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Biomedical subjects

S J Huber

Publications and source records attributed to S J Huber.

At least 19 recordsLinked to original sources

Emotional changes with multiple sclerosis and Parkinson's disease.

Multiple sclerosis (MS) and Parkinson's disease (PD) are relatively common neurological disorders. Both disorders are chronic and progressive, produce varying degrees of physical disability, and result in characteristic neuropathological changes to a variety of subcortical brain structures. Patients with MS or PD also exhibit a higher prevalence of emotional disorders relative to other patient groups with comparable degrees of physical disability. The present review (a) examines specific methodological issues associated with research in this area, (b) describes the range and severity of emotional disorders in MS and PD, and (c) examines both endogenous and reactive explanations to account for the increased prevalence of emotional dysfunction in these two disorders. Suggestions for future research are offered, as well as implications for treatment.

Adult

Magnetic resonance imaging correlates of neuropsychological impairment in multiple sclerosis.

The authors examined whether specific neuropsychological abnormalities in multiple sclerosis (MS) are associated with focal lesion areas detected by MRI. Lesion area, regardless of distribution, correlated with performance on the vast majority of neuropsychological procedures. No significant difference appeared between groups with normal/mild and moderate overall cognitive impairment on any of the MRI measures. However, patients with severe cognitive impairment had greater lesion area, regardless of location, and had significant atrophy of the corpus callosum compared with the other two groups. These results suggest that severe atrophy of the corpus callosum reflects global disease and provides a relatively focal morphological marker of severe cognitive impairment in MS.

Adult

Olfactory function in essential tremor.

Olfactory function, assessed by the University of Pennsylvania Smell Identification Test, was normal in essential tremor (ET) patients and significantly reduced in patients with Parkinson's disease (PD). This finding further supports a lack of association between ET and PD.

Female

Metabolism of glucurolactone to glucarate in Parkinson's disease.

The metabolism of oral glucurolactone to serum D-glucarate by the hepatic cytochrome P450 enzyme system was no different in 20 untreated Parkinson's disease (PD) patients as compared with 20 age- and sex-matched controls. There was no evidence for a deficit in hepatic enzymes in PD.

Administration, Oral

Magnetic resonance imaging in Parkinson's disease.

Magnetic resonance imaging was utilized to examine changes in the pars compacta associated with Parkinson's disease. We found a reduction in average width of the pars compacta in advanced- but not early-stage patients. Conversely, a significant difference in width (right minus left) was observed in early- but not late-stage patients. Width asymmetry may be a sensitive magnetic resonance imaging indicator for the early detection of Parkinson's disease when there is a predominance of unilateral clinical symptoms.

Adult

The pattern of depressive symptoms varies with progression of Parkinson's disease.

Whether or not depressive symptoms increase in severity with progression of Parkinson's disease (PD) remains uncertain. Unlike previous studies, we examined whether the severity of specific features of depression (mood, self reproach, vegetative, and somatic symptoms) differ with respect to the progression of PD. Results indicated that symptoms related to both mood and self-reproach were present in the early stages of PD but did not increase in severity with advancing disease. Somatic features of depression were evident early and increased with disease progression, and vegetative symptoms were seen only in the later stages of PD. The different patterns of these depressive features with progression of PD may account in part for the variations seen in previous studies.

Adult

A brief scale for the dementia of Parkinson's disease.

The lack of standardized criteria and methodologies to assess dementia in patients with Parkinson's disease has resulted in inconsistencies and controversies in the literature regarding the incidence and basis of this condition. The authors examined a large group of patients with Parkinson's disease using two standardized sets of criteria for dementia. The neuropsychological data obtained was analyzed using stepwise discriminant analysis, and a brief scale was developed. The results indicated that a regression equation derived from just three neuropsychological procedures (which take only 10 minutes to administer) provides maximum discrimination between patients originally classified as demented or non-demented according to standardized criteria. Cut-off values for the product of the regression equation were calculated to accurately and reliably classify patients using the brief scale.

Adult

Magnetic resonance imaging and clinical correlates of intellectual impairment in myotonic dystrophy.

Although intellectual impairment is common in patients with myotonic dystrophy, this aspect of the disease has received relatively little research attention. We examined 41 patients with myotonic dystrophy using objective neuropsychological procedures and magnetic resonance imaging. Ten patients (24%) had severe and generalized intellectual dysfunction, while lesser or no cognitive impairment characterized the remaining patients. Degree of intellectual impairment was not related to neuromuscular status or sex. Patients with severe intellectual disturbance had significantly earlier onset of both myotonia and weakness and were more likely to inherit the disease from their mother. Magnetic resonance imaging findings indicated that while degree of cerebral atrophy was not related to severity of intellectual impairment, skull thickness, focal white matter lesions, and anterior temporal lobe abnormalities were significantly more common in patients with severely disturbed intellect. This study reports a number of previously unreported cerebral magnetic resonance imaging findings associated with intellectual impairment in myotonic dystrophy, but the etiology of these changes awaits neuropathologic examination.

Adolescent

Neuropsychological differences between the dementias of Alzheimer's and Parkinson's diseases.

The question of whether dementia of the Alzheimer type and dementia associated with Parkinson's disease are clinically separable is controversial. We examined possible neuropsychological differences in these two patient groups matched for overall severity of dementia. Patients with dementia of the Alzheimer type had more severe impairment on measurements of memory, language, and orientation, and, unlike patients with Parkinson's disease, there was evidence of apraxia. Patients with Parkinson's disease had more severe impairment related to speed of information processing, and, unlike patients with dementia of the Alzheimer type, they also had disturbance of mood. The nature of visuospatial abnormalities also differentiated the two groups. The pattern of neuropsychological differences is consistent with the cortical-subcortical hypothesis.

Affect

The picture absurdities test in the evaluation of dementia.

Recognition of picture absurdities was found to be significantly impaired in a group of patients with dementia of Alzheimer type compared to patients with dementia syndrome of depression and cerebrovascular dementia, even when matched for age, education, and dementia severity. None of the other neuropsychological measures investigated, including visual recent memory, Raven's progressive matrices, geographic orientation, and copying of geometric figures, resulted in a similar pattern. Although uncertainty remains as to what the picture absurdities test measures, normal performance likely depends on the integrity of many independently assessable variables, in addition to cognition and judgment utilizing visual data.

Aged

Neuropsychological similarities in lateralized parkinsonism.

Parkinson's disease (PD) patients who have left or right predominance of motor symptoms may exhibit cognitive differences. Previous research found greater neuropsychological impairment in patients with both right and left motor predominance, and some found no differences. Variability in overall severity of disease among the patients studied makes evaluation of these reports difficult. We examined the possibility that neuropsychological differences may occur in different stages of disease by comparing patients with mild unilateral disease (Exp. 1) and advanced disease (Exp. 2). Results indicated that while overall cognitive impairment increased with advancing disease, the pattern of neuropsychological impairments were not different with respect to laterality of motor symptoms in either experiment.

Cognition

Magnetic resonance imaging in dementia of Parkinson's disease.

The possibility that dementia in Parkinson's disease is associated with specific cerebral abnormalities identifiable by magnetic resonance imaging (MRI) was examined. Sixty eight patients with Parkinson's disease and 28 age and education matched normal controls were evaluated using neuropsychological procedures that included assessment of language, memory, cognition, visuospatial skills and mood. Twenty three patients (34%) were found to have developed significant impairment in at least three of the five areas, thus meeting criteria for a dementia syndrome. Eleven patients (16%) had no intellectual impairment and thirty four patients (50%) had a mild to moderate intellectual disturbance. Patients with (n = 10) and without dementia (n = 20), matched for severity of Parkinson's disease, and normal controls (n = 10) matched for age with the two patients groups, were evaluated by MRI. MRI scans were analysed for evidence of generalised cerebral atrophy, ventricular enlargement, visualisation of the substantia nigra and severity of focal brain lesions. Results indicated that the presence of dementia in patients with Parkinson's disease was not associated with any specific pattern of MRI abnormalities.

Adult

Influence of dopamine and disease severity on primitive reflexes in Parkinson's disease.

Several primitive reflexes are common in patients with Parkinson's disease (PD), but can also be noted in patients who have diffuse cerebral degeneration. The glabellar response is present in virtually all patients with PD and this reflex has been of theoretical interest in PD. It has been suggested that the glabellar response is related to inhibition via dopamine pathways. We examined changes in several primitive reflexes as a function of the plasma dopamine level and the severity of PD. The probability of observing these reflexes did not vary with dopamine levels, but the corneomandibular and palmomental reflexes were significantly more likely to be present in patients with advanced disease. Our findings confirm that primitive reflexes are prominent in PD and suggest that they may be secondary to extrapyramidal disease.

Age Factors

Neuropsychological impairments associated with severity of Parkinson's disease.

Intellectual impairment and disease severity tend to parallel one another in patients with Parkinson's disease (PD), but the pattern of development of the neuropsychological impairments contributing to the overall intellectual decline is unknown. This problem was addressed by comparing neuropsychological performance in the early and later stages of PD. Impairment of recent memory, impairment of cognition, and somatic features of depression were seen early and worsened with more advanced disease. Impairment of visuospatial skills, remote memory, language, and mood were observed only in the later stages of PD. These findings suggest that neuropsychological impairments do not develop in a uniform manner with progression of PD.

Aged

Dose-dependent memory impairment in Parkinson's disease.

Variation in plasma dopamine level between the time of original learning and subsequent memory retrieval causes a state-dependent memory impairment in Parkinson's disease. The occurrence of this phenomenon is not related to either progression of disease or duration of therapy, but is more likely to occur with high-dosage levels of levodopa-carbidopa.

Carbidopa

Tremor disorders of aging: diagnosis and management.

Tremor disorders are commonly encountered in the elderly. Physiological tremor is present in all of us and may be enhanced by drugs or other circumstances to cause symptomatic dysfunction. Essential tremor consists of postural and kinetic tremors which may involve the hands, head, and voice. Approximately 50% of cases are hereditary. Significant disability may occur. Propranolol and primidone provide effective treatment for some patients. The tremor of Parkinson's disease occurs in resting and postural positions. Treatment with levodopa usually reduces the tremor. Anticholinergics may also decrease tremor but often cause mental side effects in the elderly. Disturbances of the cerebellum may cause a kinetic tremor of the extremities or shakiness of the trunk. Tremors may also occur on a psychogenic basis. Proper classification of tremor disorder will lead to appropriate diagnosis and, often, effective treatment.

Aged

Magnetic resonance imaging and clinical correlations in multiple sclerosis.

We examined the relationship between magnetic resonance imaging (MRI) cerebral findings and clinical evaluations in 66 patients with clinically definite multiple sclerosis (MS). MRI observations included total number and location of lesions visualized, degree of periventricular involvement, degree of degeneration of the corpus callosum, and extent of generalized parenchymal atrophy. Overall physical disability was evaluated by the Kurtzke Expanded Disability Status Scale (EDSS) and individual symptoms were rated according to the Kurtzke Functional Systems (FS) scale. Our results suggest that MRI brain abnormalities are significantly related to the overall severity of disease, but MRI is not particularly useful to predict the presence or absence of individual symptoms. These findings do suggest that the MRI may provide useful information to monitor clinical progression of patients with MS, but the lesions visualized need not always be symptomatic nor are we sure that all symptomatic lesions, particularly in the spinal cord and optic nerves, will be visualized.

Adult

The naming disorder of dementia of Alzheimer type.

We studied the characteristics of the naming disorder of dementia of the Alzheimer type (DAT) in 20 patients, who were divided on the basis of Mini-Mental State scores into a mild and a moderate-severe group. Our results confirmed the presence of a progressive anomia which was sensitive both to word frequency and to image quality. Although DAT patients as a group made a similar proportion of perceptual-recognition and aphasic errors as did our age and education-matched controls, evidence was obtained that suggested that variations in the character of the anomia exist among individual DAT patients.

Aged