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Biomedical subjects

C M Tanner

Publications and source records attributed to C M Tanner.

At least 19 recordsLinked to original sources

Ideational fluency in Parkinson's disease.

Recent research on Parkinson's disease (PD) suggests that executive functions are impaired but that visuospatial functions may be spared. This dissociation has been attributed to dysfunction in ascending dopaminergic pathways affecting the prefrontal cortex. We investigated these ideas in a sample of intellectually intact patients with idiopathic, optimally treated PD (N = 20) and in spouse controls (N = 15); the groups were divided into young (age < 60) and old subgroups, each comparable on education, vocabulary level, and Mini-Mental State scores. Six tasks were selected from a set of factor-referenced cognitive tests to measure three abilities: (1) ideational fluency (ability to generate ideas), (2) flexibility of use (ability to shift mental set), and (3) spatial orientation (ability to perceive spatial patterns). PD patients were impaired only on the ideational fluency factor (p = .01). An age-related deficit was seen on the spatial factor (p < .05) with a trend on the flexibility factor (.05 < p < .10). No interactions were significant. The findings suggest that when age and verbal intelligence are controlled, PD patients show no deficit on purely spatial tasks; in contrast, patients seem less able to generate ideas though capable of shifting from one idea to another.

Brain Mapping

Adult tics in Gilles de la Tourette's syndrome: description and risk factors.

We studied 58 adults with Gilles de la Tourette's syndrome diagnosed during childhood. Tics persisted in all patients but were moderate/severe in only 24%, compared with 60% at the time of worst function. Coprolalia persisted in 4%, compared with 22% at the time of worst function. For most patients, worst function occurred in adolescence (mode = 13 years). In spite of a high frequency of school and behavioral problems during development, 98% graduated high school and 90% were full-time students or fully employed. Features predictive of mild tic severity in adulthood were mild tics during the patient's worst pre-adulthood function and mild tics during early and late adolescence. Childhood tic severity had no predictive value, and likewise, coprolalia during development did not increase the risk for adult moderate/severe tics.

Adult

Botulinum toxin injection for spasmodic torticollis: increased magnitude of benefit with electromyographic assistance.

To determine the usefulness of EMG-assisted botulinum toxin (BOTOX) injections for the treatment of spasmodic torticollis (ST), we randomized 52 ST patients into two groups and studied them prospectively. In one group [(E+C)RX, N = 28], the muscles were selected for BOTOX injection using both clinical and EMG examination and then injected with EMG assistance. In the second group [(C)RX, N = 24] the muscles were selected for BOTOX injection based solely on clinical examination and injected without EMG assistance. The percentage of patients showing any improvement after BOTOX as similar in both the (E+C)RX and (C)RX groups. A significantly greater magnitude of improvement was present in the (E+C)RX group, as well as a significantly greater number of patients with marked improvement. In particular, patients with retrocollis, head tilt, and shoulder elevation demonstrated additional benefit with EMG-assisted BOTOX injection. EMG assistance may be effective because the technique increases the ability to effectively identify and treat the deep cervical muscles.

Adult

Dysphagia after botulinum toxin injections for spasmodic torticollis: clinical and radiologic findings.

We prospectively evaluated the frequency, severity, and radiologic features of swallowing abnormalities following Botox treatment of spasmodic torticollis. We performed both clinical and radiologic evaluations of swallowing before and following Botox in 18 consecutive cervical dystonia patients receiving their first Botox treatment. Before Botox, 11% of the patients had clinical symptoms of dysphagia and 22% had radiologic signs of a peristaltic abnormality. After Botox, the signs and symptoms of dysphagia in these patients did not change, but an additional 33% developed new dysphagic symptoms and 50% of the patients developed new peristaltic abnormalities by radiologic studies. Complaints of swallowing difficulty were always associated with abnormal radiologic findings. Neither the total Botox dose nor Botox into particular muscles differed between those with dysphagia and those without.

Adult

Epidemiology of Parkinson's disease.

Additional epidemiologic studies may provide important insights into the etiology of Parkinson's disease. Moreover as the elderly population of Europe and the United States grows, accurate public health planning requires accurate incidence and prevalence estimates. The recent development of a therapy that may slow disease progression (see article by Tetrud elsewhere in this issue) makes early identification and treatment of Parkinson's disease particularly important. Investigations of early markers of Parkinson's disease or markers of disease susceptibility are critical areas of future research, requiring careful collaboration between epidemiologists and laboratory scientists.

Epidemiologic Methods

Abnormal liver enzyme-mediated metabolism in Parkinson's disease: a second look.

If toxicant exposure contributes to the cause of Parkinson's disease, poor function of detoxifying enzymes could increase vulnerability for Parkinson's disease. Although no hepatic enzyme system has been shown universally to be dysfunctional in Parkinson's disease patients, several have been suggested to be dysfunctional in subgroups, such as those with young age at disease onset. Specific enzymes implicated include several P450 enzymes, most notably P450 IID6, and cysteine dioxygenase. If hepatic enzyme abnormalities contribute to the development of Parkinson's disease, molecular genetic techniques may allow the development of screening tests to identify at-risk subjects in order to intervene with protective therapies.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine

Liver enzyme abnormalities in Parkinson's disease.

If toxicant exposure contributes to the cause of Parkinson's disease, poor function of detoxifying enzymes could increase vulnerability for Parkinson's disease. Although no hepatic enzyme system has been shown universally to be dysfunctional in Parkinson's disease patients, several have been suggested to be dysfunctional in subgroups, such as those with young age at disease onset. Specific enzymes implicated include several P450 enzymes, most notably P450 IID6, and cysteine dioxygenase. If hepatic enzyme abnormalities contribute to the development of Parkinson's disease, molecular genetic techniques may allow the development of screening tests to identify at-risk subjects in order to intervene with protective therapies.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine

Autologous transplantation of adrenal medulla in Parkinson's disease. 18-month results.

Eighteen of 19 patients who underwent autologous adrenal medullary transplantation to the right caudate nucleus have been followed up for 18 months. During the course of this study, a statistically significant improvement was noted in percent "on" time, percent "on" time without dyskinesia, activity of daily living (ADL) scores during the "on" stages, and ADL, motor, and Schwab-England scores during the "off" stages. Benefits tended to be maximal at 6 months and to gradually lessen thereafter, although statistically significant improvement in comparison with baseline was still present at 18 months for ADL, motor, and Hoehn-Yahr scores during the "off" stages. Almost all parameters had deteriorated by 18 months compared with 12 months, including those remaining significantly improved in comparison with baseline. These patterns were similar for each of the three participating centers. Complications were largely restricted to the perioperative period.

Adrenal Medulla

Gilles de la Tourette's syndrome in twins: clinical and neurochemical data.

A pair of identical twins (probability of monozygosity by genetic marking = 99.99%) with Gilles de la Tourette's syndrome (GTS) were studied clinically and neurochemically. The two boys were distinguished within the family by dramatic differences in their behavior and tics. Twin 1 had severe motor tics, loud vocalizations, coprolalia, and school behavior problems, whereas twin 2 was only mildly affected. Neither boy had ever been treated with medication for his tic disorder. Lumbar puncture was performed on each after a night of bed rest. In spite of the marked clinical differences between the two boys, the major indices of dopamine and serotonin turnover in the cerebrospinal fluid were equivalent. Homovanillic acid levels were 65 and 60 ng/ml, respectively, and 5-hydroxyindoleacetic acid levels were 36 and 39 ng/ml. Observation of these twins suggests that the phenotypic expression of GTS relates to more factors than inheritance. Neurochemical metabolites of dopamine and serotonin did not reflect the comparative clinical severity of GTS in these boys.

Child

The rabbit syndrome.

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Antipsychotic Agents

Adrenal medullary transplant to the striatum of patients with advanced Parkinson's disease: 1-year motor and psychomotor data.

We studied motor and psychomotor changes over 1 year after surgery in 7 patients with severe idiopathic Parkinson's disease (PD) who underwent intrastriatal autologous adrenal medulla transplant. Significant clinical improvements were present 1 year after surgery and primarily involved increased quantity of "on" time and increased quality of "off" time: "on" time increased from a mean 60.7% of the waking day to 82.7%, and "off" function improved. In contrast, although "on" function also improved, statistically significant improvement occurred in only 1 measure, the Unified Parkinson's Disease Rating Scale activities of daily living subscale. Medications did not change, and motor fluctuations persisted. Improvement began several weeks after surgery, was maximal at 4 to 6 months, and was sustained thereafter. There was significant group improvement in quality of life measures of sleep and rest, social isolation, and ambulation. One patient had severe, recurrent depression postoperatively. The efficacy of adrenal transplant surgery is not transient, and specific functional improvements can be prolonged.

Activities of Daily Living

Multicenter study of autologous adrenal medullary transplantation to the corpus striatum in patients with advanced Parkinson's disease.

In 19 patients with severe Parkinson's disease, we replicated the surgical procedures developed by Madrazo et al. for transplantation of the adrenal medulla to the striatum, and followed them for six months after operation. We monitored their motor function with the use of standardized scales and determined the amount and quality of "on" and "off" time (the hours of the waking day when the antiparkinsonian medications were effective and ineffective, respectively). We found significant improvement in focal areas of motor function. The mean percentage of on time during the day increased from 47.6 percent to 75.0 percent (P = 0.012); the mean percentage of on time without chorea increased from 26.6 percent to 59.2 percent (P = 0.006); the mean severity of off time decreased as assessed by both the Activities of Daily Living subscale of the Unified Parkinson's Disease Scale (P = 0.002) and the Schwab and England scale (P = 0.037). In contrast to the finding of Madrazo et al., however, the dosages of antiparkinsonian medications could not be decreased and postoperative morbidity was substantial. Despite cautious optimism, we conclude that the widespread use of this procedure outside of research centers is premature, since the improvement we found was slighter than in the previous cases.

Activities of Daily Living

The role of environmental toxins in the etiology of Parkinson's disease.

The production by the pyridine MPTP of a parkinsonian syndrome strikingly similar to the 'idiopathic' disorder, and the paucity of evidence supporting a hereditary or infectious etiology for Parkinson's disease (PD), have stimulated a search for environmental chemicals resembling MPTP that might cause PD. In support of this, descriptive epidemiological studies have found higher prevalences of PD in highly industrialized countries. In North America and Europe, early onset PD appears to be associated with rural residence. Factors associated with this include vegetable farming, well water drinking, wood pulp, paper and steel industries. In China, living in industrialized urban areas increases the risk of developing PD. Preliminary epidemiological evidence supports the hypothesis that environmental chemicals may be related to the development of PD, but specific chemicals and their specific mechanism(s) have not been identified.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine

Hypersexuality with antiparkinsonian therapy.

Thirteen parkinsonian patients drawn from two large parkinsonism clinics experienced hypersexuality as a consequence of anti-parkinsonian therapy. The cases include only those whose sexual behavior on treatment became a concern to the patient's family or a social agency. The majority of patients were men and had a relatively early onset of parkinsonian symptomatology. There was no relation between functional improvement and increased sexuality. Most patients showed some element of dose dependency between antiparkinsonian drugs and the hypersexual behavior. Prior sexual profile included from no sexual outlet to hypersexuality. Neither the prior history of psychiatric illness nor brain damage predisposed to such response on treatment, and in most patients, it was not a part of hypomania or a more diffuse psychiatric disturbance. We propose that hypersexuality on antiparkinsonian drugs is consequent to inhibition of prolactin secretion.

Adult

Environmental factors and Parkinson's disease: a case-control study in China.

We studied the role of environment in the development of Parkinson's disease (PD) in China, where industrialization is relatively recent and the population geographically stable. Using a case-control method, we investigated the relationship between PD and exposure to the following factors before disease onset: place of residence, source of drinking water, environmental and occupational exposure to various agricultural and industrial processes. Occupational or residential exposure to industrial chemicals, printing plants, or quarries was associated with an increased risk of developing PD. In contrast, living in villages and exposure to the common accompaniments of village life, wheat growing and pig raising, were associated with a decreased risk for PD. PD cases and controls did not differ with respect to other factors investigated. These findings are consistent with the hypothesis that environmental exposure to certain industrial chemicals may be related to the development of PD.

Adult