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Biomedical subjects

C G Goetz

Publications and source records attributed to C G Goetz.

At least 91 records · Page 5Linked to original sources
PubMed1999

Movement disorders in Kuru.

OBJECTIVE: To describe the gamut of movement disorders (MD) seen during the clinical course of kuru. BACKGROUND: Kuru is a subacute spongiform encephalopathy that was confined to several adjacent cultures in the Eastern Highlands of New Guinea and resulted from contamination with brain tissue during the ritual endocannibalism practiced in those societies. This unique neurologic disease was recorded extensively with film between 1957 and 1976, and these comprehensive research documents have been donated to the American Academy of Neurology archives by one of the authors (DCG). METHODS: The comprehensive assembly of film record of kuru, which was collected by one of the authors (DCG) was reviewed. This comprised two parts: The first were films from 1957-1964 and included 17.397 ft of 16-mm film featuring 204 patients (children and adults); the second is assembled from films made from 1967-1976 and includes 9138 ft. of film featuring 47 adult patients. Two MD specialists categorized all MDs observed and a representative videotape was produced. RESULTS: Tremor is the most frequently encountered MD in kuru and is typically of the action/intention type, which appears early in the disease and is soon associated with other clinical signs of cerebellar dysfunction. Widespread clonus is characteristic of advanced disease and can be difficult to differentiate from tremor. Dystonia/athetosis and choreiform jerks also appear as the disease progresses. Dystonia can involve the torso, distal limbs, neck, or jaw. Myoclonic jerks can be superimposed on the cerebellar or dystonic features usually with an enhanced startle response. Parkinsonian symptomatology, other than resting tremor is frequent among the filmed subjects especially in the second stage of the disease. CONCLUSION: The clinical manifestations of kuru involved a wide array of MDs during all three stages of the degenerative illness.

Adult↗
PubMed1999

Delayed recognition memory in Parkinson's disease: a role for working memory?

Immediate and delayed recognition memory for words was examined in a sample of 16 non-demented patients with Parkinson's disease and 16 normal control participants of equivalent age and educational attainment. The patients, relative to control participants, had intact immediate but impaired delayed recognition memory performance. Patients were also impaired on tests of free and cued recall, working memory and a measure of psychomotor processing speed. Processing speed was a significant covariate for delayed recognition, free and cued recall and working memory performance, but not for immediate recognition performance. These results suggest that the same cognitive processes which support performance on tests of recall and working memory also support performance on tests of delayed recognition.

Aged↗
PubMed1998

5-Hydroxytryptophan-induced myoclonus in guinea pigs: mediation through 5-HT1/2 receptor subtypes.

In guinea pigs, myoclonus can be induced by 5-hydroxytryptamine (5-HT, serotonin) precursors and synthetic 5-HT receptor agonists, yet the receptor subtype specificity of this behavior is not fully delineated. Guinea pigs were pre-treated with carbidopa (50 mg) followed by one of eight 5-HT antagonists: (-)-N-tert-butyl-3-[4-(2-methoxyphenyl) piperazin-1-yl]-2-phenyl propionamide ((-)-WAY 100135) (5-HT1A), N-[2-[4-(2-methoxyphenyl)-1-piperazinyl]-ethyl]-N-(2-pyridyl)-cy clohexancarboxamide (WAY 100635) (5-HT1A), methiothepin mesylate (5-HT1/2), mesulergine hydrochloride (5-HT2A/2C), N[4-methoxy-3-(4-methyl-L-piperazinyl)phenyl]-2'-methyl-4'-(5-methyl-1,2 ,4-oxadizol-3-yl) (GR 127935) (5-HT1D), trans-4-[(3Z)3-(2-dimethylaminoethyl)oxyimino-3(2-fluorop hen yl) propen-1-yl]phenol, hemifumarate (SR 46349) (5-HT2), ondansetron hydrochloride (5-HT3), and [1-[2-[methylsulphonyl)amino]ethyl]-4-piperidinyl]methyl-5-fluoro-2-meth oxy-1H-indole-3-carboxylate (GR 125487) (5-HT4). Thirty minutes later, they received 5-hydroxytryptophan (5-HTP) (75 mg/kg, sc) and myoclonic jumping rates were assessed every 10 min for 200 min by a blinded observer. Repeated measures analysis of variance of drug-induced antagonism of 5-HTP-induced myoclonus revealed a significant effect for the 5-HT receptor antagonists methiothepin mesylate, GR127935, and mesulergine hydrochloride compared to placebo, and each of these drugs inhibited 5-HTP-induced myoclonus in a dose-dependent fashion. Based on the receptor profiles of the three effective antagonists, 5-HTP-induced myoclonus is influenced by the 5-HT1/2 receptor systems. The absence of a significant change with any other receptor subtype antagonist suggests that myoclonus is not related to diffuse activation of central serotonergic mechanisms.

Animals↗
PubMed1998

Clinical presentation and pharmacological therapy in corticobasal degeneration.

BACKGROUND: To date, to our knowledge, there is no systematic presentation of treatment outcome in large series of patients clinically diagnosed as having corticobasal degeneration. OBJECTIVE: To evaluate the clinical presentation and treatment outcome of patients clinically diagnosed as having corticobasal degeneration. SUBJECTS: We gathered case patients seen in 8 major movement disorder clinics during the last 5 years who were diagnosed as having corticobasal ganglionic degeneration. METHODS: Using a chart review method, we recorded the clinical presentation, medications used, response to medications, and adverse effects. RESULTS: A total of 147 case patients were reviewed, 7 were autopsy proven. Parkinsonian features were present in all, other movement disorders in 89%, and higher cortical dysfunction in 93%. The most common parkinsonian sign was rigidity (92%), followed by bradykinesia (80%), gait disorder (80%), and tremor (55%). Other movement disorders were dystonia in 71% and myoclonus in 55%. Higher cortical dysfunction included dyspraxia (82%), alien limb (42%), cortical sensory loss (33%), and dementia (25%). Ninety-two percent of the case patients received dopaminergic drugs, which resulted in a beneficial effect for 24%. Parkinsonian signs were the elements improving the most and levodopa was the most effective drug. Benzodiazepines, primarily clonazepam, were administered to 47 case patients, which resulted in improvement of myoclonus in 23% and dystonia in 9%. The most frequent disabling adverse effects of drug trials in these case patients were somnolence (n = 24), gastrointestinal complaints (n = 23), confusion (n = 16), dizziness (n =12), hallucinations (n = 5), and dry mouth (n = 5). CONCLUSIONS: Pharmacological intervention was largely ineffective in the management of corticobasal degeneration, and new treatments are needed for ameliorating the symptoms of this syndrome.

Antiparkinson Agents↗
PubMed1998

Accuracy of the clinical diagnoses of Lewy body disease, Parkinson disease, and dementia with Lewy bodies: a clinicopathologic study.

BACKGROUND: Whether Parkinson disease (PD) and dementia with Lewy bodies (DLB) represent 2 distinct nosologic entities or are diverse phenotypes of Lewy body disease is subject to debate. OBJECTIVES: To determine the accuracy of the diagnoses of Lewy body disease, PD, and DLB by validating the clinical diagnoses of 6 neurologists with the neuropathologic findings and to identify early predictors of the diagnoses. METHODS: Six raters who were unaware of the neuropathologic diagnoses analyzed 105 clinical vignettes corresponding to 29 cases of Lewy body disease (post hoc analysis of 15 patients with PD and 14 with DLB) and 76 patients without PD or DLB whose cases were confirmed through autopsy findings. MAIN OUTCOME MEASURES: Sensitivity and positive predictive value (PPV) were chosen as validity measures and the K statistic as a reliability measure. RESULTS: Interrater reliability for the diagnoses of Lewy body disease and PD was moderate for the first visit and substantial for the last, whereas agreement for diagnosis of DLB was fair for the first visit and slight for the last. Median sensitivity for diagnosis of Lewy body disease was 56.9% for the first visit and 67.2% for the last; median PPV was 60.0% and 77.4%, respectively. Median sensitivity for the diagnosis of PD was 73.3% for the first visit and 80.0% for the last; median PPV was 45.9% and 64.1%, respectively. Median sensitivity for the diagnosis of DLB was 17.8% for the first visit and 28.6% for the last; median PPV was 75.0% for the first visit and 55.8% for the last. The raters' results were similar to those of the primary neurologists. Several features differentiated PD from DLB, predicted each disorder, and could be used as clinical pointers. CONCLUSIONS: The low PPV with relatively high sensitivity for the diagnosis of PD suggests overdiagnosis. Conversely, the extremely low sensitivity for the diagnosis of DLB suggests underdiagnosis. Although the case mix included in the study may not reflect the frequency of these disorders in practice, limiting the clinical applicability of the validity measures, the raters' results were similar to those of the primary neurologists who were not exposed to such limitations. Overall, our study confirms features suggested to predict these disorders, except for the early presence of postural imbalance, which is not indicative of either disorder.

Adult↗
PubMed1998

Pharmacological therapy in progressive supranuclear palsy.

BACKGROUND: To our knowledge, previous reports on drug treatment in progressive supranuclear palsy have not evaluated autopsy-confirmed cases. OBJECTIVE: To evaluate pharmacological treatment responses from detailed clinical records in patients with autopsy-confirmed progressive supranuclear palsy. SUBJECTS AND METHODS: We reviewed medical records for clinical presentation and pharmacological response in 12 patients with autopsy-confirmed progressive supranuclear palsy diagnosed using the National Institute of Neurological Disorders and Stroke pathologic criteria. For each drug class, exposure, global positive response, and specific positive response (parkinsonism, other movement disorders, or gaze dysfunction) were recorded. RESULTS: Drug classes examined were dopaminergics (all patients), tricyclics (3 patients), methysergide maleate (3 patients), 5-hydroxytryptophan (2 patients), and anticholinergics and selective serotonin inhibitors (1 patient). Positive clinical response was detected in 7 of the patients receiving dopaminergic drugs and in 1 patient each receiving tricyclics, methysergide, and 5-hydroxytryptophan, respectively. None of the patients responded markedly however, and there was no persistent beneficial effect. Use of dopaminergic drugs most frequently improved parkinsonian features, but disabling adverse effects included orthostatic hypotension (6 patients), hallucinations and delusions (3 patients), gastrointestinal complaints (3 patients), and dizziness (1 patient). Only 1 patient developed dyskinesia. CONCLUSION: Use of antiparkinsonian medications and other neurotransmitter replacement therapies was largely ineffective and caused frequent adverse effects in this series of patients with autopsy-confirmed with progressive supranuclear palsy.

5-Hydroxytryptophan↗
PubMed1998

Hyperkinetic movement disorders misdiagnosed as tics in Gilles de la Tourette syndrome.

OBJECTIVE: To describe the gamut of movements misdiagnosed as tic exacerbations in Gilles de la Tourette syndrome (GTS) in a referral tertiary-care center. BACKGROUND: Movements seen in GTS can be classified as: (a) tics; (b) movements related to conditions associated with GTS, specifically obsessive-compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), and antisocial behaviors; and (c) movements secondary to treatment. METHODS: We reviewed a videotape database and patient records from a tertiary treatment center for GTS and collected GTS cases referred for disease exacerbation who had both tics and non-tic movements thought by the referring physician, the patient, and the family to be an exacerbation of tics. RESULTS: Of 373 GTS cases, 12 had movement disorders secondary to treatment, and six had non-tic movements related to conditions commonly associated with GTS. In the former group, there were 7 patients with acute akathisia, 3 with acute dystonia, 1 with tardive chorea, 1 with withdrawal emergent chorea, and 5 with tardive dystonia. Six had movement disorders related to non-tic conditions commonly associated with GTS: four patients had movements associated with OCD, one with ADHD and antisocial behavior, respectively. CONCLUSION: There is a broad spectrum of movements in GTS that are not tics but can be misdiagnosed as tics. Clinical awareness of these movements is paramount to proper diagnosis and pharmacologic intervention.

Adolescent↗
PubMed1998

The effect of changes in head posture on the patterns of muscle activity in cervical dystonia (CD).

Twelve patients with cervical dystonia (CD) and predominant rotation were studied to determine the effects of changes in head posture on the specific patterns of cervical muscle activity. Turns analysis was used to quantify muscle activity underlying head rotation, recorded simultaneously from the agonist and antagonist muscle pairs bilaterally (sternocleidomastoid [SCM] and splenius [SPL]). Muscle activity was compared between the uncompensated dystonic posture and during the maintenance of midposition. In addition, patients were separated into two groups (geste = 6; no geste = 6) based on whether they had a clinically efficacious geste to determine the effect of geste on patterns of cervical muscle activity. Muscle activity was measured during the maintenance of midposition with and without a clinical or simulated geste. Differences in muscle activity between the groups and postures were compared using repeated measure analysis of variance (ANOVA) analyses. The four muscles tested showed a significant difference in muscle activity in the uncompensated dystonic posture as a result of the increased activity in the agonist muscle pair (SCM and SPL responsible for the dystonic posture) (EMG amplitude: F[1,11] = 18.81, p = 0.0012; EMG frequency: F[1,11] = 32.07, p = 0.0001). Maintaining the head in the midposition was associated with a significant reduction in muscle activity compared with the uncompensated dystonic posture (EMG amplitude: F[1,9] = 6.36, p < 0.033; EMG frequency: F[1,9] = 10.96, p < 0.0091). This reduction in midposition muscle activity was significantly greater in the agonist muscle pair (EMG amplitude: F[1,10] = 19.70, p = 0.0013; EMG frequency: F[1,10] = 44.67, p < 0.0001). In the patients with clinically effective geste, there was no additional reduction in muscle activity observed in the midposition when they performed their geste (EMG amplitude: F[1,9] = 4.63, p = 0.060; EMG frequency: F[1,9] = 1.22, p = 0.298). These findings suggest that CD with rotation is characterized by predominantly increased agonist muscle activation. Patients with CD retain the ability to modulate this involuntary agonist muscle activity to maintain the head in the midposition. The maintenance of the midposition does not seem to be facilitated by geste.

Adult↗
PubMed1998

Factor structure of the Unified Parkinson's Disease Rating Scale: Motor Examination section.

The Unified Parkinson's Disease Rating Scale (UPDRS) is widely used to assess Parkinson's disease (PD) disability but its metric properties have not been extensively studied. We investigated the factor structure and internal consistency of the Motor Examination section of the UPDRS in a sample of 294 consecutive patients with idiopathic PD who were assessed while in the "on" state. There was a high degree of internal consistency. Factor analysis revealed six clinically distinct factors, three bradykinesia measures (axial/gait, right and left), one rigidity measure, and two tremor measures (rest and postural). These factors accounted for 78% of the variance. Total Motor Examination scores and selected factors correlated well with other examiner-determined global ratings of PD disability (Hoehn and Yahr stage and Schwab-England Activities of Daily Living score). These results suggest that the Motor Examination section of the UPDRS provides a useful measure of PD function as well as severity measures of six clinical disability domains.

Activities of Daily Living↗
PubMed1998

Retrospective application of a set of clinical diagnostic criteria for the diagnosis of multiple system atrophy.

We estimated the accuracy of a modified commonly used set of clinical diagnostic criteria for the diagnosis of multiple system atrophy (MSA) by retrospectively applying the criteria to the features recorded by six neurologists who had evaluated 105 autopsy-confirmed cases (16 MSA and 89 non-MSA disorders). Cases were abstracted from the records of the patients' first visit to an academic center, and were presented as clinical vignettes to six neurologists, each of whom recorded the main clinical features of the presented clinical vignette on a standardized form. Sensitivity and positive predictive values were chosen as validity outcome measures and were calculated by comparing the applied diagnostic criteria to the neuropathologic information. Of note, most MSA patients in this study (mainly those with Shy-Drager type) had not received levodopa therapy since the primary neurologists often had not perceived a need to administer this treatment. The validity of the retrospectively applied criteria for the diagnosis of possible MSA (sensitivity: median, 53%, range, 50-69%; positive predictive value: 30%, 28-39%) and probable MSA (sensitivity: 44%, 31-60%; positive predictive value: 68%, 54-80%) at the first visit was suboptimal. The best, still not perfect, accuracy for this set of diagnostic criteria was obtained when six out of eight features (sporadic adult onset, dysautonomia, parkinsonism, pyramidal signs, cerebellar signs, no levodopa response, no cognitive dysfunction, or no downward gaze supranuclear palsy) were present (median sensitivity, 59%; range, 50-75%; positive predictive value: 67%, 53-83%). This is the first study to validate criteria for the clinical diagnosis of MSA. Our data suggest that it is difficult to achieve an early and accurate clinical diagnosis of this disorder. The probability of correctly diagnosing MSA increases when at least six features of this modified set of criteria are present or when requiring the set for probable MSA.

Adult↗
PubMed1998

Neuropharmacology in the elderly.

Psychotropic medications present special problems in the elderly because of altered pharmacokinetics and pharmacodynamics. Aging is associated with changes in absorption, distribution, and elimination of medications. Pharmacodynamic changes refer to alterations in end-organ responsiveness occurring with aging. Common problems that further complicate psychotropic drug use in the elderly include polypharmacy, compliance, lack of specific diagnosis, and concomitant physical illness. The treating physician must be aware of these issues when prescribing psychotropic medications for the elderly.

Aged↗
PubMed1998

Drug-induced movement disorders.

Drug-induced movement disorders are often unrecognized, especially when not due to dopamine receptor blockers. This review discusses acute, subacute, and chronic syndromes. Pathophysiology relates almost always to dopaminergic transmission. Patient-dependent vulnerability and drug-dependent sensitivity are contributing factors. Young patients are more prone to acute reactions, and tardive or chronic conditions are more frequent in the elderly. Subclinical Parkinsonism can be unmasked by medication exposure. Treatment of tardive dyskinesia remains a challenging task for the clinician, but novel antipsychotics and dopamine depleting agents can be beneficial.

Drug-Related Side Effects and Adverse Reactions↗
PubMed1998

Intravenous levodopa in hallucinating Parkinson's disease patients: high-dose challenge does not precipitate hallucinations.

In five nondemented Parkinson's disease patients with daily visual hallucinations, we tested whether high-dose IV levodopa (LD) infusions precipitated hallucinations. Two infusion paradigms were studied, each with 1.5-mg/kg hourly dose for 4 hours--steady infusion and pulse infusion of the full hour dose over 5 minutes each hour. In both protocols, plasma LD levels changed significantly during the infusion protocol. The cumulative area under the curve was equivalent for the two infusions. All patients remained alert, and none developed visual hallucinations. The two patients with peak-dose dyskinesias on oral LD developed prominent dyskinesias during the infusion. Visual hallucinations do not relate simply to high levels of LD or to sudden changes in plasma levels.

Aged↗
PubMed1998

Georges Guillain's neurologic exploration of America.

This is a study of the status of American neurology at the beginning of the 20th century as perceived by European neurologist Georges Guillain. Guillain trained in Paris, the international "Mecca" of neurology at that time. Whereas many Americans traveled and studied in Europe, very few European neurologists made the reverse journey to the United States. In 1902 Georges Guillain, age 26, traveled to the United States with the specific aim of evaluating neurologic services in America. Recent access to Guillain's personal travel journal and an article in French that he wrote after his return to Europe provide first-hand documentation of American neurology at the turn of the century from a European perspective. Using introductory references from his teacher, Pierre Marie, Guillain visited New York, Chicago, Boston, Philadelphia, and Baltimore. He studied hospitals, outpatient departments, laboratories, and universities, comparing them with institutions in his native France and other European capitals. Guillain was particularly struck by the creative energy of American neurologists, the broad-ranging programs in research and patient care, and the strong financial support provided by local philanthropists, universities, and governments. Guillain clearly foresaw the rising international role of American neurology and the prominence it would gain by the end of his career in the 1950s.

France↗
PubMed1998

Highlights of the North American and European experiences.

Two large, randomized, double-blind, placebo-controlled multicenter studies, one North American and one European, examined the efficacy and safety of 300 and 600 mg/day tolcapone in similar populations of patients (n = 379) with Parkinson's disease currently taking levodopa and experiencing motor fluctuations. Change in "on-off" function was the most important end-point assessment. The two studies found similar results. The percentage of "on" time improved significantly and "off" time was reduced. Daily levodopa dosage requirements decreased significantly. The most common and significant adverse event was increased dyskinesia; hallucinations were not frequently encountered.

Antiparkinson Agents↗