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Query: UMLS:C0233565 (bradykinesia)
 2,352 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pediatric movement disorders constitute a relatively small cluster of symptoms that can be associated with many different underlying diseases. To provide effective treatment, it is essential to understand the relationship between etiology and clinical expression. This article reviews the recent literature on several common pediatric movement disorders, including spasticity, dystonia, chorea, myoclonus, bradykinesia, and tics, and it discusses current models of physiology that may help link the cellular pathology of specific diseases to the expression of clinical symptoms.
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PMID:Pathophysiology of pediatric movement disorders. 1367 68

Functional disability of patients with Huntington's disease (HD) is determined by impairment of voluntary motor function rather than the presence of chorea. However, only few attempts have been made to quantify this motor impairment. By using a simple reaction time paradigm, we measured the time needed for movement initiation (akinesia) and execution (bradykinesia) in 76 HD patients and 127 controls. Akinesia and bradykinesia were already evident in early stages and increased linearly with increasing disease stage. Quantified motor slowness correlated with clinical impairment of voluntary movements but also with cognitive impairment and medication use. In patients without severe cognitive impairment, quantified motor slowness reflected clinical motor impairment more purely. During 1.9 years follow-up (range, 0.8-3.8 years), quantified akinesia and bradykinesia progressed concomitantly with progression of clinical impairment of voluntary movements, cognition, and functional capacity. However, rate of change in motor slowness did not discriminate between patients whose disease stage remained stable and those whose disease stage progressed. We conclude that the reaction time paradigm may be used to quantify akinesia and bradykinesia in HD, at least in patients without severe cognitive impairment. Although reaction and movement times increased in time, these measures failed to detect functionally important changes during our follow-up period.
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PMID:Objective assessment of motor slowness in Huntington's disease: clinical correlates and 2-year follow-up. 1502 82

Bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) was performed in a patient with Huntington's disease (HD) with severe chorea. Stimulation at 40 and 130 Hz improved chorea. Stimulation at 130 Hz slightly worsened bradykinesia overall, whereas 40 Hz had little effect. A [15O] H2O positron emission tomography showed increased regional cerebral blood flow in motor decision making and execution areas more evident at 40 Hz. Adjustment of stimulation parameters in GPi DBS may have the potential to optimize the motor response in HD, improving chorea without aggravating bradykinesia.
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PMID:Bilateral globus pallidus stimulation for Huntington's disease. 1598 18

Huntington's disease (HD) is a neurodegenerative disorder characterised by cognitive, psychiatric and motor abnormalities including a range of involuntary movements. Currently, assessment of these movements involves the use of subjective rating scales such as the Unified Huntington's Disease Rating Scales (UHDRS) for bradykinesia and maximal dystonia and chorea, without any objective measures. As new therapies emerge, it is critical that an objective means of evaluating these abnormal movements is developed and we have investigated the use of a wrist-worn activity monitor, the Actiwatch-Neurologica, to determine whether these movements can be measured. In addition, this activity monitor and subjective reports were used to objectively measure the degree of sleep disruption in these same HD patients. Eight patients with mild-moderate HD and 8 age- and sex-matched control subjects wore the monitor for a period of 48 hours and recorded in a diary whether they were asleep or awake for each hour over the 2-day period. Assessment of various movement parameters revealed that HD patients exhibited significantly greater total and maximum activity levels and spent longer performing high acceleration movements while they were awake compared with controls. During sleep, patients not only showed significantly more activity and spent more time making high acceleration movements, but they also made significantly more movements than control subjects. These results demonstrate that the Actiwatch-Neurologica activity monitor can be used to objectively assess movements in HD patients during periods of high activity as well as during sleep.
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PMID:The use of the Actiwatch-Neurologica system to objectively assess the involuntary movements and sleep-wake activity in patients with mild-moderate Huntington's disease. 1574 12

We developed and tested the clinimetric properties of a scale for psychogenic movement disorders (PMDs). PMDs are disabling but lack any generally accepted treatment strategies. To develop treatments, means of assessing disease severity must be provided. No scale to assess PMDs existed. The PMD scale developed here rates 10 phenomena (rest tremor, action tremor, dystonia, chorea, bradykinesia, myoclonus, tics, athetosis, ballism, cerebellar incoordination), 2 functions (gait, speech), and 14 body regions. To study interrater agreement, three movement disorder neurologists independently rated 88 videotapes of PMD patients. Data analysis was performed using a kappa coefficient of agreement, Kendall's coefficient of concordance, Spearman correlations, and intraclass correlation coefficients. Validity and scale responsiveness were tested as well. All phenomena and speech and gait dysfunction occurred in the patient sample. A wide range of affected body regions, severity, and incapacitation was captured. Ratings showed excellent interrater reliability for presence or absence of each phenomenon (kappa range, 0.63 to 0.86). Kendall's concordance coefficients for phenomenology, function, and total PMD scores were 0.92, 0.93, and 0.91. Spearman correlations between raters ranged from 0.86 to 0.90. The scale was responsive to changes that occurred as a result of a neuropsychiatric intervention. The PMD scale adequately captures the complex movements of PMDs and can be used to assess PMDs and test the efficacy of intervention strategies.
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PMID:Rating scale for psychogenic movement disorders: scale development and clinimetric testing. 1610 25

Whereas adult-onset Huntington disease is a well-characterized clinical entity, childhood-onset cases have not received as much attention. In this report, the clinical, demographic, and genetic characteristics in 12 patients with childhood-onset Huntington disease are presented and compared with data in the literature. The patients were divided into two groups based on age at onset of symptoms (< 10 or > or = 10 years old). The majority of patients had onset of symptoms before 10 years of age and most at or below 5 years of age. The delay in diagnosis was longer in those with earlier onset of symptoms. Inheritance was paternal in all patients with onset beyond 10 years of age. We found a preponderance of male patients in the younger age at onset group and of female patients in the older age at onset group. The most frequent heralding symptom was cognitive decline in the group with earlier onset and oropharyngeal dysfunction in the later-onset group. Seizures occurred only in the younger age at onset group. Chorea was not a presenting sign but developed later in the course of the disease and, with dystonia, was more prevalent in the early age at onset group, whereas rigidity and bradykinesia were more prevalent in the older age at onset group. Patients in both groups developed gait, cognitive, and behavioral disorders at some point during the course of the disease. Furthermore, a slow and steady decline in IQ was observed on serial neuropsychologic testing in patients from both groups. Imaging studies were normal early and most commonly revealed neostriatal atrophy later in the course of the disease. In this report, we describe the characteristics of 12 patients with childhood-onset Huntington disease and review those previously reported, expanding our knowledge about the features of childhood-onset Huntington disease, underlining the differences with patients with adult-onset Huntington disease, and suggesting a differential phenotype within patients with childhood-onset Huntington disease depending on the age at onset.
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PMID:Clinical characteristics of childhood-onset (juvenile) Huntington disease: report of 12 patients and review of the literature. 1690 24

Instrumental measurement of complex motion sequences with a peg insertion paradigm reflects impairment in patients with Huntington's disease (HD). Objectives were to study progress of HD symptoms and peg insertion results in 39 HD patients without symptomatic drug treatment over an interval of 3 years. Assessments were at baseline and 3 years later. Unified Huntington's Disease Rating Scale (UHDRS) total score, computed arm score and the specific rating outcomes for bradykinesia, chorea, dystonia and oculomotor symptoms significantly increased over a 3-year period. Motor test outcomes significantly worsened. Cognitive scores did not change significantly, but were significantly related to the peg insertion outcomes and to the various UHDRS rating results at both assessments. Peg insertion scores are nonspecific diagnostic marker for progress in HD. Peg insertion particularly reflects motor impairment and additionally higher cognitive and executive dysfunction. These higher cognitive functions are associated with frontostriatal pathology in HD. Our study results suggest that our motor test reflects these emerging deficits of higher cognitive and motor function abilities in HD. We conclude, that instrumental assessment of complex movement sequences is an additional simple method to follow impairment in HD patients in addition to clinical rating.
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PMID:Complex movement behaviour and progression of Huntington's disease. 1732 83

The rate model regarding the development of movement disorders of basal ganglia origin suggests that hyperkinetic and hypokinetic disorders occur as a result of changes in the firing rates in the GPi and SNr, which in turn suppress thalamocortical output. Dopamine depletion in Parkinson's disease increases basal ganglia output, then decreases thalamocortical output, leading to bradykinesia. This model, however, cannot explain a lack of deterioration of parkinsonian signs following thalamic coagulation surgery. Instead of the rate model, the beta oscillation hypothesis has been proposed, explaining that synchronized oscillation in the beta frequency in the basal ganglia disturbs initiation of voluntary movement. We observed that effective high-frequency STN stimulation in parkinsonian monkeys was associated with increase in the firing rate and the pattern shift from irregular burst firing to regular high-frequency firing in the projecting sites. High-frequency neural activation by deep brain stimulation is supposed to cancel lower frequency oscillation including beta oscillation, leading to improvement of bradykinesia. Our observation supports the significance of the neural activity pattern, rather than the tonic activity level, in the development of movement disorders. The rate model cannot explain the improvement of ballismus and chorea by pallidotomy because pallidotomy increases the disinhibition of the thalamocortical projection, which should increase the movements. We observed repetitive bursts or pauses of neuronal firing of the globus pallidus synchronized to ballistic movements in patients with hemiballism or chorea, suggesting that phasic neuronal driving in the basal ganglia is important as their pathophysiology.
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PMID:[Functional models of movement disorders of basal ganglia origin and effects of functional neurosurgery]. 1749 32

Our goal was to determine whether bradykinesia is present in choreic adult-onset Huntington's disease (HD) patients, and determine the impact of chorea on their voluntary movements. We recorded whole-body involuntary movements (WBIM) and voluntary motor acts simultaneously, using a magnetic tracker system, in 15 choreic HD patients and 15 healthy age- and gender-matched control subjects. Participants were asked to perform two distinct tasks; a manual-tracking (MT) task yielding a measure of chorea intrusion during accurate movements, and a rapid alternating movement (RAM) task, yielding measures of bradykinesia. Results show that patients with HD presented with deviations from the target that hindered their ability to match the target velocity during the MT task. Furthermore, error in performance was correlated with the amplitude of whole-body chorea (Rho=0.67), illustrating the deleterious effect of chorea during accurate movements. However, patients with choreic HD presented with significantly higher RAM range and velocity than matched controls, therefore ruling out the idea that bradykinesia is a systematic feature of HD even when chorea is predominant. The present results imply that patients may have benefited from an intact direct pathway ("select ON" pathway in the focused attention model of basal ganglia function) that allowed them to supersede any dysfunctions associated with the progressive alteration of the "control function" (striatal-globus pallidus-subthalamic) pathway responsible for generating the chorea. Finally, the present results suggest that patients with adult-onset HD having chorea would greatly benefit from improved treatments aiming at reducing their involuntary movements while maintaining proper motor function.
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PMID:Bradykinesia is not a "systematic" feature of adult-onset Huntington's disease; implications for basal ganglia pathophysiology. 1817 45

The rate model regarding the development of movement disorders of basal ganglia origin suggests that hyperkinetic and hypokinetic disorders occur as a result of changes in the firing rates in the GPi and SNr, which in turn suppress thalamocortical output. Dopamine depletion in Parkinson's disease increases basal ganglia output, then decreases thalamocortical output, leading to bradykinesia. This model, however, cannot explain a lack of deterioration of parkinsonian signs following thalamic coagulation surgery. Instead of the rate model, the beta oscillation hypothesis has been proposed, explaining that synchronized oscillation in the beta frequency in the basal ganglia disturbs initiation of voluntary movement. We observed that effective high-frequency STN stimulation in parkinsonian monkeys was associated with increase in the firing rate and the pattern shift from irregular burst firing to regular high-frequency firing in the projecting sites. High-frequency neural activation by deep brain stimulation is supposed to cancel lower frequency oscillation including beta oscillation, leading to improvement of bradykinesia. Our observation supports the significance of the neural activity pattern, rather than the tonic activity level, in the development of movement disorders. The rate model cannot explain the improvement of ballismus and chorea by pallidotomy because pallidotomy increases the disinhibition of the thalamocortical projection, which should increase the movements. We observed repetitive bursts or pauses of neuronal firing of the globus pallidus synchronized to ballistic movements in patients with hemiballism or chorea, suggesting that phasic neuronal driving in the basal ganglia is important as their pathophysiology.
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PMID:[Functional models of movement disorders of basal ganglia origin and effects of functional neurosurgery]. 1821 Jul 85


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