Showing papers in "Movement Disorders in 1992"

Pathophysiology of cerebellar ataxia.

Abstract: Human and animal experiments performed recently have resulted in a more detailed understanding of limb movement and body posture disorders associated with cerebellar dysfunction. The delay in movement initiation can be explained by a delay in onset of phasic motor cortex neural discharge owing to decreased input from the cerebellar hemispheres. Disorders of movement termination (dysmetria), which can occur for movements at proximal and distal joints, result from disturbances of the timing and intensity of antagonist electromyographic (EMG) activity necessary to break the movement. Disorders in velocity and acceleration of limb movements result from muscular activity that is smaller in amplitude and more prolonged. The cerebellum is important for control of constant force but not for generation of maximal force. Dysdiadochokinesia is explained by a combination of the above mentioned mechanisms. During complex movements in three-dimensional space, the cerebellum contributes to timing between single components of a movement, scales the size of muscular action, and coordinates the sequence of agonists and antagonists. The basic structure of motor programs is not generated in the cerebellum. Hypotonia can be observed only in acute cerebellar lesions. Cerebellar tremor appears to result from a central mechanism, but is modulated or provoked through increased long-loop EMG responses. The common assumption that cerebellar ataxia of stance does not improve with visual feedback is true only of vestibulocerebellar lesions, not for ataxia resulting from atrophy of the anterior lobe of the cerebellum.

Levodopa-induced dyskinesias in Parkinson's disease: clinical and pharmacological classification.

Abstract: Levodopa-induced dyskinesias (LID) in Parkinson's disease (PD) may be classified into three main categories: "On" dyskinesias, diphasic dyskinesias (DD), and "off" periods. The study of 168 parkinsonian patients showed that about half (n = 84) showed one pattern of LID only. A combination of two was present in 68, and 16 had the three presentation patterns. A fairly good correlation between type of dyskinesia and presentation pattern was established. Chorea, myoclonus, and dystonic movements occurred during the "on" period. Dystonic postures, particularly affecting the feet, were mainly present in the "off" period, but a few patients had a diphasic presentation. Repetitive stereotyped movements of the lower limbs always corresponded to DD. Acute pharmacological tests using dopamine agonists (subcutaneous apomorphine 3-8 mg; intravenous lisuride 0.1-0.15 mg) and dopamine antagonists (intravenous sulpiride 200-400 mg and intravenous chlorpromazine 25 mg) were performed in 40 patients. Dopamine agonists enhanced "on" dyskinesias and markedly reduced or abolished "off" period dystonia and DD. Dopamine antagonists reduced all types of LID but usually aggravated parkinsonism. These clinical and pharmacological results indicate that LID in PD are a heterogeneous phenomenon difficult to explain on the basis of a single pathophysiological mechanism.

Cerebrospinal fluid antibodies to coronavirus in patients with Parkinson's disease.

Abstract: The etiology of Parkinson's disease remains unknown, and a search for environmental agents continues. In 1985, Fishman induced infection of the basal ganglia by a coronavirus in mice. Although coronavirus is recognized primarily as a respiratory pathogen in humans, its affinity for the basal ganglia led us to investigate its possible role in human Parkinson's disease. The cerebrospinal fluid of normal controls (CTL) (n = 18), and patients with Parkinson's disease (PD (n = 20) and other neurological disease (OND) (n = 29) was analyzed in a blinded manner by enzyme-linked immunosorbent assay [measurements in optical density (OD) units] for antibody response to four coronavirus antigens: mouse hepatitis virus JHM (J) and A59 (A), and human coronavirus 229E (E) and OC43 (O). When compared with CTL, PD patients had an elevated (p less than 0.05) mean OD response to J (0.0856 vs. 0.0207) and A (0.1722 vs. 0.0636). Response (p greater than 0.05) to O (0.0839 vs. 0.0071) was greater than that to E (0.1261 vs. 0.0743). When compared to OND, PD patients had an elevated mean OD response to J (0.0856 vs. 0.0267, p less than 0.05). Responses (p greater than 0.05) to A (0.1722 vs. 0.0929) and O (0.0839 vs. 0.0446) were greater than that to E (0.1261 vs. 0.0946). These results suggest that there may be an association between coronavirus and PD.

Machado‐Joseph disease: An autosomal dominant motor system degeneration

Abstract: Machado-Joseph disease is an autosomal dominant spinocerebellar degeneration. It expresses itself clinically with variable expression. Type one patients have early onset with a rapid progression of symptoms including spasticity, rigidity and myokymia. Type two patients are the most common phenotype with ataxia and spasticity. Type three patients develop progressive ataxia with variable amyotrophy. All patients have ophthalmoparesis and normal mental status. The neuropathology consists of neuronal loss and gliosis in the substantia nigra, motor cranial nuclei, dentate nucleus of the cerebellum, and variable neuronal loss with gliosis in the cerebellar cortex and neostriatum. The cerebral cortex is normal histologically. The inferior olivary nuclei are normal, thus separating this disease from olivopontocerebellar atrophy (OPCA). The disease has a worldwide distribution including families described in Portugal, the Azores, Spain, Italy, United States, Canada, Brazil, China, Taiwan, and Japan. The gene has not been mapped for this disease but the locus on chromosome 6p mapped for most families with OPCA has been excluded for this disorder.

Neurotoxicity of levodopa on catecholamine-rich neurons.

Abstract: The human neuroblastoma cells NB69 are a catecholamine-rich cell line with pharmacological properties similar to dopamine neurons. This cell line was used to study the neurotoxicity of levodopa on catecholamine neurons. Levodopa, at 50 x 10(-6) M or higher concentrations, produced a dose- and time-dependent reduction in the number of live cells, [3H]thymidine uptake, levels of protein and DNA, and an enhancement of the quinone formation. This is a specific effect of levodopa since it did not happen in NB69 cells incubated with equimolar concentrations of leucine and tryptophan. Treatment with deprenyl, an inhibitor of monoamine oxidase type B, partially prevented levodopa neurotoxicity, suggesting that the mechanism of toxicity was, at least in part, related to an increase in the metabolism of dopamine catalyzed by monoamine oxidase.

Voluntary stimulus-sensitive jerks and jumps mimicking myoclonus or pathological startle syndromes.

Abstract: Five patients who presented with stimulus-induced jerking as part of an apparent myoclonic or pathological startle syndrome are reported. Neurophysiological observations in these patients suggested the jerks were voluntary in origin. These included (a) variable latencies to the onset of stimulus induced jerks, (b) latencies were greater than that seen in reflex myoclonus of cortical or brainstem origin, and were (c) longer than the fastest voluntary reaction times of normal subjects, (d) variable patterns of muscle recruitment within each jerk and, (e) significant habituation with repeated stimulation. It is argued that these features are consistent with a voluntary origin for the jerks and enable them to be distinguished from the stereotyped electrophysiological characteristics of myoclonus of cortical and brainstem origin. Electrophysiological recordings may help identify patients with this form of psychogenic movement disorder.

Dystonia following head trauma: a report of nine patients and review of the literature

Abstract: We report nine patients who developed dystonia following head trauma. The most frequent form was hemidystonia only (six patients). One patient presented with hemidystonia plus torticollis, one with bilateral hemidystonia and one with torticollis only. Seven patients sustained a severe head injury, and two had a mild head injury. At the time of injury, six were younger than 10 years, two were adolescents, and the patient with torticollis only was an adult. Except in the patient with torticollis only, the onset of dystonia varied considerably from months to years. All patients with hemidystonia had posthemiplegic dystonia of delayed onset. Seven out of 8 patients with hemidystonia had lesions involving the contralateral caudate or putamen, as demonstrated by CT and MR. The patient with hemidystonia plus torticollis had no lesion to the basal ganglia, but a contralateral pontomesencephalic lesion. Response to medical treatment was generally poor. Functional stereotactic operations were performed in seven patients. A variety of factors may be responsible for the vascular or nonvascular posttraumatic basal ganglia lesions, which may lead to dystonia. The pathophysiology seems to be more complex than thought previously. We believe that dystonia following head injury is not as rare as is assumed. Awareness of its characteristics and optimized diagnostic procedures will lead to wider recognition of this entity.

When did Ray Kennedy's Parkinson's disease begin?

Abstract: Ray Kennedy's Parkinson's disease probably began during his distinguished career as a professional soccer player at least 10 years before the first unequivocal physical signs and 14 years before the diagnosis was finally made, when he was 35-years old. Early prodromal symptoms included intermittent subtle disturbances of movement and posture affecting the right arm and leg, mild facial immobility, episodes of profound malaise and lack of energy, inner feelings of tremulousness, excessive unprovoked bouts of perspiration, and accompanying feelings of heat. Abnormalities of movement in the right arm can be seen in video footage of soccer games up to 8 years before his disability came to medical attention. Many of his premorbid personality traits are characteristic of those believed to be associated with the subsequent development of the malady. At least in some patients with Parkinson's disease, the search for instigating aetiological factors should focus 10-20 years before the cardinal signs can be recognised with certainty.

Premorbid smoking, alcohol consumption, and coffee drinking habits in Parkinson's disease: A case-control study

Abstract: A number of studies have reported lower cigarette consumption in patients with Parkinson's disease (PD) previous to onset of the disease. In an attempt to determine whether there existed a "premorbid attitude" by patients against the use of socially accepted "drugs," the premorbid tobacco, alcohol, and coffee consumption habits were compared in 128 PD patients and 256 controls. Patients and controls were selected by case control method and were recruited from the same health area and socioeconomic stratum. In males, the habits of smoking more than 10 cigarettes/day (p < 0.001) and drinking more than 50 g/day of alcohol (p < 0.001) were significantly less frequent in the PD patients than in the controls, but the differences in coffee consumption were nonsignificant. In females behavior did not differ significantly between the PD group and the controls for any of the three habits. There was no correlation between the amount of smoking and alcohol drinking and age at onset of PD or current Hoehn and Yahr's staging. Our results suggest the existence of a premorbid personality in males with PD, possibly conditioning a restrictive attitude toward the consumption of such toxic substances as tobacco and alcohol, yet a more tolerant attitude toward habits more widely accepted socially, like coffee consumption.

Clozapine prevents recurrence of psychosis in Parkinson's disease

Abstract: Psychosis secondary to dopaminergic therapy can limit the ability to manage motor symptoms of advanced Parkinson's disease (PD). We report the results of an open label 3-month trial that evaluated the antipsychotic effects of clozapine in eight PD patients with drug-induced psychosis. Response was quantified using a simplified brief psychiatric rating scale and two PD scales. Clozapine significantly improved psychiatric scores at low doses. The use of every other day regimens (not previously utilized) led to good control of symptoms and minimized side effects. Clozapine also had a positive sleep effect in four patients and improved dyskinesia in one. Finally, this treatment prevented recurrence of psychosis while levodopa doses were significantly increased and while other antiparkinsonian medications were added. Motor disability related to PD improved as a result of these treatment adjustments. We conclude that clozapine is effective in treating drug-induced psychosis in PD and allows for safe optimization of antiparkinsonian therapy.

Corticobasal degeneration: Decreased and asymmetrical glucose consumption as studied with PET

Abstract: Cerebral energy metabolism was studied by positron emission tomography and [18F]fluorodeoxyglucose in five patients with clinical diagnoses of probable corticobasal degeneration. A reduction in glucose consumption was observed in most cortical and subcortical structures compared to age-matched controls. The reduction was greatest on the side of the brain contralateral to the most affected limbs, as shown by the significantly lower ratios of contralateral to homolateral metabolic rates, in the temporal and sensorimotor cortex of patients compared to controls. A distinct asymmetry between the two hemispheres could be observed in a patient who was examined twice in the course of his illness. Detection of this asymmetrical decrease in brain cortical and subcortical glucose metabolism may prove useful as additional evidence supporting clinical diagnoses of the disease.

Is nocturnal paroxysmal dystonia a form of frontal lobe epilepsy

Abstract: The sex ratio, age at onset, and ictal features of nine patients with nocturnal paroxysmal dystonia were compared with those of eight patients with daytime frontal lobe seizures and eight patients with nocturnal motor attacks of known epileptic origin. All patients underwent video electroencephalography telemetry. There were no clinical features that would allow distinction between the groups and no single phenomenon was uniquely seen in nocturnal paroxysmal dystonia. This study provides no evidence that nocturnal paroxysmal dystonia is a separate diagnostic entity.

Impairment of high-contrast visual acuity in Parkinson's disease.

Abstract: Several studies have shown that the visual system is affected in Parkinson's disease (PD) with reduced contrast sensitivity, low-contrast acuity, and flicker sensitivity, as well as altered electroretinograms (ERGs) and pattern visual evoked potentials (VEPs). Apparently, however, no study has yet specifically determined whether visual acuity to high-contrast stimuli is impaired in PD. Visual acuity was measured in a group of 16 patients with PD, both on and off drugs (for 24 h), and 16 age- and sex-matched normal control subjects. Acuity was impaired in the PD group both on standard Snellen chart and on a screen in a computerized test of visual resolution. The degree of impairment was 24 and 25%, respectively, in the two tests. The PD patients had marginally better acuity on both tests while receiving drugs, but the differences were not significant. The difference between the two groups was consistent with impaired resolution and could not be accounted for by any perceptual dysfunction that may also have been present in the PD group. Conversely, however, impaired acuity may be implicated in studies that have reported mild deficits of visuospatial/visuoperceptual function in PD. Reduced acuity appears to be a subtle sequela of dopaminergic deficiency in the visual system.

Exposure to well water and pesticides in Parkinson's disease: a case-control study in the Madrid area.

Abstract: Past exposure to well water and pesticides was assessed in 128 unselected Parkinson's disease (PD) patients and 256 age and sex-matched controls. All were residents in a defined urban area of Madrid, Spain. In keeping with other reports, we found that exposure to well water might be a factor associated with the likelihood of developing PD, though only prolonged exposures of 30 years or longer were significantly different between PD and controls (p less than 0.02). In contrast, past exposure to pesticides did not appear to be associated with an increased risk of developing PD. Prolonged well water drinking antedating the development of PD was not associated with early onset of the disease, nor did such cases progress to greater disability. Future case-control studies addressing prolonged well water consumption as a risk factor in PD should look for differences in the content of substances other than pesticides in the water as determined by the source of water to which patients may have been specifically exposed.

Cellular interactions in the striatum involving neuronal systems using "classical" neurotransmitters: possible functional implications.

Abstract: The neostriatum contains a wide variety of neuroactive substances associated with several well-defined functional neuronal systems. This structure, which is the seat of numerous neurological pathological disorders, is commonly used as a model for studying the basic mechanisms of neurotransmitter interactions in the brain and their putative involvement in striatal functions. Increasing interest has been focusing lately on the cellular interactions that may occur between the corticostriatal putatively glutamatergic system and the nigrostriatal dopaminergic input. Current evidence suggests that the activatory corticostriatal glutamatergic input may play a more crucial role in regulating striatal functions than was formerly assumed in comparison with the dopaminergic input. The key role of cholinergic interneurons in the striatum may therefore be attributable to the fact that they modulate the glutamatergic transmission to GABA striatal efferent neurons. Likewise, dopamine may actually act indirectly in the striatum by "tuning down" the cortical excitation of striatal neurons. Consequently, an impairment of the dopaminergic transmission such as that occurring in Parkinsonism may lead to an increase in the corticostriatal glutamatergic transmission, which may further contribute towards reinforcing the "imbalance" between subsets of striatal neuronal systems controlling the output of the basal ganglia.

Diffuse Lewy body disease presenting with supranuclear gaze palsy, parkinsonism, and dementia: a case report.

Abstract: A 67-year-old man with a family history of parkinsonism had visual complaints due to difficulty in convergence, which was followed 2 years later by development of bradykinesia and rigidity. The diagnosis of Steele-Richardson-Olszewski syndrome was made on the basis of a supranuclear gaze palsy, bradykinesia, rigidity, and poor response to levodopa. However, subsequent neuropathological examination revealed diffuse Lewy body disease with no evidence of neurofibrillary tangles involving either subcortical or brain stem structures.

The coordination of posture and voluntary movement in patients with cerebellar dysfunction

Abstract: Postural adjustments associated with the task of rising on tiptoes were investigated in a reaction time paradigm in 10 normal subjects and 18 patients with cerebellar disorders. Cerebellar dysfunction was due to either degenerative cerebellar disease, tumor, or ischemia. Displacements of the center of foot pressure (CFP) were recorded. The task, accomplished by the triceps surae muscle (executional activity, mean latency of 411 ms), is mechanically effective only if the center of gravity has been shifted forward in advance. To this effect, a phasic burst of preparatory EMG activity in the tibialis anterior normally occurs at a mean latency of 163 ms, shifting the center of gravity forward. Shortly thereafter, activity of the quadriceps femoris (175 ms) extends the knee and aids the forward shift of the center of gravity. Different aspects of this motor sequence were disturbed in individual patients: Latencies of preparatory and executional activity were uncorrelated in 15 of the 18 patients. Executional (n = 16) or preparatory (n = 13) EMG activity was tonic instead of phasic. Latencies of either preparatory or executional EMG activities or both were prolonged (n = 10). The time interval between motor preparation and execution was increased (n = 9). The trial-to-trial variability of biomechanical parameters and EMG latency was increased. Preparatory EMG activity in the quadriceps was entirely missing (n = 9), resulting in knee bending at the unsuccessful attempt to rise on tiptoes. Patients who were most severely affected had no preparatory activity at all (n = 2), and therefore were unable to perform the task.(ABSTRACT TRUNCATED AT 250 WORDS)

Baclofen in the treatment of idiopathic dystonia in children.

Abstract: Baclofen benefits some patients with adult onset dystonia, but few reports document the response to baclofen of children with idiopathic dystonia. Sixteen of 80 patients less than age 21 years with idiopathic dystonia seen by the Movement Disorder Group at Columbia-Presbyterian Medical Center in New York were treated with baclofen. Five had substantial improvement in symptoms, two had moderate improvement, and nine failed to benefit. Three of the improved patients had transient improvement on high dose anticholinergics, but had sustained, dramatic improvement when baclofen was added. At last follow-up, five patients maintained improvement for a mean 3.8 years (19 months-8 years) on a mean 79 mg of baclofen (40-120 mg). Response to baclofen did not correlate with age at onset of dystonia or age at onset of therapy, but did correlate with duration of symptoms before therapy (3 years for those who improved vs 7.8 years for those who did not: p less than .002 by t-test). Baclofen can be an effective treatment for childhood dystonia.

Golfers' cramp: clinical characteristics and evidence against it being an anxiety disorder.

Abstract: The clinical characteristics of 20 golfers suffering from golfers' cramp or the "yips" are described. The typical description is that of a middle-aged golfer who has played competitive golf since his teens and develops the problem during a tournament in the form of a jerk, spasm, or freezing of movement while putting or chipping, with the rest of the game being relatively unaffected. The problem generally takes a chronic fluctuating course, and a number of 'trick' strategies are partially or fully successful. In this study, the subjects were compared with a matched group of 20 unaffected golfers on a number of indices of psychopathology; no significant differences emerged. The more severely affected golfers also did not differ significantly from the mildly affected ones, except on the subjective report of anxiety. These data support the argument that golfers' cramp is not an anxiety disorder or a neurosis. The important role of anxiety and arousal in its manifestation is, nevertheless, recognized and its pathophysiology speculated upon.

Hemifacial spasm due to peripheral injury of facial nerve: a nuclear syndrome?

Abstract: Four cases of hemifacial spasm (HFS) are reported. The spasm followed a few months after injury to a peripheral branch of the seventh nerve. An EMG examination of facial muscles disclosed the typical finding of HFS: spontaneous activity, paradoxical cocontraction, and diffusion of spontaneous or provoked blinking. A nuclear involvement, secondary to the nerve lesion, is the most likely pathophysiological explanation for similar cases in HFS.

Interlimb coordination in Parkinson's disease

Abstract: This study examined the degree to which Parkinson's disease (PD) patients could "spatially link" the upper limbs to facilitate the performance of bimanual simultaneous movements. Six right-handed PD patients, and seven normal age- and sex-matched controls performed three different tasks: (a) an isotonic elbow flexion as rapidly as possible through an angle of 30 degrees; (b) an isometric contraction of the flexor muscles at the elbow joint to 40% and 60% of maximal volitional force (MVF) for a period of 5 s; (c) an isometric contraction for 2.5 s with one limb, then simultaneously performing an isotonic flexion with the contralateral limb while maintaining the isometric contraction for 2.5 s more. As expected, PD patients were significantly slower in performing the isotonic movement and produced lower peak velocities than the controls. More importantly, the two groups were differentially affected during the bimanual condition. In normals, movement time decreased and peak velocity increased in the bimanual condition. In contrast, PD patients showed increased movement times and sometimes decreased peak velocities in the bimanual condition. The results suggest that normal subjects utilize bilateral outflow to symmetrical muscle groups to synchronize the two limbs in the bimanual task, whereas PD patients dissociate the two limbs.

Effect of aging and dopaminomimetic therapy on mitochondrial respiratory function in Parkinson's disease

Abstract: Oxygen consumption and enzyme activity were evaluated in platelet mitochondria from 17 patients with Parkinson's disease. In comparison with age-matched controls, no consistent abnormality could be discerned in complex I, complex II-III, or complex IV oxygen consumption, or in the enzyme activity of these respiratory chain complexes. Neither chronic therapy with levodopa/carbidopa alone nor in combination with deprenyl significantly affected any measure of mitochondrial respiratory function. There was no discernible relationship between patient age or disease severity and any parameter of mitochondrial respiration. Moreover, blood lactate levels following glucose loading were not different in patients and controls. These results fail to support the occurrence of a generalized defect in any mitochondrial respiratory function in Parkinson's disease.

Severe generalised dystonia associated with a mosaic pattern of striatal gliosis.

Abstract: A mosaic pattern of striatal pathology is described in a male who developed severe generalised dystonia from the age of 10 years, and died at the age of 18 years. There was no family history of dystonia, and extensive investigations during his life failed to identify a cause for the dystonia. The caudate nucleus and putamen showed a network of cell loss and gliosis surrounding islands of preserved striatum. Dorsal parts showed confluent gliosis, and ventral parts were spared. The pattern suggested a correlation with patch-matrix organisation, but there was no correlation with the distribution of calbindin immunoreactive cells, which are present in the matrix of the classical striosome-matrix organisation. The pathological findings were unlike those in status marmoratus, perinatal hypoxia-ischaemia, Huntington's disease, and neuroacanthocytosis, but similar to those reported in a 44-year-old man with predominantly cranial dystonia.

Movement disorders as sequelae of central pontine myelinolysis: Report of three cases

Abstract: In three survivors of central pontine myelinolysis, dystonia (in two patients) and rest tremor (in one) were sequelae. The onset of these movements occurred 3 weeks to 5 months after the initial presentation with central pontine myelinolysis. Magnetic resonance imaging revealed basal ganglia lesions suggestive of extra-pontine myelinolysis in all three patients. We propose that the movement disorders seen in our cases are clinical correlates of extra-pontine myelinolysis.

Effects of a selective partial D1 agonist, CY 208-243, in de novo patients with Parkinson disease.

Abstract: A selective dopamine D1-receptor agonist, CY 208-243, was administered to 23 de novo patients who had had Parkinson disease (PD) for less than or equal to 3 months. The drug was first used as monotherapy and then in some patients in combination with a dopamine D2-receptor agonist, bromocriptine. Results showed that CY 208-243 exerted a mild antiparkinsonian action, and tremor was the main symptom that consistently improved. The addition of bromocriptine less than or equal to 15 mg to CY 208-243 did not result in additional improvement, but this might be due to the short duration of treatment and the low doses of bromocriptine. The study was prematurely discontinued for safety reasons. We conclude that D1-receptor stimulation may result in improvement of motor disability in PD.

Effect of ethosuximide on rest tremor in the MPTP monkey model.

Abstract: Based on the hypothesis that low-threshold calcium conductance in the thalamus might be involved in the pathophysiology of parkinsonian tremor, ethosuximide was given chronically to a monkey previously treated with MPTP and displaying exceptionally a typical rest tremor. After 5 days of daily treatment, the tremor was reduced by 60%. Diltiazem and verapamil which act on different calcium channels had no such effect. Ethosuximide also potentiated the anti-tremor effect of the dopamine D2 agonist LY-171555.

"Rubral" tremor induced by a neuroleptic drug

Abstract: "Rubral" tremor is a rare movement disorder that occurs typically with midbrain damage. It is defined by its presence at rest, with sustained posture, and with movement. Whether it is a single-tremor disorder or a combination of two distinct tremors is debated. This report chronicles a severe neuroleptic induced "rubral" tremor in a patient who had had a stable posttraumatic ataxia. The dramatic response to benztropine and bromocriptine is illustrated in the videotape.