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Nursing time to program and assess deep brain stimulators in movement disorder patients.

The use of deep brain stimulation (DBS) to treat movement disorders such as Parkinson's disease, essential tremor, and dystonia is increasing. Although some published literature describes the methods for DBS programming, the time and nursing requirements to run a DBS surgical program have not been examined previously. For this study, we prospectively recorded the time required for both assessments and programming of the DBS from the preoperative period to 1 year after surgery in a variety of patients. Results showed that the mean total time spent programming the stimulator and assessing these patients ranged from 18.0-36.2 hours per patient. It took twice as long to program the stimulator in patients with Parkinson's disease as it did in patients with essential tremor or dystonia. When setting up a program for movement disorders surgery, nursing time spent on patient assessment and programming should be considered in the workload.

Activities of Daily Living↗

DSM-IV stereotypic movement disorder: persistence of stereotypies of infancy in intellectually normal adolescents and adults.

BACKGROUND: As part of a broader series of studies on unwanted repetitive behaviors, DSM-IV stereotypic movement disorder (SMD) was examined in an intellectually normal population. Repetitive nonfunctional behaviors, or stereotypies, are expressed during early normal development but have not been described in adults without severe psychiatric or intellectual impairment. METHOD: Lifetime and current psychiatric Axis I diagnoses were determined by structured and clinical interviews in subjects who responded to a newspaper advertisement that specifically mentioned rocking and head banging. RESULTS: Of 52 potential subjects who were screened by telephone, 32 had been previously diagnosed with an Axis I psychiatric disorder, which presumably accounted for the repetitive behavior, or were otherwise excluded. Of 20 who were interviewed in person, 12 met DSM-IV criteria for SMD; rocking or thumb sucking was present in 8 of these 12. Four of 8 rockers had a first-degree relative who had a lifetime history of a similar repetitive behavior. A lifetime history of an affective or anxiety disorder was found for 11 of 12 SMD subjects. CONCLUSION: DSM-IV stereotypic movement disorder can be diagnosed in intellectually normal individuals. Although sampling bias was probable, prominent stereotypies in individuals meeting the DSM-IV criteria for stereotypic movement disorder, which are narrower than the DSM-III-R criteria for stereotypy/habit disorder, seem likely to include rocking and thumb sucking. The likelihood of persistence of these behaviors, which are developmentally appropriate in infancy, may be enhanced by comorbidity with anxiety or affective disorders.

Adolescent↗

Movement disorders in geriatric rehabilitation.

This article discusses the role of rehabilitation in movement disorders in the elderly. Diagnosis and differential diagnosis are discussed with an emphasis on the akinetic rigid syndromes. Treatment and its complications are specifically addressed. The hyperkinetic movement disorders are considered separately. New and impending advances in therapy are examined.

Aged↗

Control of poststroke involuntary and voluntary movement disorders with deep brain or epidural cortical stimulation.

The effects of deep brain or epidural cortical stimulation on poststroke involuntary and voluntary movement disorders were analyzed in a total of 64 patients. Among them, 22 patients underwent either deep brain or epidural cortical stimulation in order to control their poststroke involuntary movements. The remaining 42 underwent epidural cortical stimulation for the purpose of controlling their poststroke pain. In the latter group of patients, we analyzed the changes in coexistent involuntary or voluntary movement disorders during stimulation for pain control. Stimulation of either the thalamic nucleus ventralis intermedius or the motor cortex proved to be useful in 13 (59%) of the patients who underwent deep brain or epidural cortical stimulation for control of poststroke involuntary movements. Satisfactory control was often achieved in patients with hemiballismus, hemichorea or resting tremor. In 8 (19%) of the patients who underwent epidural cortical stimulation for pain control, stimulation of the motor cortex improved motor performance which had been impaired in association with hemiparesis. Such an effect was independent of pain control and apparently resulted from an inhibition of their rigidity. We believe that these findings justify further clinical studies on deep brain or epidural cortical stimulation for the control of poststroke movement disorders.

Brain↗

Electroconvulsive therapy in movement disorders: an update.

Electroconvulsive therapy (ECT) is a well-recognized treatment for psychiatric illness, primarily depressive disorders. Its use in patients with neurological illnesses is steadily increasing. Older reviews indicate that ECT may also benefit Parkinson's disease and similar movement disorders independent of its effects on comorbid psychiatric disorders. In this updated review, recent literature regarding ECT and movement disorders is summarized from 1990 to 2000. Considerable evidence indicates that ECT improves motor symptoms of Parkinson's disease in patients with and without mood disorders. A few case reports, ranging from one to six patients per disorder, suggest that ECT may ameliorate the motor symptoms of other movement disorders. ECT affects a variety of neurotransmitters that play a role in these diseases. Limitations of current reports are reviewed, and recommendations for further investigation are made.

Akathisia, Drug-Induced↗

The movement disorders of Coffin-Lowry syndrome.

Coffin-Lowry syndrome (CLS) is an X-linked semi-dominant condition with learning difficulties and dysmorphism caused by mutations in the gene RSK2. Originally, epilepsy was reported as a feature. We and others have since described predominantly sound-startle induced drop attacks that have been labelled 'cataplexy', abnormal startle response and hyperekplexia. We sought to clarify why there should be controversy over the type of paroxysmal events. Review of the literature and our patients confirmed that each centre had studied only a small numbers of individuals (mean = 2). The type of movement disorder varied both with age and between individuals. One individual might have more than one movement disorder. One of our adult patients had several types of movement disorder and epilepsy that merged seamlessly: there was true cataplexy triggered by telling a joke, something close to cataplexy ('cataplexy') triggered by sound-startle, a predominantly hypertonic reaction varying from hyperekplexia to a more prolonged tonic reaction resembling startle epilepsy, and true unprovoked epileptic seizures. In the large database of the Coffin-Lowry Syndrome Foundation family support group, 34 of 170 (20%) individuals with CLS and known age had 'drop attacks' and an additional 9 (5%) of these had additional epileptic seizures. The onset of such events was usually after age 5 years, prevalence peaking at 15-20 years (27%). Many became wheelchair bound as a result. This unique combination of more than one non-epileptic movement disorder and epilepsy deserves further semiological and genetic study both for the patients with CLS and for the wider implications.

Adolescent↗

Clonic perseveration following thalamofrontal disconnection: a distinctive movement disorder.

We describe four patients who developed asymmetrical, rhythmic, stereotyped, and repetitive movements of the upper and lower limbs hours to days after infarction that involved the thalamus and/or basal ganglia. The movements appeared to occur spontaneously and were initially labeled as focal motor seizures, ballism, or tremor; they could however, be induced by passive movement of the limbs. The movements most commonly observed were scratching or rubbing movements of the hands that were of such persistence as to cause trauma to the skin; in the lower limbs, the heel was run up and down the bed sheet, often until it bled. The movements were part of a syndrome characterised initially by a reduced level of consciousness and followed by aspontaneity, usually with mutism and frontal release signs. One patient who had relatively preserved cognition and language repeated words or phrases again and again when encouraged to speak, but had no difficulty changing responses appropriately to different cues. In drawing, he overwrote each figure but could change the figure on command. The distinctive movement disorder in these patients was due to clonic perseveration. We suggest that clonic perseveration results from disconnection of prefrontal cortico-basal ganglia-thalamo-cortical loops that are important for the termination of motor plans. Clonic perseveration should be recognised as a movement disorder following thalamic lesions.

Aged↗

Stereotaxic thalamotomy for treatment of posttraumatic movement disorders.

Stereotaxic ventrolateral thalamotomies have been successful in treating a wide spectrum of involuntary movement disorders, but very little has been reported concerning their use in posttraumatic movement disorders (MD's). This procedure has been used to treat 11 patients who developed persistent MD following severe closed head injuries. Among these, seven had action tremors, nine hemiballismic movements, two choreoathetoid movements, and two truncal ataxia. In two patients the MD was significant bilaterally, and in eight patients more than one type of MD was present. Standard thermal lesions based on the middle anterior commissure-posterior commissure line, 10 to 15 mm lateral to the midline, were performed following stimulation. Within the immediate postoperative period, all 11 patients had some degree of improvement in their MD; five showed marked improvement, four moderate improvement, and two minor improvement. One patient had recurrence of the MD 24 hours postoperatively requiring a second procedure, with marked improvement subsequently. In five of the six patients who underwent a left-sided procedure, a transient increase in preoperative dysarthria was noted. Nine patients had follow-up examinations 2 months to 3 years following surgery. Some persistent improvement in the MD was noted in all. Of three patients whose dysarthria was worse at 3 to 4 months, subsequent improvement was noted at 9 to 12 months in two. Stereotaxic thalamotomy appears to be an effective form of treatment for persistent posttraumatic MD. The major limitation is increased postoperative dysarthria. Further studies to evaluate risk factors associated with dysarthria and further refinement to prevent its occurrence are needed.

Adolescent↗

Masturbation in infancy and early childhood presenting as a movement disorder: 12 cases and a review of the literature.

PURPOSE: Infantile masturbation (gratification behavior) is not commonly identified as a cause of recurrent paroxysmal movements. Extensive and fruitless investigations may be pursued before establishing this diagnosis. Sparse literature is available regarding masturbatory behavior as a whole, but literature available as case reports describes common features. The purpose of this case series is to describe consistent features in young children with posturing accompanying masturbation. METHODS: Twelve patients presenting to a pediatric movement disorders clinic with a suspected movement disorder were determined to have postures and movements associated with masturbation. We reviewed the clinical history, examination, and home videotapes of these patients. RESULTS: Our patients had several features in common: (1) onset after the age of 3 months and before 3 years; (2) stereotyped episodes of variable duration; (3) vocalizations with quiet grunting; (4) facial flushing with diaphoresis; (5) pressure on the perineum with characteristic posturing of the lower extremities; (6) no alteration of consciousness; (7) cessation with distraction; (8) normal examination; and (9) normal laboratory studies. CONCLUSIONS: The identification of these common features by primary care providers should assist in making this diagnosis and eliminate the need for extensive, unnecessary testing. Direct observation of the events is crucial, and the video camera is a useful tool that may help in the identification of masturbatory behavior.

Child, Preschool↗

Advances in the genetics of movement disorders: implications for molecular diagnosis.

Recent developments in molecular genetics have had a profound influence on the diagnosis and classification of inherited movement disorders. Huntington's disease is caused by the expansion of an unstable trinucleotide repeat sequence. Molecular diagnosis can now be performed by a simple PCR-based assay, and the study of the effects of the repeat expansion on the function of the encoded protein will allow to elucidate the molecular pathogenesis of the disease. Wilson's disease is caused by a large number of different mutations, which complicates molecular diagnosis. Genes for a number of inherited dystonic syndromes have been mapped, one of them, the gene for dopa-responsive dystonia, has already been identified. The genetic basis of several other prevalent movement disorders, such as essential tremor and the restless-legs syndrome however, is still obscure. Current research is also directed at the identification of inherited risk-factors in genetically complex movement disorders, such as Parkinson's disease.

Atrophy↗

The schedule for the assessment of drug-induced movement disorders (SADIMoD): inter-rater reliability and construct validity.

The Schedule for the Assessment of Drug-Induced Movement Disorders (SADIMoD) is a newly developed instrument, consisting of a compilation of rating scales, to measure the severity of drug-induced movement disorders: dystonia, dyskinesia, Parkinsonism, akathisia, ataxia, and several types of tremors. The inter-rater reliability and the construct validity of this scale were investigated. Six investigator teams were trained by means of a standard package of instruction material to such an extent that a single member of the team could represent the entire team. Thirty-one patients [20 male/11 female; 57.1+/-6.5 yr (mean+/-S.D.)] with a variety of movement disorders were recorded on videotape according to the SADIMoD Schedule. Single representatives of all six teams scored these video recordings. To this set the existing SADIMoD ratings of 82 patients were added to form the so-called 'total data set'. These patients were examined by 6 different researchers, who rated 4, 8, 10, 14, 18 and 28 patients, respectively, mostly in the context of a research protocol. A specific subset consisted of 12 patients that were examined three times with a two-weekly interval without any change of their medical condition or treatment. The 6 ratings of the 31 individual patients correlated to a highly significant degree, with Kendall's Coefficients of Concordance of 0.436 to 0.891 (median 0.717). The same was true for the 6 ratings of the 7 SADIMoD subscales (median 0.578, range 0.462-0.715) Considering the total data set, the homogeneity of the various subscales was good (Cronbach's alpha = 0.81-0.94, median: 0.87). The SADIMoD dyskinesia and dystonia subscales showed a highly significant mutual correlation. The Parkinsonism subscale correlated highly significantly with the rest and postural tremor subscales and to a lesser extent with the akathisia and ataxia subscales. An analysis of variance showed that the three ratings in the subset of 12 patients were not significantly different for any scale. Also Scheffé tests for homogenous subsets did not reveal any significant differences. When investigated under 'real world' circumstances, the inter-rater reliability of the SADIMoD was found to be satisfying. The instruction material, that was developed and used in this study, fully comes up to the requirements. The construct validity of the SADIMoD is more than sufficient.

Adult↗

Choreas, hereditary and other ataxias, tics, myoclonus, and other movement disorders.

Developments in the field of Huntington's disease have focused on the potential benefits of predictive testing. Markers have been described for autosomal dominant cerebellar ataxia and for certain subtypes of Friedrich's ataxia. Argentophilic neuronal and glial inclusions appear to be the first specific pathologic hallmark of multiple system atrophy. "Pure" hereditary spastic paraplegia is not a multisystem disorder of the central nervous system, but a monomorphic and stereotyped disease. Advances in Tourette's syndrome are limited because the presumed gene eludes identification. A new type of myoclonus, propiospinal myoclonus, has been described. Clinical and electrophysiologic criteria for defining primary orthostatic tremor have been proposed. Understanding of the neurophysiologic substrate of essential tremor and myoclonus is improving. New neurologic disorders presenting clinically with prominent movement disorder continue to be described.

Ataxia↗

Acute movement disorders with bilateral basal ganglia lesions in uremia.

Acute and subacute extrapyramidal movement disorders are rarely reported in uremic patients. We report three such cases with basal ganglia lesions. All three had advanced renal failure with high serum creatinine levels. One of the patients had a history of ischemic heart disease and acute pulmonary edema with hypoxemia. Another patient had experienced arterial hypotension during previous hemodialysis. The third had prominent metabolic acidosis. One of the patients developed generalized dyskinesias, whereas the other two developed gait disturbances. Neuroimaging studies in all three cases showed bilateral changes in the basal ganglia. The natural history was self-limiting with gradual improvement. Diminution of the basal ganglia lesions was demonstrated on follow-up imaging in two of the three cases. We conclude that acute or subacute movement disorders with bilateral basal ganglia lesions may occur in uremia. Hypoperfusion with global brain ischemia and selective vulnerability of the basal ganglia to uremic toxins may account for these lesions.

Acute Disease↗

Antipsychotic medications and drug-induced movement disorders other than parkinsonism: a population-based cohort study in older adults.

OBJECTIVES: To study the relationship between initiating therapy with an antipsychotic medication and a subsequent new diagnosis of a drug-induced movement disorder other than parkinsonism in older adults with dementia. DESIGN: Retrospective, population-based cohort study. SETTING: Ontario, Canada. PARTICIPANTS: Ontario residents aged 66 and older with a diagnosis of dementia newly started on treatment with typical or atypical antipsychotic therapy. MEASUREMENT: Estimated relative risk of developing a drug-induced movement other than parkinsonism in the 1-year follow-up period after starting therapy with an antipsychotic medication. RESULTS: From April 1, 1997, to March 31, 2001, 21, 835 older adults with dementia who were newly started on antipsychotic medications were identified. Nine thousand seven hundred ninety subjects were started on atypical antipsychotics and 12,045 subjects started on typical antipsychotics. Demographic characteristics were similar between the groups. There were 5.24 cases of tardive dyskinesia (TD) or other drug-induced movement disorder per 100 person-years on therapy with a typical antipsychotic and 5.19 cases per 100 person-years on therapy with an atypical antipsychotic. The risk of developing drug-induced movement disorder while being treated with an atypical agent was not statistically different from that with a typical antipsychotic (relative risk=0.99, 95% confidence interval=0.86-1.15; P<.93). CONCLUSION: Older adults with dementia who are treated with typical or atypical antipsychotic therapy are at risk for developing TD and other drug-induced movement disorders.

Aged↗

Tetrabenazine therapy of pediatric hyperkinetic movement disorders.

Tetrabenazine (TBZ), a presynaptic dopamine depletor and postsynaptic dopamine receptor blocker, is widely used for the treatment of hyperkinetic movement disorders in adults. However, reports of its use in children are limited. We review the efficacy and tolerability of TBZ therapy in 31 children with hyperkinetic movement disorders refractory to other medications. TBZ was effective in reducing the severity of movement disorders resistant to treatment with other medicines. When compared to adult patients, pediatric patients required higher doses. Side effects were similar to the adult population; however, children had a lower incidence of drug-induced Parkinsonism.

Adolescent↗

[Movement disorders of drug origin].

The involvement of a drug must be suspected in each patient suffering from a movement disorder. Besides classical neuroleptics used as antipsychotics (butyrophénones, phenothiazines or benzamides), many drugs, mainly "hidden" neuroleptics (prescribed as antinausea, antivomiting, antivertigo, antispasmodic or antihypertensive drugs) or agents prescribed in psychiatric (antidepressants, lithium) or neurological (levodopa in Parkinson's disease, antiepileptics) diseases are known to be able to reveal or produce a movement disorder. Other drug prescribed in internal medicine can also be involved. This review discusses the main characteristics of drug-induced movement disorders as well as their pharmacological approach.

Adverse Drug Reaction Reporting Systems↗

[Functional surgery for movement disorders: implications for anaesthesia].

Functional surgery for movement disorders is a recent stereotactic neurosurgical operation, restricted yet to patients with advanced Parkinson's disease or with generalized primary dystonia. One or two electrodes are implanted in the basal ganglia, namely in the globus pallidus pars interna or in the subthalamic nucleus, to realize a deep brain stimulation at high frequency. While this approach needs additional data to demonstrate clinical benefits, first results observed after short and long-term follow up are encouraging. Perioperative problems in patients with Parkinson's disease are possible respiratory disorders, a postoperative miss in medication doses and potential drug interactions with anaesthesia. The objectives of anaesthesia will be to allow stereotactic neurosurgical procedure, to maintain the upper airway patency and to be quickly reversible.

Anesthesia↗

Computed-tomography-directed stereotaxis for movement disorder with postoperative magnetic resonance imaging confirmation.

Movement disorders may respond dramatically to properly placed lesions in thalamic and subthalamic areas. Proper location of lesions may be verified postoperatively by magnetic resonance imaging (MRI). Herein is reported a case of stereotactically placed bilateral lesions under computed tomography control using the Leksell apparatus, with successful clinical outcome, and the verification of lesion placement by subsequent MRI images.

Demyelinating Diseases↗