Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0233565 (bradykinesia)
 2,352 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Current models of basal ganglia dysfunction in primary dystonia propose that the excessive muscle activity results from an increase in the excitability of the primary motor cortex. Neurophysiological and neuroimaging studies, however, have shown consistently reduced movement-related sensorimotor cortical activity. To explore this paradox, we used transcranial magnetic stimulation (TMS) to examine changes in corticospinal excitability preceding and during ballistic movements of the wrist in 9 patients with primary dystonia affecting the arm and 9 matched control subjects. The onset time, rate of rise, and duration of changes in the excitability of corticospinal projections to the agonist muscle were normal in the patients with dystonia. Increases in excitability were selective to the initial agonist muscle, suggesting that the spatial recruitment of corticospinal neurons was normal. Nonetheless, movements were slower in the patients by an average of 26%. The onset of the first agonist muscle burst was normal in magnitude and timing but the activity in this muscle subsequently became attenuated as movement progressed. Muscle activity in antagonist and proximal muscles of the upper arm was reduced significantly in the dystonia patients. These findings support the view that movement preparation and initiation at the level of the primary motor cortex is normal in patients with dystonia. Bradykinesia could not be attributed to co-contraction or overflow of activity and was associated with reduced rather than excessive muscle activity.
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PMID:Corticospinal excitability accompanying ballistic wrist movements in primary dystonia. 1502 81

Many patients with Parkinson's disease (PD) suffer from severe bilateral appendicular off (bradykinesia, rigidity, tremor) and on (dyskinesia, dystonia) symptoms. After unilateral pallidotomy several of these patients still suffer from severe bradykinesia, rigidity, or dyskinesia of the ipsilateral side. In addition such symptoms as walking difficulty, freezing, trunk, neck, or facial dyskinesia are not significantly alleviated after unilateral pallidotomy. These patients seem to be good candidates for bilateral staged pallidotomy. The aim of this study is to evaluate the motor symptoms after staged bilateral pallidotomy in advanced PD patients. 34 patients were studied. The patients were assessed using UPDRS version 3, Hoehn and Yahr scale, Schwab and England scale before and up to 24 months after surgery in off and on state. In off drug state, the total motor score of the UPDRS compared to preoperative off drug state was improved by 61% at 24 months of follow-up. All cardinal features of PD improved significantly in postoperative drug off state compared to drug off state before bilateral pallidotomy--parkinsonian tremor (items 20-21) by 62%, rigidity (item 22 UPDRS) by 81% and bradykinesia (items 23-26) by 67%. Also gait including falling, freezing, walking (items 13-14-15 UPDRS) and gait and postural stability (items 29-30 UPDRS) showed good improvement by 69% with bilateral pallidotomy in off drug phases. There was minimal improvement in motor score of UPDRS in on state. Duration of dyskinesia and severity of dyskinesia (items 32-33 UPDRS) showed dramatic improvement after bilateral pallidotomy. Bilateral pallidotomy affords impressive elimination of all appendicular and truncal dyskinesias, dystonias, and generally improved all symptoms in off state.
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PMID:[Bilateral pallidotomy for the treatment of advanced Parkinson disease]. 1509 53

We have investigated the electromyographic (EMG) and kinematic characteristics of horizontal arm extension movements in patients affected by idiopathic cervical dystonia (ICD) as well as in normal subjects. In spite of the lack of an overt dystonic involvement of the muscles acting at upper arm level, all these patients were considerably bradykinetic. Although the degree of bradykinesia observed was comparable to that previously reported for the body segment directly affected by this patholgy (21,15,8), the EMG analysis of the agonist muscles indicated a specific pathophysiological mechanism. In particular, the recruitment of the posterior deltoid (pD) in ICD patients was severely impaired within the initial phase (130 ms) of the movement. On the other hand, within the same time span, the activation of the mD, a muscle that plays a more important postural role than the pD, was not significantly different between patients and normal subjects. This reduced recruitment in the initial phase of the AG1 appears responsible of the slowness of voluntary movements.
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PMID:Impaired agonists recruitment during voluntary arm movements in patients affected by spasmodic torticollis. 1524 67

We report on a large Brazilian kindred with young-onset parkinsonism due to either a homozygous or heterozygous mutation in parkin. A total of 6 members were affected: 5 were homozygous and 1 heterozygous for a deletion in exon 4. Two other heterozygotes also had extrapyramidal signs. All affected subjects showed characteristic features of parkin disease with foot dystonia and an excellent response to levodopa complicated by motor fluctuations and dyskinesia within 3 years of therapy. Careful clinical follow-up over 10 years showed the phenotype was similar in all the homozygotes with asymmetrical limb bradykinesia and early walking difficulties. Some acceleration of disability was observed in some of the cases as they entered the third decade of illness, but dementia was absent.
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PMID:Parkin disease in a Brazilian kindred: Manifesting heterozygotes and clinical follow-up over 10 years. 1564 Oct 13

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

Progressive multifocal leukoencephalopathy (PML) is caused by replication of JC virus in oligodendrocytes of immunocompromised patients. Common manifestations are focal motor and sensory deficits, gait abnormalities, speech and language disturbances, cognitive disorders, headache, and visual impairment. Although the occurrence of movement disorders is rare in PML, bradykinesia, rigidity, dystonia, myoclonic jerks and myoclonic ataxia have been described. Head tremor associated with PML has not been previously reported. We report two cases of PML in whom head tremor was present.
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PMID:Head tremor and progressive multifocal leukoencephalopathy in AIDS patients: report of two cases. 1583 82

The definition of apraxia specifies that the disturbance of performed skilled movements cannot be explained by the more elemental motor disorders typical of patients with movement disorders. Generally this does not present a significant diagnostic problem when dealing with 'higher-level' praxic disturbances (e.g. ideational apraxia), but it can be a major confound in establishing the presence of limb-kinetic apraxia. Most motor disturbances characteristic of extrapyramidal disorders, particularly bradykinesia and dystonia, will compromise the ability to establish the presence of loss of dexterity and deftness that constitutes this subtype. The term 'apraxia' has also been applied to other motor disturbances, such as 'gait apraxia' and 'apraxia of eyelid opening', that perhaps are misnomers, demonstrating the lack of a coherent nomenclature in this field. Apraxia is a hallmark of corticobasal degeneration (CBD) and historically this has received the most attention among the movement disorders. Corticobasal degeneration is characterized by various forms of apraxia affecting limb function, particularly ideomotor apraxia and limb-kinetic apraxia, although buccofacial and oculomotor apraxia can be present as well. The syndrome of parkinsonism and prominent apraxia, designated the 'corticobasal syndrome' (CBS), may be caused by a variety of other central nervous system pathologies including progressive supranuclear palsy (PSP), Alzheimer's disease, dementia with Lewy bodies and frontotemporal dementias. Distinct from the CBS, PSP and Parkinson's disease can demonstrate varying degrees of apraxia on selected tests, especially in those patients with more severe cognitive dysfunction. Diseases that cause the combination of apraxia and a primary movement disorder most often involve a variety of cerebral cortical sites as well as basal ganglia structures. Clinical-pathological correlates and functional imaging studies are compromised by both this diffuse involvement and the confusion experienced in the clinical evaluation of apraxia in the face of the additional elemental movement disorders. Finally, although apraxia results in clear disability in patients with the CBS, it is not clear how milder ideomotor apraxia found on specific testing contributes to patients' overall day-to-day motor disability.
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PMID:Apraxia in movement disorders. 1593 45

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

A group of neurodegenerative diseases is outlined that affect cortical and subcortical areas of the brain. These diseases give rise to atypical forms of dementia and, unlike Alzheimer's disease (AD), are often associated with neurological symptoms. Clinical symptoms reflect the localization of the degenerative process rather than the nature of the underlying histopathology. Degeneration of the frontal and anterior temporal lobe presents initially with behavioral alterations, but later in the course, impairment of cognition and activities of daily living develops. Posterior cortical atrophy affects the parietal and occipital association cortices and causes complex visual disturbances. In corticobasal degeneration (CBD) the focus of pathology includes the frontoparietal cortex and several subcortical nuclei, causing symmetrical rigidity, bradykinesia, myoclonus and dystonia. Progressive supranuclear palsy (PSP) involves the frontal, temporal and parietal cortex as well as parts of the brain stem. Clinical features include a hypokinetic rigid syndrome with nuchal dystonia and vertical gaze palsy. Huntington's disease is a prototypical autosomal dominant disorder that affects the extrapyramidal system and causes choreatic movements in combination with personality changes and cognitive deterioration. Amyotrophic lateral sclerosis (ALS) with dementia is a neurodegeneration of the frontotemporal cortex and of the anterior horn of the spinal cord. Behavioral change similar to frontotemporal dementia (FTD) is paralleled or followed by the classic features of motor neuron disease.
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PMID:Uncommon neurodegenerative causes of dementia. 1624 Apr 82

We report a case of a 46-year-old Japanese woman with hereditary progressive dystonia with marked diurnal fluctuations and dopa-responsive dystonia (HPD/DRD). She developed difficulty in walking at the age of 44 years due to bradykinesia as well as hand tremors, muscle rigidity, increased tendon reflexes and mild dystonia in the lower extremities, all of which responded remarkably to low doses of levodopa (150 mg/day). Biopterin and neopterin concentrations in the cerebrospinal fluid (CSF) were decreased. Analysis of the guanosine 5'-triphosphate cyclohydrolase I (GCH1) gene revealed a novel mutation (W53X) in one allele. The GCH1 activity that was expressed in mononuclear blood cells was almost half the normal value (usually 2-20% of the normal value (39.0+/-9.2 pmol/ml) in patients with HPD/DRD). The relatively conserved GCH1 activity that is expressed in stimulated peripheral blood mononuclear cells may be related to the late clinical symptoms in this patient.
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PMID:A case of late-onset Segawa syndrome (autosomal dominant dopa-responsive dystonia) with a novel mutation of the GTP-cyclohydrase I (GCH1) gene. 1628 69


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