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Biomedical subjects

D E Goodkin

Publications and source records attributed to D E Goodkin.

89 records · Page 5Linked to original sources
PubMed1991

Diagnostic criteria for multiple sclerosis research involving multiply affected families.

Existing diagnostic criteria for multiple sclerosis present significant limitations when assessing multiplex families for three reasons: (1) restricting age of onset to 10 to 50 years is likely to exclude 10% of patients known to have a later onset, (2) diagnoses based on subjective information can potentially result in a false-positive diagnosis, and (3) including progressive myelopathies occurring within families, particularly when highly symmetrical, may result in the improper inclusion of genetically determined neurological diseases such as familial spastic paraparesis. The validity of any molecular genetic approach for determining disease susceptibility critically depends on diagnostic accuracy. We present adapted diagnostic criteria that address each of these diagnostic pitfalls unique to multiplex multiple sclerosis family research.

Family↗
PubMed1991

The efficacy of azathioprine in relapsing-remitting multiple sclerosis.

We randomized 59 patients with relapsing-remitting multiple sclerosis to receive azathioprine (AZA) 3.0 mg/kg daily or placebo in a double-masked therapeutic trial. Analysis of data for predetermined primary outcome measures demonstrated a significant difference favoring AZA for observed mean exacerbation rate after 2 years of therapy and time to deterioration in both Ambulation Index and Kurtzke Expanded Disability Status Scale score. This study confirms a modest therapeutic benefit for azathioprine previously reported by other investigators.

Adolescent↗
PubMed1991

EDSS reliability.

Explore the source record for details and available documents.

Disability Evaluation↗
PubMed1990

Screening for cognitive impairment in multiple sclerosis. An evaluation of the Mini-Mental State Examination.

The usefulness of the Mini-Mental State Examination (MMSE) as a screening test for identifying cognitive impairment in patients with multiple sclerosis (MS) has been questioned because many patients who score above the suggested cutoff score of 24 exhibit severe focal or global cognitive deficits. We reevaluated the usefulness of the MMSE by studying 85 patients with clinically definite MS who also received an extensive battery of neuropsychological tests. Although scores on the MMSE were negatively correlated with the number of neuropsychological tests on which patients with MS displayed impairment, the MMSE was not sufficiently sensitive to identify precisely defined dementia in these patients. The MMSE was nonetheless a useful predictor of focal cognitive impairment, particularly in relapsing-remitting patients with relatively minor physical disabilities. Additional analyses suggested strategies for minor modifications of the MMSE that may improve its sensitivity and specificity for identifying dementia while preserving its major advantages as a screening examination: brevity and ease of administration and scoring.

Adult↗
PubMed1990

Clinical and demographic predictors of cognitive performance in multiple sclerosis. Do diagnostic type, disease duration, and disability matter?

Patients with chronic progressive multiple sclerosis often perform more poorly on cognitive tasks than do patients with the relapsing-remitting form of this disease. Whether these differences reflect an independent influence of disease type on cognitive performance is uncertain. We used multiple regression techniques to determine how well performance on a number of tasks done poorly by groups of patients with multiple sclerosis could be predicted by disease type and its confounds: age, disease duration, and disability status as well as other demographic variables. Disease types were assigned longitudinally, based on serial neurological examinations at 6-month intervals over a minimum of 2 years. None of the demographic or clinical variables predicted cognitive performance with more than minimal accuracy. These findings fail to provide support for the assertion that disease type is an important independent determinant of cognitive impairment in multiple sclerosis.

Adult↗
PubMed1989

Seasonal variation of multiple sclerosis exacerbations in North Dakota.

A prospective study of the relationship between exacerbations and season in 336 patients with clinically definite multiple sclerosis showed a significant monthly variation as well as a seasonal pattern of exacerbations. The data obtained are compared with earlier studies that have used varying methodologies. The results continue to support the hypothesis that undefined environmental factors influence the course of multiple sclerosis.

Adolescent↗
PubMed1989

Exacerbation rates and adherence to disease type in a prospectively followed-up population with multiple sclerosis. Implications for clinical trials.

Two hundred fifty-four patients with definite multiple sclerosis were followed up prospectively for 1 to 5 years (mean, 2.6 years). None of the patients received immunosuppressive medication. Yearly exacerbation rates and each patient's adherence to initial disease type were determined. Disease type was defined at entry and prospectively each subsequent year as stable, relapsing remitting stable, relapsing remitting progressive, or chronic progressive. Exacerbation rates determined prospectively did not decline significantly during 3 years of follow-up, even if patients were stratified by disease duration. Adherence to the initially assigned disease type was highly variable. When followed up for 2 years, 30% of patients with chronic progressive disease had conditions become stable, 32% of patients with stable disease had conditions become chronic progressive, 20% of patients with relapsing remitting disease had conditions become stable, and 20% of patients with relapsing remitting disease had conditions become chronic progressive. Patients with stable or relapsing remitting stable disease switched to one of the progressive categories as frequently (44%) as patients with progressive disease stabilized (46%). Progression of disease measured by changes in Kurtzke Expanded Disability Status Scores did not differ between the different disease types. The results challenge dogma regarding the natural history of exacerbation rates and the assumption that we can reliably assign patients to a specific disease type. The findings have important implications for understanding the natural history of multiple sclerosis and designing clinical trials.

Clinical Trials as Topic↗
PubMed1989

Cognitive disturbances in patients with relapsing remitting multiple sclerosis.

The performance of 42 patients with relapsing remitting (RR) multiple sclerosis was compared with that of 24 age-, education-, and gender-matched control subjects on a battery of neuropsychological tests known from previous studies to be sensitive to the impairments of patients with chronic progressive (CP) multiple sclerosis. Like CP patients, RR patients exhibited deficits on tests of information-processing speed, verbal fluency, and problem solving, and on recall measures of anterograde and remote memory. Although a few patients were mildly dysnomic, the RR patients were not generally impaired on visual confrontation naming and they did not exhibit perseverative responding on verbal fluency measures. The pattern of neuropsychological deficits exhibited by RR patients closely approximates the profile observed in other subcortical dementias and does not contain the features of cortical dementia evident in some CP patients. The impairment of RR patients on cognitive tests were less severe than those observed in CP patients in our previous studies. Differences in the age of patients in the CP and RR groups did not account for group differences in the severity of cognitive impairments, but differences in disease duration or severity of disability, as well as disease course, could explain why CP patients exhibit more serious cognitive disturbances than RR patients.

Adult↗
PubMed1989

Frontal lobe dysfunction and memory impairment in patients with chronic progressive multiple sclerosis.

Deficits in semantic encoding have been described in patients with frontal lobe disease who also show memory impairments. As a group, patients with multiple sclerosis (MS) exhibit memory impairment, fail to make effective use of semantic encoding to aid memory, and perform poorly on verbal fluency and concept formation tests which are sensitive to frontal lobe damage. In the present study the semantic encoding capacity of MS patients was measured using a modification of Wicken's release from proactive interference (PI) paradigm. Individual patients varied considerably in the severity of their impairments on verbal fluency, verbal recognition memory and on Wisconsin Card Sorting Task, but even patients who evidenced both memory impairment and signs of frontal lobe dysfunction showed normal release from PI after a categorical shift. Memory disturbances in MS are unlikely to result from an incapacity for semantic encoding, which seems preserved in MS, but may arise instead from deficits in processing information rapidly.

Adult↗
PubMed1988

Anterograde and retrograde amnesia in patients with chronic progressive multiple sclerosis.

The performance of 38 patients with chronic progressive multiple sclerosis was compared with that of 26 age- and education-matched controls on a battery of tests of information-processing speed, verbal fluency, naming, egocentric perception, and anterograde and remote memory. Although there were marked differences in the extent and severity of cognitive disturbance among individual patients, as a group they were impaired compared with controls on all measures. Deficits were most striking on the Symbol-Digit Modalities Test and the verbal fluency measures, tests that require rapid information processing. More than 75% of the patients scored below the tenth percentile for controls on the Symbol-Digit Modalities Test, while 61% scored below the tenth percentile on verbal fluency. Memory disturbances were also common. More than 45% of the patients scored below the tenth percentile. The proportion of impaired patients was quite similar for anterograde and remote memory tests and for recall and recognition procedures. The pattern of memory disturbance and slowed information processing resembled deficits generally observed in subcortical dementias, such as Huntington's disease, but in addition, the patients with multiple sclerosis showed naming difficulties that are usually associated with cortical dementias, such as Alzheimer's disease.

Adult↗
PubMed1988

Upper extremity function in multiple sclerosis: improving assessment sensitivity with box-and-block and nine-hole peg tests.

The need for standardized operational definitions, as well as a more sensitive, easily applied, and reproducible upper extremity functional assessment for following patients in multiple sclerosis clinical trials is evident. Experience with the use of two upper extremity functional assessment instruments--the nine-hole peg test (9HPT) and the box-and-block test (BBT)--is described. The patients, who were followed for six months, experienced subjective change in functional status but failed to show significant change on the pyramidal, cerebellar, or visual functional scores of the Kurtzke Expanded Disability Status Scale (EDSS). The prevalence of upper extremity dysfunction in multiple sclerosis, as measured by the 9HPT and the BBT, is higher than previously appreciated. The 9HPT and BBT are more sensitive in detecting upper extremity functional status change than the EDSS and should prove helpful in following patients in clinical trials.

Adult↗
PubMed1987

Cyclophosphamide in chronic progressive multiple sclerosis. Maintenance vs nonmaintenance therapy.

Twenty-seven patients with chronic progressive multiple sclerosis were treated with high-dose intravenous cyclophosphamide induction on either an impatient or outpatient basis. Following induction, patients were randomized to alternate-month outpatient "maintenance" or "no maintenance" therapy. These groups, as well as 24 nonrandomized control patients, were compared with each other after 12, 18, and 24 months of follow-up. All groups were similar in age, sex, duration of disease, and degree of disability before treatment. Fifty-nine percent of all cyclophosphamide-treated patients were stable at 12 months compared with 17% of all patients in the nonrandomized control group at 12 months. A statistically significant difference persisted at 18 and 24 months. A trend favoring maintenance therapy when compared with no maintenance therapy was evident at 12, 18, and 24 months, but was not statistically significant. Inpatient vs outpatient induction therapy failed to influence treatment outcome. Toxic side effects of nausea and vomiting presented a serious obstacle to maintenance therapy as administered in this protocol.

Administration, Oral↗
PubMedDate not supplied

Volume T1-weighted gradient echo MRI in multiple sclerosis patients.

Three-dimensional (3D) volume turbo fast low angle shot (FLASH) techniques have become available which produce heavily T1-weighted images, similar to inversion recovery scans, utilizing the appropriate flip angle and inversion time. The purpose of this study was to compare the sensitivity of a rapid volume gradient echo technique [3D magnetization prepared rapid acquisition gradient echo (MP RAGE)] in identifying multiple sclerosis (MS) plaques with a conventional T2-weighted spin echo (SE) sequence. Ten patients with clinical MS were evaluated. Patients underwent a routine examination consisting of an axial T2-weighted SE sequence (2,500,22/90) and a coronal 3D MP RAGE, 10/4/10, acquired as 128 two mm partitions. In six patients, area measurements of 22 plaques were determined on both the axial T2-weighted SE examinations and the axial reformatted MP RAGE examinations. The overall number of plaques utilizing each technique was approximately the same. One hundred twenty-two plaques were visualized for the 3D MP RAGE sequence, and 128 plaques for the T2-weighted SE sequence. There were differences in detection of plaques in different regions, with plaques in gray matter better demonstrated utilizing the conventional T2-weighted SE sequence. Plaques in the corpus callosum, pons, and brachia pontis were better demonstrated utilizing 3D MP RAGE. No significant difference was found between the areas measured on the MP RAGE sequence and on the T2-weighted SE sequence. Three-dimensional MP RAGE provides a sensitive and complementary method to conventional T2-weighted SE sequences in the evaluation of patients with MS.

Adult↗
PubMedDate not supplied

Access to semantic memory in Parkinson's disease and multiple sclerosis.

The capacity to access semantic memory and the efficiency of the memory search were studied in patients with Parkinson's disease (PD) or multiple sclerosis (MS) using a modified version of the "Supermarket Test." PD and MS patients who performed normally on the Boston Naming Test generated as many specific exemplars and category labels as did controls, and these patients also searched their semantic memories as efficiently as controls. In contrast, PD and MS patients with impaired confrontational naming generated fewer specific exemplars, but a normal number of generic category labels. These naming-impaired patients searched their semantic memories less efficiently than controls. For both MS and PD patients, the efficiency of semantic memory search was significantly correlated with measures that reflect access to semantic memory (eg, performance on letter or category fluency tasks), but was not significantly related to measures of problem-solving ability derived from the Wisconsin Card Sorting Test. Inefficient search of semantic memory in MS and PD appears to arise from an inability to retrieve knowledge from semantic memory quickly or from the loss of information from semantic memory stores, rather than from impairment in the ability to formulate and deploy appropriate strategies for task solution.

Aged↗
PubMedDate not supplied

Experimental therapies for multiple sclerosis: current status.

Multiple sclerosis is the most common cause of nontraumatic disability affecting young adults in the United States. Its etiology remains unclear, but evidence points to alteration of normal immune system function in the pathogenesis of this illness. This article reviews the results of clinical trials of experimental therapeutic agents in multiple sclerosis. The background and action of each treatment are described, and the clinical experience with each agent is reviewed. Obstacles to evaluating the effectiveness of treatment methods in MS and challenges to the design of future clinical trials and the interpretation of trial outcomes are discussed.

Azathioprine↗
PubMedDate not supplied

Pharmacotherapy of multiple sclerosis: current status.

Pharmacotherapy plays an important part in the overall management of patients with multiple sclerosis. Most therapies directed at altering the natural history of the underlying disease process are only partially effective or are controversial or experimental. However, many effective symptomatic therapies are available to the clinician. The action and uses of corticosteroids in multiple sclerosis are discussed, and approaches to the treatment of spasticity, paroxysmal disorders, bladder dysfunction, cerebellar ataxia, neurobehavioral manifestations, fatigue, and acute and chronic pain in patients with multiple sclerosis are examined.

Acute Disease↗