population, and they do not appear to influence cognitive function and depression in PD patients.
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PMID:MCP-1 and CCR2 gene polymorphisms in Parkinson's disease in a Han Chinese cohort. 2537 Sep 17


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