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Biomedical subjects

L Schelosky

Publications and source records attributed to L Schelosky.

At least 19 recordsLinked to original sources

Reduction of Parkinsonian signs in patients with Parkinson's disease by dopaminergic versus anticholinergic single-dose challenges.

We investigated the effect of an anticholinergic (biperiden) and a dopamine agonist (apomorphine) on tremor, rigidity, and akinesia in patients with idiopathic Parkinson's disease. In a standardized, crossover study design 17 patients received single-dose challenges of 5 mg biperiden intravenously and a previously determined dose of apomorphine subcutaneously on 2 consecutive days. Resting (RT), postural (PT), and action tremor (AT) were assessed using spectral analysis of accelerometer data, and Unified Parkinson's Disease Rating Scale (UPDRS) scores for rigidity and akinesia were determined before and after administration of the study drug. Both single-dose challenges significantly reduced the amplitude of RT, PT, and AT, but only apomorphine significantly reduced UPDRS scores for rigidity and akinesia. In only one patient was tremor reduced by the dopamine agonist but not by the anticholinergic. We found that anticholinergic and dopaminergic agents are both effective in reducing tremor in IPD, and there was no evidence for a selective anticholinergic responsiveness of parkinsonian tremor. Akinesia and rigidity, on the other hand, were not improved by biperiden. We therefore conclude that dopaminergic substances are as effective as anticholinergics in patients with parkinsonian tremor and additionally improve other parkinsonian signs.

Adult↗

Unilateral painful legs and moving toes syndrome with moving fingers--evidence for distinct oscillators.

This case report presents a patient with painful legs and moving toes on the right side followed by the development of involuntary movements in his right hand. The frequencies of the semirhythmic muscle activities in both extremities were different. This finding excludes one central pacemaker for both and supports the notion that separate oscillators in the segmental interneuron pool of different spinal areas may drive the individual movements in this case.

Electromyography↗

Loss of dopamine-D2 receptor binding sites in Parkinsonian plus syndromes.

UNLABELLED: This study analyzed temporal changes of striatal dopamine-D2 receptor binding during the course of different extrapyramidal movement disorders using 123I-iodobenzamide (IBZM) SPECT. METHODS: Eighteen patients (9 with Parkinson's disease, 9 with parkinsonian plus syndrome) were followed for 11-53 mo. Dopamine-D2 receptor binding was assessed using 123I-IBZM SPECT at the beginning and at the end of the follow-up period. SPECT data were acquired 120 min postinjection of 3-5 mCi 123I-IBZM. A semiautomated algorithm was applied to the raw data for semiquantitative evaluation of regional cerebral receptor binding. RESULTS: Intraobserver (r = 0.992) and interobserver (r = 0.930) variance was low for the semiautomated interpretation of the SPECT examination of the dopaminergic D2 receptor binding, reflecting a highly reproducible SPECT algorithm. Mean specific dopamine-D2 receptor binding was lower in patients with parkinsonian plus syndrome compared to patients with Parkinson's disease on the initial (p < 0.001) as well as the follow-up study (p < 0.001). In patients with Parkinson's disease, we observed an unaffected receptor binding compared to a reduced binding of radiotracer in patients with parkinsonian plus syndrome during the course of the disease (p < 0.001). CONCLUSION: During the follow-up, patients with Parkinson's disease showed a constant dopamine-D2 receptor binding. In contrast, patients with parkinsonian plus syndrome revealed a decline of the binding of dopamine-D2 receptor. These findings are in agreement with histopathological data that demonstrated a preserved dopamine-D2 receptor status in patients with Parkinson's disease and a decline of the dopamine-D2 receptors in patients with parkinsonian plus syndrome. SPECT examinations using 123I-IBZM are useful for assessing dynamic changes of dopamine-D2 receptors in extrapyramidal movement disorders. Semiquantitative SPECT evaluations may provide valuable information for clinical management and prognosis of the patient with extrapyramidal movement disorders.

Adult↗

Quantitative assessment of botulinum toxin treatment in 43 patients with head tremor.

We treated 43 patients who had head tremor as the major complaint with local botulinum toxin type A (Btx A) injections into neck muscles: 29 patients were classified as suffering from tremulous cervical dystonia (TCD), and 14 had head tremor without dystonia (HT). All patients were clinically assessed by means of the Tsui scale and a 4-point pain scale at baseline and follow-up visit. Quantitative recordings of head tremor with a bidirectional accelerometer system (horizontal and vertical planes) placed on the forehead were obtained before and 2-3 weeks after Btx A injections. Muscle selection for an injection was based on the visible and palpable tremor oscillation in the involved neck muscles and on analysis of standardized simultaneous electromyographic recordings of six cervical muscles. Patients with HT received mean total doses of 400 units (U) of Dysport (Btx A) (range, 160-560 U) distributed between the two splenius capitis muscles. Patients with TCD received a mean total dose of 500 U Dysport (range, 320-720 U) injected into a mean of 3 muscles (range, 2-4 muscles). The condition of all patients with HT and of 26 of the 29 patients with TCD improved subjectively. The total on the Tsui scale as well as pain scores decreased significantly (p < 0.05) following treatment. Latency of onset, duration of improvement, and side effects showed no significant difference in HT and TCD. Amplitude of HT decreased significantly for both groups following treatment. The mean dominant peak frequency in TCD and HT was slightly less than 5 Hz and did not change significantly after treatment.

Adult↗

A new tremor mutant in the Pietrain pig: an animal model of orthostatic tremor? Clinical and neurophysiological observations.

A new tremor mutant, the "Campus syndrome" was studied in a breed of Pietrain pigs. Affected pigs showed a coarse tremor of the extremities when standing and walking, so they tended to remain recumbent, during which the tremor was suppressed. Needle electromyographic recordings of the semitendinosus muscle plus accelerometry revealed a high-amplitude 14- to 15-Hz tremor pattern activated specifically upon standing but maintained during walking. The observed syndrome thus bears similarities to the human condition of orthostatic tremor and might serve as an animal model for this as yet poorly understood disorder.

Animals↗

[Magnetic resonance tomography in patients with Parkinson's disease and Parkinson-plus syndromes].

PURPOSE: To define characteristic MR-findings in patients with clinically typical extrapyramidal movement disorders. METHODS: 15 patients with Parkinson's disease (PD), 9 with multisystem atrophy (MSA), and 6 with progressive supranuclear palsy (PSP) underwent MRI using a 1.5 T. Magnetom unit. Two investigators analysed the images with special regard to global and/or focal atrophy and to changes in signal intensity of the CNS in the consensus mode. Normal images of 10 subjects served as controls to patient's images. RESULTS: In all patients with PSP and MSA characteristic pathological findings on MRI were observed including regional changes within the extrapyramidal nuclei. In contrast all patients with PD had an unremarkable MRI study of the CNS. CONCLUSION: MRI enables us to define characteristic morphological changes of the brain in patients with extrapyramidal movement disorders. Early recognition of these findings avoids misdiagnoses in patients who are difficult to diagnose.

Adult↗

Directional bias of initial visual exploration. A symptom of neglect in Parkinson's disease.

In the present study, side preferences in spontaneous visual exploration were assessed systematically in 27 patients with idiopathic Parkinson's disease (IPD) and 17 age-matched controls. Assessment of initial visual exploration asymmetry (IVE) was based on the exploration of texture arrays requiring attentive oculomotor scanning. As shown in a previous study, most healthy subjects exhibit a marked asymmetry of IVE with a strong left-sided bias when assessed by this paradigm, while most neglect patients initiate exploration in the right half of the arrays. Standard assessments for symptoms of neglect (line bisection, line cancellation and double simultaneous stimulation) were performed as reference tests. In the IVE task 65% of normal controls and 69% of patients with predominantly right-sided IPD started exploration in the left half of the arrays. By contrast only 14% of patients with predominantly left-sided disease showed a leftward IVE. The majority shows an ambiguous (21%) or rightward (64%) directional bias for initial exploration and thus a behaviour that corresponds to the IVE abnormalities found in neglect patients. No abnormalities were found in the standard neglect tests in any of the groups. The atypical IVE in patients with predominantly left-sided Parkinson's disease should be interpreted in the context of recent concepts of attention postulating that a bias in early spontaneous orientation directed to the ipsilesional hemifield reflects a mild and residual manifestation of hemineglect. Since this subtle orientational bias is less subject to compensation than more conspicuous clinical signs of neglect, sensitivity is higher in IVE testing than in conventional neglect assessments in chronic disorders with subclinical neglect. The present findings contribute a new aspect to the complex picture of cognitive and visuospatial abnormalities in Parkinson's disease. Furthermore our results extend previous knowledge on the mechanisms of neglect and the role of dopamine in the mediation of attention.

Aged↗

Botulinum toxin in writer's cramp: objective response evaluation in 31 patients.

OBJECTIVES: To quantify the treatment effect of local botulinum toxin injections in writer's cramp a newly developed rating scale of writing performance and a computer assisted analysis of writing speed were used in 31 patients undergoing botulinum toxin therapy. METHODS: Baseline data of the writer's cramp rating scale (WCRS, see appendix) and computer based writing speed analysis were compared with those obtained at the time of subjective best response as recorded during follow up visits. RESULTS: The mean dose injected per session was 133.2 units Dysport divided between two forearm muscles. Of all 124 injection sessions during mean follow up of one year 76% produced a good improvement. The most common side effect was weakness (72% of the follow up visits). The WCRS scores as assessed by a blinded videotape review by four independent raters showed good reliability between raters and a significant improvement after treatment (P < 0.001). The speed of pen movements showed a significant (P < 0.05) increase after treatment at subjective best effect recordings and a significant correlation with WCRS subscores, documenting the validity of the scale. CONCLUSION: The present study is the first to show significant effects of botulinum toxin treatment in patients with writer's cramp on the basis of a quantifiable scale for writing performance which correlates significantly with writing speed measurements. The WCRS as employed in this study might therefore prove a useful rating instrument in other studies assessing severity and treatment response in patients with writer's cramp.

Adult↗

Iodine-123-iodo-lisuride SPECT in Parkinson's disease.

UNLABELLED: Recently, [123I]iodo-lisuride was synthesized for possible applications in SPECT studies. The purpose of this investigation was to compare the striatal binding and kinetics of this radioligand in patients with Parkinson's disease and normal controls. METHODS: Six patients with Parkinson's disease and three normal controls were examined. After intravenous injection of 111 MBq [123I]iodo-lisuride, sequential SPECT examinations at 20, 40, 80 and 120 min were performed. For each SPECT series the basal ganglia-to-cerebellum ratio of tracer accumulation was calculated. In one patient a repeat SPECT examination was undertaken under identical conditions to test the reproducibility of the procedure. In two other patients a second SPECT examination was performed after injection of cold lisuride as a receptor saturation study. In addition, the time course of the radioactivity was measured in the plasma and red blood cells in each individual. RESULTS: In both patients and controls, the highest tracer accumulation was found within the striatum. The basal ganglia-to-cerebellum ratio was 1.182 and 1.303 at 20 min, 1.353 and 1.450 at 40 min, 1.490 and 1.533 at 80 min, 1.550 and 1.583 at 120 min for patients and controls, respectively, which was not statistically different. In the saturation study, 50 micrograms and 100 micrograms cold lisuride led to a 28% and 33% reduction, respectively, of the basal ganglia-to-cerebellum ratio at 120 min. The ligand showed a rapid decline in plasma and red blood cells. The percent injected dose per liter was calculated to be 1.6 and 0.9, respectively, for plasma and red blood cells at 20 min. CONCLUSION: Iodine-123-iodo-lisuride SPECT seems useful for imaging intact striatal dopamine D2 receptors in patients with Parkinson's disease and may provide clinically relevant information for quantitative assessment of the availability and integrity of dopamine D2 receptors.

Adult↗

[Cerebral SPECT with iodine-123 IBZM in patients with extrapyramidal system disorders: the evaluation of its sensitivity in therapy with dopaminergic drugs].

The functional integrity of striatal post-synaptic dopamine D2 receptors is requested for an effective pharmacologic treatment in patients with extrapyramidal movement disorders. Iodine-123 IBZM Single Photon Emission Computed Tomography (SPECT) is a noninvasive radionuclide technique for the morpho-functional imaging of post-synaptic dopamine D2 receptors. In this study, the results of iodine-123 IBZM SPECT and those of apomorphine tests were compared in 32 patients with extrapyramidal movement disorders--22 patients with idiopathic Parkinson's disease (IPD) and 10 with Parkinson's plus syndrome (PPS). Iodine-123 IBZM uptake was measured as the ratio between striatum and frontal cortex activities. Twenty of 22 IPD patients (91%) responded to apomorphine administration, while in 8 of 10 PPS patients (80%) the apomorphine test was negative. Iodine-123 IBZM uptake was significantly higher (p < 0.01) in IPD patients (1.39 +/- 0.114) than in PPS patients (1.27 +/- 0.078). Similarly, iodine-123 IBZM uptake was significantly higher (p < 0.01) in the patients with positive than in those with negative apomorphine test (1.38 +/- 0.113 vs. 1.26 +/- 0.078). In conclusion, the results of this study demonstrate that iodine-123 IBZM SPECT is a radionuclide technique capable of characterizing the patients with extrapyramidal movement disorders and of selecting the subjects who may respond to pharmacological dopamine treatment.

Adult↗

Hemimasticatory spasm in hemifacial atrophy: diagnostic and therapeutic aspects in two patients.

We report two cases of hemimasticatory spasm in association with progressive hemifacial atrophy. On the basis of neurophysiological and magnetic resonance imaging assessments, a peripheral irritation of the trigeminal nerve--probably due to entrapment of the motor branches in the infratemporal fossa--is suggested as the cause of the involuntary movement. Local injections of botulinum toxin type A into the masticatory muscles proved to be a successful treatment in both patients.

Adult↗

The "campus syndrome" in pigs: neurological, neurophysiological, and neuropharmacological characterization of a new genetic animal model of high-frequency tremor.

Inherited neurological diseases in animals are of interest to a wide range of scientific disciplines, particularly because such animals may be suited as genetic animal models for respective human disorders. Because the pig has a number of anatomic and physiologic features similar to those of human beings, this species is becoming increasingly popular in biomedical research. The usefulness of pigs as genetic models of neurological diseases is illustrated by the porcine model of malignant hyperthermia (MH), i.e., a frequently fatal myopathic disease in both pigs and humans. In the present study, we describe a new hereditary movement disorder in Pietrain pigs, which may represent a useful genetic animal model of high-frequency tremor. Because the disorder was first detected in the offspring of a boar named "Campus," we use the term "Campus syndrome" in this respect. Segregation analysis of breeding studies indicates that the syndrome is inherited as a monogenic dominant trait. DNA-based testing of the mutation involved in MH myopathy showed that expression of the Campus syndrome in pigs is not dependent on homozygosity for the MH mutation. In affected pigs, the Campus syndrome develops at an average age of 27 days. The syndrome is characterized by muscular weakness and a very intense tremor of the legs when standing and walking but not when at rest in a lying position. The intensity of tremor and muscular weakness progressively increases with age, resulting in pronounced postural instability. Despite these neurological abnormalities, body weight gain in affected pigs does not differ from that in unaffected siblings.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Ischemic stress induces deposition of amyloid beta immunoreactivity in human brain.

The histoblot immunostaining technique for locating and characterizing amyloidogenic proteins was used to obtain information about the relationship of cerebral ischemia/hypoxia to the accumulation of amyloid beta protein (A beta). We investigated brains of 131 subjects (ages 25-94 years, mean 72 years). Three distribution patterns of A beta immunoreactivity were identified: (1) colocalization with diffuse and neuritic plaques of Alzheimer's disease (AD) and aging; (2) diffuse punctuate deposits in the cerebral cortex in association with small vessel cerebral vascular disease ; and (3) cerebral cortical accumulation localized to arterial boundary zones and other regions susceptible to ischemic/hypoxic injury designated "stress-induced deposits" (SID). SID were not identified in tissue sections by immunohistochemical, Congo red or Bielschowsky silver techniques; no histological abnormalities were present in adjacent formalin-fixed tissue sections, SID occurred in subjects with histories of cerebral ischemia, and severe orthostatic hypotension. There was also an association with aging in general and with the incidence of neuritic plaques specifically. These latter findings are consistent with the hypothesis that brain ischemia/hypoxia plays a role in the pathogenesis of AD.

Adult↗

[Brain SPECT with 123I-lisuride in patients with Parkinson's disease and controls].

The goal was to visualize cerebral dopamine-D2 receptors in 6 patients with Parkinson's disease and in 3 healthy controls using iodine-123-Lisuride-SPECT. In addition, we performed receptor-replacement studies using 123I-Lisuride and cold Lisuride as competitive ligands. The highest uptake of 123I-Lisuride was observed in the striatum, a region with known high dopamine receptor density. In two patients premedication with cold Lisuride displaced 123I-Lisuride from the dopamine receptor. 123I-Lisuride is valuable as a radiotracer in cerebral dopamine-D2 receptor scintigraphy. Whether or not it is possible to determine dynamic changes of dopamine receptor density or function by receptor replacement studies needs further evaluation in larger patient populations.

Adult↗

[Life expectancy and disability in Parkinson disease].

Idiopathic Parkinson's disease is a chronic progressive neurodegenerative disease with no possibility of curative treatment up until now. The natural course leads to severe handicap or death within 10-15 years, but due to the development of dopaminergic drug therapy life expectancy of parkinsonian patients has normalized and productivity remains good for a longer period during the course of disease.

Aged↗

Dystonia in ataxia telangiectasia: report of a case with putaminal lesions and decreased striatal [123I]iodobenzamide binding.

A 6-year-old girl with ataxia telangiectasia and severe progressive dystonic posturing is presented. Magnetic resonance imaging showed cerebellar atrophy and a right-sided putaminal lesion. A single-photon emission computed tomography study of cerebral dopamine-(D2)-receptor binding with [123I]iodobenzamide showed a decreased tracer uptake in the striatum bilaterally. Dystonia deteriorated with levodopa treatment, whereas trihexyphenidyl led to significant improvement. Although dystonic symptoms have been repeatedly described in ataxia telangiectasia, this is the first report demonstrating structural and functional basal ganglia abnormalities in this disorder.

Ataxia Telangiectasia↗