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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Selected spastic patients with cerebral palsy can be helped by peripheral reconstructive surgery, of the upper limb. Although surgery cannot make a limb that was functionally poor into a perfect one, it can greatly improve the preoperative condition. Most poor surgical results are caused by incorrect selection of patients or poor execution of surgical procedures. The worst mistake is to perform soft tissue procedures--tendinous release or tendinous transfers--on a patient with pure athetosis. The results in these cases are unpredictable and often fail. The same concept is applied to rigidity, hypotonia, dystonia, and ataxia. The surgical program is organized according to the type and severity of the deformity (clinical groups). The goals are to correct the deformities and to improve the muscular balance of the hand in one surgical stage. It must be remembered that spastic muscle cannot be used for tendon transfer with the same efficiency as in patients with a flaccid paralysis. Results were satisfactory in 92% of cases of groups I and II. If group III is included, the percentage of satisfactory results reduces significantly.
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PMID:Surgical management of the hand in infantile spastic hemiplegia. 1459 53

Upon clinical examination of involuntary movement, its correct diagnosis and classification is most important for choosing its most appropriate treatment. Correct diagnosis of involuntary movements should be based on their clinical characteristics rather than laboratory data. First attention should be paid to the way of its occurrence; whether it repeats itself at a relatively regular period or it occurs irregularly. Among irregular movements, chorea and ballism (us) belong to the same category clinically as well as neuropharmacologically; both respond to benzodiazepine and D2 receptor blockers. Dyskinesia and akathisia are often drug-induced. Athetosis often overlaps chorea and dystonia. Dystonia is characterized by excessive muscle contraction in terms of strength, spread and duration. Recently involvement of the sensori-motor cortex is emphasized in generation of focal dystonia. Myoclonus is usually caused by abrupt muscle contraction but also by sudden cessation of muscle contraction (negative myoclonus). Myoclonus and tics are usually irregular, but they can be rhythmic or quasiperiodic. Tremor is often associated with dysfunction of basal ganglia, cerebellum or thalamus, but recently an increasing attention has been paid to involvement of the sensori-motor cortex. Some involuntary movement could show features of two or more kinds of involuntary movements or it may shift among different kinds of involuntary movements or among different subgroups of that particular involuntary movement. Therefore, it is not advisable to feel obliged to classify every single involuntary movement into any pre-existing category. Instead a more flexible approach is warranted.
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PMID:[How to clinically approach involuntary movements]. 1515 56

The main clinical characteristics and aetiology of drug-induced tremor and acute movement disorders (myoclonus, chorea, athetosis, dyskinesia, dystonia, tics) are reviewed and discussed in this article. Tremor is the most frequent drug-induced acute movement disorder. Drug intake (and, initially, true or 'hidden' neuroleptic or psychoactive drugs) should be suspected as the cause of every abnormal movement. Drug-induced acute movement disorders usually disappear spontaneously after withdrawal of the suspected drug without any drug treatment. Drug-induced tremor and movement disorders are often poorly described in the Summary of Products Characteristics (SPC).
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PMID:[Drug-induced tremor and acute movement disorders]. 1520 22

The term athetosis has progressively disappeared from the anglo-saxon literature which considers that athetosis is part of the spectrum of dystonia. These two clinical entities can be distinguished, however. Athetosis can be identified, searching for subtle semiological traits, in particular at the level of the hand. The earlier appearance of athetosis may be result from its onset during the early phases of development of the central nervous system. Despite its rarity, the clinical diagnosis of athetosis is important to consider from a prognostic point of view. Indeed, it results from brain lesions, and is therefore not a hereditary disorder as it may be the case for dystonia, and its evolution is relatively stable. The efficacy of treatments used in patients with dystonia, in particular high frequency pallidal stimulation, remains to be assessed in patients with athetosis. The concept of athetosis is still helpful in clinical practice.
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PMID:[Athetosis or dystonia?]. 1545 63

Severe injury to the cerebellum as a complication of extreme prematurity with extremely low birthweight was recently described in 13 children with the clinical diagnosis of cerebral palsy. We report another 10 cases of this syndrome. The clinical features include striking motor impairment and variable degrees of ataxia and athetosis or dystonia, which represent a distinct clinical type of cerebral palsy. Most are severely damaged, with cognitive, language, and motor delays. All are microcephalic, except one with hydrocephalus. Neuroimaging studies demonstrate the absence of major portions of the cerebellum involving both the inferior vermis and hemispheres. Most also have injury of a less severe nature in the cerebrum. This report indicates that this is not an uncommon outcome of extremely low birthweight infants, and we hope to encourage further investigations into the relative frequency and likely etiologies of the condition.
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PMID:Cerebellar injury in the extremely premature infant: newly recognized but relatively common outcome. 1579 81

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

Most movement disorders, reflecting degenerative disorders, develop in a slowly progressive fashion. Some movement disorders, however, manifest with an acute onset. We wish to give an overview of the management and therapy of those acute-onset movement disorders.Drug-induced movement disorders are mainly caused by dopamine-receptor blockers (DRB) as used as antipsychotics (neuroleptics) and antiemetics. Acute dystonic reactions usually occur within the first four days of treatment. Typically, cranial pharyngeal and cervical muscles are affected. Anticholinergics produce a prompt relief. Akathisia is characterized by an often exceedingly bothersome feeling of restlessness and the inability to remain still. It is a common side effect of DRB and occurs within few days after their initiation. It subsides when DRB are ceased. Neuroleptic Malignant Syndrome is a rare, but life-threatening adverse reaction to DRB which may occur at any time during DRB application. It is characterised by hyperthermia, rigidity, reduced consciousness and autonomic failure. Therapeutically immediate DRB withdrawal is crucial. Additional dantrolene or bromocriptine application together with symptomatic treatment may be necessary. Paroxysmal dyskinesias are childhood onset disorders characterised by dystonic postures, chorea, athetosis and ballism occurring at irregular intervals. In Paroxysmal Kinesigenic Dyskinesia they are triggered by rapid movements, startle reactions or hyperventilation. They last up to 5 minutes, occur up to 100 times per day and are highly sensitive to anticonvulsants. In Paroxysmal Non-Kinesiogenic Dyskinesia they cannot be triggered, occur less frequently and last longer. Other paroxysmal dyskinesias include hypnogenic paroxysmal dyskinesias, paroxysmal exertional dyskinesia, infantile paroxysmal dystonias, Sandifer's syndrome and symptomatic paroxysmal dyskinesias. In Hereditary Episodic Ataxia Type 1 attacks of ataxia last for up to two minutes, may be accompanied by dysarthria and dystonia and usually respond to phenytoin. In Type 2 they can last for several hours, may be accompanied by vertigo, headache and malaise and usually respond to acetazolamide. Symptomatic episodic ataxias can occur in a number of metabolic disorders, but also in multiple sclerosis and Behcet's disease.
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PMID:Diagnosis and management of acute movement disorders. 1620 29

Chronic bilirubin encephalopathy, characterized clinically by extrapyramidal movement abnormalities, vertical gaze abnormalities, and hearing loss, results from neuronal injury after marked hyperbilirubinemia in term and preterm infants. In premature infants, bilirubin staining of specific brain structures has been described at autopsy after only moderate hyperbilirubinemia, but classic chronic bilirubin encephalopathy without marked hyperbilirubinemia has been reported only rarely. We report a case of a 7-year-old, former 29-weeks' gestation, gravely ill premature infant with a peak bilirubin level of 13.3 mg/dL in the neonatal period. We compare this case with a 12-year-old, former term infant with a peak bilirubin level of 49.4 mg/dL on day 10 of life. Both children have dystonia, athetosis, upward gaze palsy, and sensorineural hearing loss, with MRIs showing characteristic abnormal signal in the globus pallidus. We add previously unreported cerebrospinal fluid neurotransmitter levels that show a mild decrease in the dopamine metabolite homovanillic acid in the former premature infant only.
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PMID:Clinical (video) findings and cerebrospinal fluid neurotransmitters in 2 children with severe chronic bilirubin encephalopathy, including a former preterm infant without marked hyperbilirubinemia VIDEO. 1626 13

Since its introduction in the late 1980s, intrathecal baclofen (ITB) therapy has become the standard treatment for severe generalized spasticity and dystonia in children. Treatment with ITB decreases spasticity in the upper and lower extremities and has been associated with improved function and decreased musculoskeletal contractures. In addition, ITB decreases generalized secondary dystonia and has been associated with improved comfort and ease of care in approximately 85% and with improved function in approximately 33% of patients. Continued effectiveness of ITB in treating spasticity has been observed for up to 17 years, and its effectiveness in treating dystonia has been observed for up to 10 years. Although ITB therapy is frequently associated with complications such as infections, catheter malfunctions, and cerebrospinal fluid leaks, the benefits of therapy appear to outweigh the risks. Additional investigation is needed to determine the effects of ITB on other movement disorders such as athetosis and chorea.
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PMID:Intrathecal baclofen therapy in children. 1691 24

We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7-37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%).
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PMID:Neurological manifestations in Wilson's disease: Report of 119 cases. 1707 70


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