Core assessment program for intracerebral transplantations (CAPIT).
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Activities of Daily Living↗
Biomedical subjects
C G Goetz
Publications and source records attributed to C G Goetz.
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↗
Blinded evaluation confirms long-term asymmetric effect of unilateral thalamotomy or subthalamotomy on tremor in Parkinson's disease.
In the past, stereotactic surgery was a regular treatment for prominent unilateral tremor in Parkinson's disease (PD), but follow-up studies were usually short-term and always unblinded. We examined 17 PD patients in long-term follow-up (mean, 10.9 years after surgery) and used videotapes and the Unified Parkinson's Disease Rating Scale to blindly compare tremor ipsilateral and contralateral to the side of surgery. Since the patients were specifically selected for stereotactic surgery because of asymmetric tremor, and the surgical side chosen was contralateral to the predominant tremor, a sign of long-term efficacy would be current postoperative reversal of tremor side predominance. Upper extremity tremor was significantly better contralateral to the surgery compared with the ipsilateral side. We conclude that stereotactic surgery improved the absolute magnitude of tremor or ameliorated its rate of progression. Since asymmetric bradykinesia and dyskinesia were not a prerequisite for the choice of surgical side, we cannot make any conclusion about long-term impact of surgery on these features.
Aged↗
Dopaminergic agonists in the treatment of Parkinson's disease.
Dopaminergic agonists are drugs that directly stimulate the dopamine receptors of the striatum and have proved useful in treating Parkinson's disease. In the United States, they are primarily used in conjunction with levodopa. Most have long half-lives and have been particularly useful in controlling and attempting to prevent motor fluctuations.
Apomorphine↗
Clonidine and clonazepam in Tourette syndrome.
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Clonazepam↗
Trauma and movement disorders.
Movement disorders have been linked to trauma; however, these cases are uncommon and best substantiated if the movement disorder occurs in close temporal association with the trauma, occurs in context of other clinical signs of neurologic damage, and if evidence of CNS lesions on neuroimaging scans is evident. The pathophysiology of movement disorders relates to basal ganglia dysfunction, and traumatic lesions of peripheral nervous system structures or cortical regions could theoretically alter basal ganglia function indirectly and influence movement disorders. Parkinson's disease, the prototype of movement disorders, does not appear to be caused by trauma.
Brain↗
Visual art in the neurologic career of Jean-Martin Charcot.
Jean-Martin Charcot, the world's first chaired professor of neurology, incorporated visual art into his daily practice of neurology. Art served as scientific documentation and was a pivotal tool in the development and dissemination of Charcot's clinicoanatomic method. Although Charcot drew extensively in clinical and laboratory studies, very few of these visual documents have ever been published or are currently available for public study. Charcot was central to the incorporation of medical photographs into the study of neurologic disease and relied heavily on visual material in his capacity as an international teacher. Art also misguided Charcot's career when he relied heavily on artwork in his attempt to convince critics that disorders seen at the Salpêtrière Hospital, Paris, France, were independent of his suggestive influence.
History, 19th Century↗
Effects of head trauma from motor vehicle accidents on Parkinson's disease.
During 1 year we monitored changes in clinical disability scores in 10 patients with Parkinson's disease (PD) who sustained head trauma from motor vehicle accidents. Disability significantly increased immediately after trauma, but returned to baseline levels in subsequent weeks. These patients' signs deteriorated over the subsequent year, but this decline was equivalent to that of a matched control group with PD who did not have a motor vehicle accident or trauma. Based on these data, we conclude that trauma or stress caused by motor vehicle accidents can transiently exacerbate parkinsonian dysfunction without causing persistent increased disability or an alteration in disease course.
Accidents, Traffic↗
Putative chromaffin cell survival and enhanced host-derived TH-fiber innervation following a functional adrenal medulla autograft for Parkinson's disease.
We report heretofore undescribed clinical and histological features of a patient with idiopathic Parkinson's disease who showed marked and persistent motoric benefit from an adrenal medulla autograft for 18 months following grafting. The patient returned to the preoperative level of disability prior to his death 30 months after implantation, the longest survival to date for an adrenal transplant. The graft site was primarily necrotic and large numbers of macrophages were still present at the time of death. A few tyrosine hydroxylase-immunoreactive cells were found within the graft, providing the first evidence that adrenal medullary cells may be viable and produce catecholamines following human transplantation. A dense network of tyrosine hydroxylase-immunoreactive terminals and processes was also seen ventral to the implant site and on both of its lateral borders. This response may represent sprouting by residual host dopaminergic neurons mediated by the vigorous response to injury displayed by the host striatum. The relative paucity of surviving chromaffin cells, however, provides further evidence that adrenal medulla grafts survive poorly following intracerebral implantation, and factors other than dopamine replacement may be responsible for most of the functional effects observed following chromaffin cell transplantation.
Adrenal Medulla↗
Levodopa reduces the growth promoting effects of striatal extracts on rostral mesencephalic tegmentum cultures.
Rats with unilateral 6-hydroxydopamine lesions (6-OHDA) of the mesencephalon and vehicle controls (SHAM) were chronically treated with carbidopa (CD) or CD plus levodopa (CD/LD) for 18 days. Seventy-two hours following the last treatment, ipsilateral striata, contralateral striata, and cerebellums from each treatment group were homogenized separately and the supernatant extracts were incubated with rostral mesencephalic tegmentum cultures. As indices of growth-promoting activity (GPA), number of viable neurons and their process lengths were measured 40 h later. In all cultures exposed to striatal extracts, the 6-OHDA lesion was associated with greater GPA than the SHAM extracts. CD/LD consumption reduced this GPA in a dose-dependent fashion in both the lesioned and the SHAM animals. These data suggest that denervation of the striatum enhances the production of a striatally derived neurotrophic factor, the production of which is sensitive to levodopa. Chronic levodopa treatment in Parkinson's disease may therefore contribute to disease progression by reducing the compensating effects of this neurotrophic factor on remaining mesencephalic neurons.
Animals↗
Clinical improvement in parkinsonian patients undergoing adrenal to caudate transplantation is not reflected by chromogranin A or basic fibroblast growth factor in ventricular fluid.
Fifteen patients with Parkinson's disease underwent open transplantation of autologous adrenal medulla to the caudate nucleus. Motor function was evaluated before and after surgery and was found to be significantly improved at 5-9 months following surgery. Cerebrospinal fluid was taken from the ventricle adjacent to the implant site at the beginning of the operation and at 1 week, 3 months, and 5-9 months following surgery. The cerebrospinal fluid was assayed for chromogranin A (CgA), the major soluble protein in chromaffin granules, and basic fibroblast growth factor (bFGF), a neurotrophic growth factor found in normal brain and adrenal medulla. CgA levels did not increase following surgery, suggesting that a significant number of chromaffin cells did not survive or that surviving chromaffin cells did not secrete a significant amount of CgA. Basic fibroblast growth factor was undetectable in the ventricular cerebrospinal fluid.
Adrenal Medulla↗
Effect of MK-458 (HPMC) in Parkinson's disease previously untreated with dopaminergic drugs. A double-blind, placebo-controlled multicenter study.
Ninety-four patients with early Parkinson's disease were investigated in a double-blind, placebo-controlled evaluation of MK-458 [hydroxypropyl methylcellulose/lactose matrix (HPMC)], a sustained release formulation of a novel naphthoxazine compound with selective D-2 dopamine receptor agonism. Patients were previously untreated with dopaminergic drugs. Efficacy was assessed by clinical rating scales and by patient self-evaluation. MK-458 (HPMC) caused a significant decrease in most parkinsonian symptoms. Though disability rating scores were lowered by the drug, the scores did not differ significantly from placebo. However, statistically significant improvement occurred with MK-458 (HPMC) on both the physician and the patient global assessments. Adverse reactions such as nausea and vomiting, sedation, confusion, and hallucinations occurred more with MK-458 (HPMC) than with placebo. MK-458 (HPMC) possesses antiparkinsonian efficacy in early Parkinson's disease; however, side-effects are frequently associated with its use. Selective D-2 receptor agonists, such as MK-458 (HPMC), may not be the ideal treatment as monotherapy for Parkinson's disease.
Aged↗
United Parkinson Foundation Neurotransplantation Registry on adrenal medullary transplants: presurgical, and 1- and 2-year follow-up.
Thirteen centers participated in a multicenter database with systematic evaluation of US and Canadian patients who had adrenal medullary transplantation for Parkinson's disease. This voluntary registry collected demographic, safety, and efficacy data using the same scoring measures over a 2-year follow-up period. Baseline data on 61 patients and 2-year follow-up data on 56 patients were compared. Eighteen percent died during the study period, and one-half of these deaths were related or questionably related to the surgery. Of the remaining 45 patients with data, global improvement, defined as an improved summed score of the "on" and "off" motor and activities of daily living functions from the Unified Parkinson's Disease Rating Scale, occurred in 32% of the patients at 2 years after surgery. At follow-up, significant group improvement persisted in the amount of daily "on" time and the quality of "off" function, but other measures were no better than baseline. When the global improvement calculation was based on the total sample and included deaths and patients lost to follow-up as "not improved," only 19% were improved 2 years after surgery. Twenty-two percent of survivors had persistent psychiatric morbidity not present prior to surgery. These data document a modest group improvement in "off" function after neurotransplantation, but a serious level of mortality and morbidity.
Adrenal Medulla↗
The potential use of a dopamine neuron antibody and a striatal-derived neurotrophic factor as diagnostic markers in Parkinson's disease.
The cerebral spinal fluid (CSF) of patients with Parkinson's disease (PD) contains an antibody that immunocytochemically reacts with dopamine (DA) neurons in the substantia nigra (SN). This antibody was found in 78% of the CSF samples taken from patients with clinical PD. In contrast, only 3% of the CSF samples taken from control patients or patients with neurologic symptoms other than PD possessed this antibody. The production of this antibody might contribute to disease progression but does not appear to be the etiologic factor responsible for PD. In other experiments, concentrates of the CSF of patients with PD enhanced growth of mesencephalic cultures relative to control CSF. Both the antibody and the growth-promoting activity found in CSF are associated with degeneration of the SN and might therefore be useful as potential diagnostic markers for PD.
Adrenal Medulla↗
New approaches in the treatment of the dystonias.
At this point the treatment of dystonias remains highly empirical. Secondary dystonias, especially those related to specific drug treatment, have to be ruled out carefully. A few dystonic subgroups respond well to levodopa medication. In the other syndromes, anticholinergics are the usual first choice. In focal conditions botulinum toxin injections seem to be the most effective regimen, although there are only a few long-term studies. Surgical procedures are an ultimate option.
Botulinum Toxins↗
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↗