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Biomedical subjects

Jim Grigsby

Publications and source records attributed to Jim Grigsby.

At least 19 recordsLinked to original sources
PubMed2007

Progression of tremor and ataxia in male carriers of the FMR1 premutation.

Premutation alleles of the fragile X mental retardation 1 (FMR1) gene give rise to a late-onset movement disorder, fragile X-associated tremor/ataxia syndrome (FXTAS), characterized by progressive intention tremor and gait ataxia, with associated dementia and global brain atrophy. The natural history of FXTAS is largely unknown. To address this issue, a family-based, retrospective, longitudinal study was conducted with a cohort of 55 male premutation carriers. Analysis of the progression of the major motor signs of FXTAS, tremor and ataxia, shows that tremor usually occurs first, with median onset at approximately 60 years of age. From the onset of the initial motor sign, median delay of onset of ataxia was 2 years; onset of falls, 6 years; dependence on a walking aid, 15 years; and death, 21 years. Preliminary data on life expectancy are variable, with a range from 5 to 25 years.

Adult↗
PubMed2006

Impairment in the cognitive functioning of men with fragile X-associated tremor/ataxia syndrome (FXTAS).

Disorders associated with fragile X syndrome involve a trinucleotide (CGG) repeat expansion in the FMR1 gene. Recently, a progressive movement disorder (fragile X-associated tremor/ataxia syndrome [FXTAS]) has been identified in premutation carriers, persons with 55 to 200 CGG repeats. In addition to ataxia, action tremor, and Parkinsonism, early case reports suggested that FXTAS involves impaired cognition, but the precise nature of the impairment has not been elucidated. In this first, preliminary study of the subject, circumscribed aspects of cognitive functioning were examined in 25 men with FXTAS. Subjects' performance on the cognitive tests was compared with normative data. Scores on two measures of executive cognitive functioning showed a high prevalence of substantial impairment. Capacity for inhibition was severely affected in one-quarter of this highly educated sample; information processing speed was profoundly impaired in most subjects. Although mean verbal and performance IQ scores were not significantly different from the general population, they were quite low given the sample's educational level. Cognitive and functional impairment was greater for men with more CGG repeats, although number of repeats was not associated with age of onset of either tremor or ataxia. The results provide evidence that FXTAS involves marked impairment of executive cognitive abilities.

Aged↗
PubMed2006

Research recommendations for the american telemedicine association.

The American Telemedicine Association (ATA) convened a panel of experts to generate a research agenda for the telemedicine community to further support and promote the long-term acceptance and use of telehealth. Three principles to guide research and four key areas within which research is greatly needed were identified. These four areas are technical, clinical, human factors and ergonomics, and economic analyses. It is the hope of the panel that the research recommendations put forth in this document will give investigators the inspiration, tools and goals to make this happen.

Health Planning Guidelines↗
PubMed2006

Cognitive impairment in a 65-year-old male with the fragile X-associated tremor-ataxia syndrome (FXTAS).

OBJECTIVE: This is the first case report of a comprehensive neuropsychologic examination of an older man with the fragile X-associated tremor-ataxia syndrome (FXTAS). BACKGROUND: FXTAS, a newly identified phenotype affecting older male carriers of the fragile X premutation allele, is a progressive disorder marked by gait ataxia, action tremor, peripheral neuropathy, executive cognitive deficits, generalized brain atrophy, and neuronal and astrocytic intranuclear inclusion bodies throughout the brain. The patient previously had undergone neurologic evaluation, molecular analysis, and magnetic resonance imaging. METHOD: The patient was administered a neuropsychologic examination, assessing motor and somatosensory functioning, visual and spatial functioning, speech and language, attention, executive abilities, learning and memory, and reasoning. RESULTS: The patient showed a pattern of cognitive impairment characterized by essentially normal speech and language, moderately impaired control of attention, and moderate to severe deficits in working memory, executive functioning, and both declarative and procedural learning. Visual and spatial abilities were relatively unimpaired, and verbal reasoning was only mildly deficient. CONCLUSIONS: The findings suggest that a cognitive disorder, with especially marked executive cognitive function and memory deficits, accompanies FXTAS. The findings in FXTAS are compared with those in several other neurodegenerative disorders.

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PubMed2006

Alternatives to randomized controlled trials in telemedicine.

Research designs other than true experiments may be useful in the evaluation of telemedicine. Potential methods include those that do not rely on randomization and tight control of the intervention, and analysis of existing administrative and clinical databases. Quasi-experimental designs may also be useful, especially when conducted in association with careful statistical methods that allow the investigator to control for certain differences between groups. Databases, such as those maintained by the Centers for Medicare and Medicaid Services, contain information on both outcomes and claims, as well as disease/procedure registries and electronic health records. This may provide a potential tool for understanding the effects of telemedicine on access to care in conjunction with costs and quality. These different approaches have advantages and disadvantages, but may be useful in telemedicine, where the conduct of randomized controlled trials is generally very expensive and frequently not feasible.

Health Services Research↗
PubMed2006

Psychiatric phenotype of the fragile X-associated tremor/ataxia syndrome (FXTAS) in males: newly described fronto-subcortical dementia.

OBJECTIVE: The authors describe and quantify the neuropsychiatric symptoms present in a cohort of males with the fragile X mental retardation 1 (FMR1) premutation allele who have developed fragile X-associated tremor/ataxia syndrome (FXTAS). METHOD: Fourteen male carriers of the FMR1 premutation who had clinical manifestations of the FXTAS syndrome and 14 age- and education-matched controls were assessed with the Neuropsychiatric Inventory (NPI), formal cognitive testing, and genetic analysis. RESULTS: Males with FXTAS had significantly higher total NPI scores (p < .004) and significantly higher scores on the agitation/aggression (p < .004), depression (p < .004), apathy (p < .004), disinhibition (p < .004), and irritability (p < .004) scales, compared with controls. Cognitive performances on the Mini-Mental State Examination did not correlate with severity of symptoms on the NPI. CONCLUSIONS: The neuropsychiatric manifestations of FXTAS, based on this preliminary report, appear to cluster as a fronto-subcortical dementia. Clinicians encountering patients with clinical dementia with motor symptoms suggesting FXTAS should consider genetic testing to determine whether the patient's dementia syndrome is secondary to a fragile X premutation carrier status.

Aged↗
PubMed2005

Assessing motor and cognitive regulation in AD, MCI, and controls using the Behavioral Dyscontrol Scale.

The objective of this study was to evaluate the utility of the Behavioral Dyscontrol Scale (BDS) as a measure of behavioral regulation in Alzheimer's disease (AD) and mild cognitive impairment (MCI). Two patients groups (n=40 MCI and 40 AD) recruited from a memory clinic and an elderly control (EC) group (n=40) recruited from the community were administered a battery of neuropsychological tests including the BDS and a measure of functioning of activities of daily living (ADLs). Results of ROC analyses revealed that performance on the BDS discriminated between the AD group and the MCI and EC groups but did not discriminate between the MCI and EC groups. Performance on the BDS was an independent predictor of ADLs in patient groups after controlling for the effects of performance on a memory measure.

Activities of Daily Living↗
PubMed2005

Racial and ethnic disparities in the outcomes of elderly home care recipients.

The purpose of this study was to examine racial/ethnic disparities in functional outcomes of elderly home health care recipients. Analyses were conducted using Outcome and Assessment Information Set (OASIS) data for a nationally representative sample of home health care episodes for patients aged 65 and older. Risk-adjusted regression analyses examined the association between race/ethnicity and functional outcomes. Fourteen outcome measures reflected improvement in specific functional areas (e.g., ambulation) and two reflected overall functional change. Non-Hispanic Whites (Whites) experienced substantially better functional outcomes than did home health care recipients of other racial/ethnic backgrounds. The disparity in outcomes was most pronounced between Whites and African Americans.

Aged↗
PubMed2004

Predictors of cognitive decline following coronary artery bypass graft surgery.

BACKGROUND: A significant number of patients develop cognitive impairment that persists for months following coronary artery bypass grafting (CABG) surgery. Our objectives were to identify patient-related risk factors, processes of care, and the occurrence of any perioperative complications associated with cognitive decline. METHODS: Nine hundred thirty-nine patients enrolled in the Processes, Structures, and Outcomes of Care in Cardiac Surgery study undergoing CABG-only surgery at 14 Veterans Administration medical centers between 1992 and 1996 completed a short battery of cognitive tests at baseline and 6-months post-CABG. The composite cognitive score was based on the sum of errors for each individual item in the battery. Multiple linear regression analyses were used to identify independent predictors of the 6-month composite cognitive score. RESULTS: In multivariable analyses, patient characteristics associated with cognitive decline included cerebrovascular disease (p = 0.009), peripheral vascular disease (p = 0.007), history of chronic disabling neurologic illness (p = 0.016), and living alone (p = 0.049), while the number of years of education (p = 0.001) was inversely related to cognitive decline. After adjustment for baseline patient risk factors, the presence of any postoperative complication(s) (p = 0.001) was also associated with cognitive decline while cardiopulmonary bypass time (p = 0.008) was inversely related to cognitive decline. CONCLUSIONS: Patients with noncoronary manifestations of atherosclerosis, chronic disabling neurologic illness, or limited social support are at risk for cognitive decline after CABG surgery. In contrast, more years of education were associated with less cognitive decline. Preoperative assessment of risk factors identified in this study may be useful when counseling patients about the risk for cognitive decline following CABG surgery.

Aged↗
PubMed2003

Fragile X premutation tremor/ataxia syndrome: molecular, clinical, and neuroimaging correlates.

We present a series of 26 patients, all >50 years of age, who are carriers of the fragile X premutation and are affected by a multisystem, progressive neurological disorder. The two main clinical features of this new syndrome are cerebellar ataxia and/or intention tremor, which were chosen as clinical inclusion criteria for this series. Other documented symptoms were short-term memory loss, executive function deficits, cognitive decline, parkinsonism, peripheral neuropathy, lower limb proximal muscle weakness, and autonomic dysfunction. Symmetrical regions of increased T2 signal intensity in the middle cerebellar peduncles and adjacent cerebellar white matter are thought to be highly sensitive for this neurologic condition, and their presence is the radiological inclusion criterion for this series. Molecular findings include elevated mRNA and low-normal or mildly decreased levels of fragile X mental retardation 1 protein. The clinical presentation of these patients, coupled with a specific lesion visible on magnetic resonance imaging and with neuropathological findings, affords a more complete delineation of this fragile X premutation-associated tremor/ataxia syndrome and distinguishes it from other movement disorders.

Aged↗
PubMed2003

The fragile X premutation presenting as essential tremor.

CONTEXT: The fragile X premutation has recently been reported to be associated with a neurodegenerative syndrome, chiefly characterized by intention tremor, gait ataxia, and executive cognitive deficits in men older than 50 years. Essential tremor is a frequent cause of tremor in elderly patients and in some cases is associated with impaired tandem gait and cognitive deficits. OBJECTIVE: To describe 2 fragile X carriers whose clinical presentation mimicked essential tremor. DESIGN: The 2 patients described herein underwent neurologic examinations by experienced movement disorders neurologists, magnetic resonance imaging, and fragile X gene, messenger RNA, and protein analyses. One underwent detailed neuropsychological testing. SETTING: Patients were studied at 2 large university movement disorders clinics. PATIENTS: Both patients were white men older than 50 years who had been diagnosed as having essential tremor and then found to be fragile X carriers. RESULTS: Besides disabling intention tremor, the 2 patients had impaired tandem, generalized brain atrophy, and unusual bilateral T2 middle cerebellar hyperintensities on magnetic resonance imaging. The patient who underwent neuropsychological testing had frontal executive deficits. Both patients had elevated fragile X mental retardation gene 1 messenger RNA and reduced fragile X mental retardation 1 protein levels. CONCLUSION: The fragile X carrier state may underlie the clinical findings in some older men diagnosed as having essential tremor.

Aged↗
PubMed2003

Effect of the fragile X status categories and the fragile X mental retardation protein levels on executive functioning in males and females with fragile X.

The effects of a fragile X disorder on executive function impairment were assessed in 144 extended families, which included individuals with fragile X premutation and full mutation and their relatives without fragile X. A modification of the maximum-likelihood estimators for pedigree data, as well as ordinal logistic regression, were used in data analysis. The most outstanding deficit, occurring especially in males, involved impaired capacity to use an intention to regulate purposeful behavior. This deficit occurred independently of general cognitive impairment but was related to depletion of fragile X mental retardation 1 gene protein product. The other executive function deficits were accounted for by the general cognitive impairment. Possible mechanisms of the effect of fragile X premutation on impairments of executive functioning are considered.

Adolescent↗
PubMed2002

Neuropsychologic impairment and MRI abnormalities associated with chronic solvent abuse.

OBJECTIVE: Individuals chronically abusing organic solvents have been reported in the medical literature for almost four decades to have a variety of neurological abnormalities. Most have been single case reports or studies in small numbers of individuals. The purpose of this study was to evaluate the neurologic and neuropsychologic effects of chronic solvent abuse in a larger group of individuals and compare the results to a control group of other drug abusers. Attempts to estimate a dose-response relationship between solvent abuse and either neuropsychological tests or magnetic resonance imaging (MRI) abnormalities were also undertaken. METHODS: A sample of 55 solvent abusers was compared to a sample of 61 users of other drugs, especially cocaine and alcohol, on a battery of cognitive and neuropsychological tests. Fifty of the solvent abusers and 51 members of the comparison sample also underwent MRI of the brain. Comparisons were made by means of multivariate analysis of variance and covariance. RESULTS: Compared to the general population, both groups performed poorly, scoring below the mean on most neuropsychological measures. Solvent abusers performed more poorly than the comparison sample on measures of working memory and executive cognitive functions. Both groups showed evidence of abnormal MRI findings, but a greater percentage of the solvent abuse group showed such abnormalities. No clear dose-response relationship was seen between solvent abuse and abnormalities on neuropsychological tests, but a strong dose-response relationship was seen in the presence of MRI abnormalities. CONCLUSION: The findings in this study document widespread cognitive impairment, which could not be attributed to the effects of solvents entirely. These findings expand upon previous data regarding neurological abnormalities in solvent abusers, by demonstrating a dose-response relationship with MRI abnormalities. An unexpected finding was the high prevalence of neuropsychological impairment and MRI abnormalities among the comparison group. The results of this study has implications regarding low-level solvent exposure, as in the occupational setting, suggesting that MRI may be a more useful tool to sort out the presence of neurological abnormalities rather than neuropsychological tests. Since no dose-response relationship was seen between solvent abuse and neuropsychological testing, reliance upon these tests in assessing neurological injury from solvents is a highly dubious practice.

Adult↗
PubMed2002

Memory, neurodynamics, and human relationships.

In this article we discuss the implications of the functional organization and dynamics of the brain for understanding human relationships. In particular, we focus on the brain's multiple memory systems and the various roles they play in organizing the interactions of people as they come to know one another. The distinction between the relatively independent declarative, procedural, and emotional learning systems is especially significant in this regard, as the former mediates what we know about one another, the second mediates what we do with one another, and the third affects behavior by altering our emotional state. Knowledge of the functioning of these dissociable memory systems provides a novel perspective on relationships--both ordinary social relationships and those that develop in psychotherapy--and further illuminates psychotherapeutic transference and countertransference phenomena. We begin with a review of the neural basis of these processes, then turn our attention to the interpersonal level of analysis.

Brain↗
PubMedDate not supplied

Prevalence of disorders of executive cognitive functioning among the elderly: findings from the San Luis Valley Health and Aging Study.

The San Luis Valley Health and Aging Study is a population-based epidemiologic study of chronic illness and disability among Hispanic and non-Hispanic white persons over the age of 60 in two counties in rural southern Colorado, USA. Between 1993 and 1995, we examined the prevalence of impaired executive cognitive functioning in a sample of 1,313 individuals living both in the community and in nursing homes. Overall, 1 person in 3 was found to have at least mild impairment, while 1 in 6 had moderate to severe deficits. Increasing levels of executive functioning impairment were associated with lower education, advancing age, and Hispanic ethnicity. After controlling for education and acculturation, the differences by ethnic group were no longer significant.

Aged↗