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Movement disorders and AIDS.

We studied seven patients with AIDS or AIDS-related complex (ARC) and movement disorders. Three had hemichorea-ballismus, two had segmental myoclonus, one had postural tremor with dystonia, and one had paroxysmal dystonia. Besides the hyperkinesias, two patients had parkinsonism, and one had cerebral Whipple's disease. In two, the movement disorder preceded other evidence of AIDS; in three others, the diagnosis of AIDS was not considered until there was a movement disorder. The movement disorders were attributed to toxoplasmosis in four patients (one confirmed at autopsy), viral encephalitis, vacuolar myelopathy, and CNS Whipple's disease.

Acquired Immunodeficiency Syndrome↗

Stereotypy and motor control: differences in the postural stability dynamics of persons with stereotyped and dyskinetic movement disorders.

We examined whether dynamic measures of postural stability differentiated persons with stereotyped movement disorder from persons with dyskinetic movement disorder. Participants from three groups (stereotypy, dyskinesia, control) were given a goal-oriented postural stability task, and performance was measured using a force platform and computerized posturographic techniques. The results showed that both movement disorder groups differed from the control group in the posture task. Further, the stereotypy and dyskinesia groups demonstrated markedly different postural movement profiles. The postural motion of the stereotypy group was characterized by greater amplitude and variability but lower complexity than the dyskinesia group. These results provide support for a motor control model of stereotypy.

Adult↗

Lesion therapy for Parkinson's disease and other movement disorders: update and controversies.

An analysis of the international literature on lesioning for movement disorders was undertaken to review lesion therapy for Parkinson's disease (PD) and other movement disorders and to highlight important controversies surrounding this surgical technique. Lesions have been placed throughout the neuraxis with varying approaches and success. Our understanding of the pathophysiological basis underlying the development of PD and other movement disorders has led to a better understanding of why lesioning certain portions of the nervous system should improve motor function. Advances in imaging technology and electrophysiological techniques used for localization of brain structures, such as microelectrode mapping, have improved the ability to accurately identify and lesion target structures deep in the brain. This improvement has led to an increase in the degree and consistency of clinical benefit. The major controversies in lesion therapy include: (1) which target for which disorder; (2) determination of the optimal lesion site and whether the external globus pallidus (GPe) should be included in the pallidotomy lesion for PD; (3) determination of the size of the lesion; (4) whether bilateral lesions can be placed without the high incidence of side effects reported by some investigators; (5) whether microelectrodes aid in the ability to improve clinical outcomes or increase the risk of side effects by making multiple microelectrode penetrations; (6) whether the subthalamic nucleus (STN) should be explored further as a lesioning target; and (7) whether lesioning should be abandoned entirely in favor of deep brain stimulation (DBS). Many important questions and controversies regarding lesion therapy remain unanswered. It is unlikely given the pro-DBS environment that these questions will be answered in the near future. We should, however, be careful not to abandon an effective therapy before fully exploring through randomized trials the relative effect of different surgical approaches for the treatment of patients with movement disorders.

Electric Stimulation Therapy↗

Neurobehavioral aspects of movement disorders.

Cognitive, behavioral, affective, and psychiatric symptoms occur in almost all movement disorders. Diagnosis and management of movement disorders depends critically on an understanding of these neurobehavioral symptoms. This article reviews the neurobehavioral aspects of two representative movement disorders; Parkinson's disease and Huntington's disease.

Cognition Disorders↗

Management of movement disorders. An overview.

Functional neurosurgery has been involved with management of movement disorders since the first days of stereotactic surgery. Those movement disorders that can be managed surgically are reviewed, along with general criteria for patient selection. A flow sheet of surgically managed movement disorders is supplemented by descriptions of how patients are assigned to various treatment categories and the various treatments available.

Humans↗

Movement disorders induced by dopamine blocking agents.

Movement disorders are frequently due to dopamine blocking agents (DBAs) prescribed for psychiatric illnesses. DBAs are sometimes also prescribed inappropriately for other maladies. These drugs can cause a wide variety of involuntary movements, sometimes in combination. When these appear in the setting of psychiatric illnesses, the association is easy to recognize but the treatment remains a difficult problem. However, in other situations the diagnosis may be missed for extended periods of time, resulting in unnecessary diagnostic work-up and inappropriate therapy. The diagnosis depends on a compulsive drug history not only from the patient but also from patient's family, primary physician, and the pharmacist. The treatment options include removal of the offending agent or substitution with an atypical neuroleptic. Symptomatic treatments are varied and have an inconsistent effect on the movement disorders. Prevention remains the most important strategy.

Acute Disease↗

Movement disorders in multiple sclerosis.

Movement disorders other than cerebellar tremor are rare clinical manifestations of multiple sclerosis (MS). Two cases of parkinsonism and a case of chorea associated with MS are reported, and the literature is reviewed.

Adult↗

A defect in pyruvate decarboxylase in a child with an intermittent movement disorder.

A patient with an intermittent movement disorder has been found to have an inherited defect in pyruvate decarboxylase ((2-oxo-acid carboxy-lyase, E.C. 4.1.1.1.). The patient is a 9 yr old boy who since infancy has had repeated episodes of a combined cerebellar and choreoathetoid movement disorder. He has an elevated level of pyruvic acid in his blood, an elevated urinary alanine content, and less marked elevations in blood alanine and lactate. Methods were developed to study his metabolic abnormality in dilute suspensions of white blood cells and cultured skin fibroblasts, as well as in cell-free sonicates of fibroblasts. Oxidation of pyruvic acid-1-(14)C and pyruvic acid-2-(14)C by his cells and pyruvate decarboxylase activity in sonicates of his cells were less than 20% of those in cells from control subjects. Oxidation of glutamic acid-U-(14)C, acetate-1-(14)C, and palmitate-1-(14)C was normal, as was incorporation of alanine-U-(14)C into protein. The rate of oxidation of pyruvic acid by the father's cells and the activity of pyruvate decarboxylase in the father's sonicated fibroblasts were intermediate between those of the patient and those of controls. Values for the mother were at or just below the lower limits of the ranges in controls. Kinetic data suggested the posibility of several forms of pyruvate decarboxylase in this family. Possible mechanisms relating the chemical abnormality and the clinical symptoms in this patient are discussed.

Alanine↗

Movement disorders in people with Parkinson disease: a model for physical therapy.

People who are diagnosed with idiopathic Parkinson disease (PD) experience movement disorders that, if not managed, can lead to considerable disability. The premise of this perspective is that physical therapy for people with PD relies on clinicians having: (1) up-to-date knowledge of the pathogenesis of movement disorders, (2) the ability to recognize common movement disorders in people with PD, (3) the ability to implement a basic management plan according to a person's stage of disability, and (4) problem-solving skills that enable treatment plans to be tailored to individual needs. This article will present a model of physical therapy management for people with idiopathic PD based on contemporary knowledge of the pathogenesis of movement disorders in basal ganglia disease as well as a review of the evidence for physical therapy interventions. The model advocates a task-specific approach to training, with emphasis on treating people with PD-related movement disorders such as hypokinesia and postural instability within the context of functional tasks of everyday living such as walking, turning over in bed, and manipulating objects. The effects of medication, cognitive impairment, the environment, and coexisting medical conditions are also taken into consideration. An argument is put forward that clinicians need to identify core elements of physical therapy training that apply to all people with PD as well as elements specific to the needs of each individual. A case history is used to illustrate how physical therapy treatment is regularly reviewed and adjusted according to the changing constellation of movement disorders that present as the disease progresses.

Accidental Falls↗

Evaluation of clinical diagnostic criteria for psychogenic movement disorders.

Previous studies showed that there are certain features suggestive of a psychogenic disorder, and Fahn and Williams proposed criteria for psychogenic movement disorders. Data on the sensitivity and specificity of these criteria are lacking. We expanded on the Fahn and Williams criteria to create a new set of diagnostic criteria. We retrospectively reviewed 79 patients in a movement disorders specialty clinic. We applied the proposed diagnostic criteria to both cases and controls and analyzed sensitivity and specificity. The diagnostic criteria correctly identified "clinically probable" (or better) psychogenic movement disorders with a sensitivity of 83% and specificity of 100%. For "clinically possible" or greater, sensitivity was 97% and specificity was 96%. In addition, a significantly higher proportion of patients with psychogenic disease were female and reported either a family history of neurological disease or other exposure to neurological disease (P = 0.001 and 0.01, respectively). The diagnostic criteria are simple to apply and have high sensitivity and specificity for psychogenic movement disorders. Also, we report a correlation between previous exposure to a disease model and psychogenic disease.

Adult↗

Copper-64 metabolism in two patients with non-Wilsonian movement disorders and copper deficiency.

Copper-64 studies are presented of 2 patients with non-Wilsonian movement disorder and with abnormal copper handling. Both patients differed from the usual phenotype of non-Wilsonian low copper movement disorder as they had choreiform movement disorders with an onset in the first decade; one patient lacked significant intellectual impairment. Both patients had reduced serum total copper and marginal free copper and caeruloplasmin levels, and both patients were capable of incorporating 64Cu2+ into caeruloplasmin but the second case did so at markedly reduced level. Both showed slightly increased basal and stimulated urinary copper loss compared to normal controls with the rate in patient 1 being capable of leading to copper depletion. Liver copper content was normal in both cases. These 2 patients add to the reports of cases with copper deficiency and movement disorder in whom copper chelation therapy is unlikely to be beneficial.

Adult↗

A forward look at therapy for pediatric movement disorders.

The mainstay of current therapy for pediatric movement disorders is oral symptomatic medication, unless a reversible etiology can be found. However, this approach is apt to pale in comparison with innovative strategies on the clinical forefront. Classical pharmacotherapy is restricted by the blood-brain barrier, which prevents access to the brain of potentially therapeutic molecules. Recent developments in molecular biotechnology include antibody-mediated drug release, feedback-responsive delivery systems, carrier-mediated transport, microspheres composed of polymers and liposomes, permeabilizers, and selective delivery to localized sites and vectors. Neuroprotective strategies for delivering neurotrophic factors and antiapoptotic and antioxidant molecules in neurodegenerative disorders are currently under study in clinical trials. Stem cell transplantation has great potential for tissue engineering and also as a carrier for gene therapy, although its use raises complex societal issues. These approaches, together with a plethora of transgenic knockout animal models of neurodegenerative disorders, offer real promise for a previously untreatable group of movement disorders.

Animals↗

A familial awake movement disorder mimicking restless legs in a sleep apnea patient.

We report on a patient with sleep apnea and an unusual familial movement disorder. The movements were present only during wakefulness and nocturnal arousals caused by disordered breathing. A 27-year-old obese man was referred with sleep onset insomnia, symptoms suggesting restless legs syndrome, daytime sleepiness, loud snoring and awakening with choking sensations. He was proven to have obstructive sleep apnea (apnea hypopnea index = 60.6). He also had a daytime movement disorder that was characterized by almost continuous stereotypic tapping of one or both legs. The movements were suppressible and not associated with any unpleasant or abnormal leg sensation. Virtually identical movements were present in three generations of his family. The severity of the movements did not worsen late in the day or with supine posturing. The nocturnal movements, consisting of a visible shaking of one or both legs, occurred only during arousals secondary to the apnea, had a mean duration of 5.7 +/- 3.0 (standard deviation) seconds and could not be defined as periodic limb movements in sleep (PLMS). Successful treatment of apnea by nasal continuous positive airway pressure dramatically reduced the movements during sleep (from 88.2 to 1.9 per hour). The clinical significance and the mechanism of this movement disorder is unknown. We discuss the features inconsistent with restless legs syndrome and consider other possible phenomenology, including akathisia. We conclude that this patient may have a previously unreported familial movement disorder and in addition developed the sleep apnea syndrome related to obesity.

Adult↗

[Sleep and movement disorders].

The three different states of being (wakefulness, NREM and REM sleep) are associated with profound neurophysiological and neurochemical changes in the brain. These changes explain the existence of movement disorders appearing only or preferentially during sleep, and the effects of sleep on movement disorders. Sleep-related movement disorders are of clinical relevance for multiple reasons: 1) high frequency (e.g. restless legs syndrome (RLS)); 2) diagnostic relevance (e.g. REM sleep behavior disorder (RBD) as first manifestation of Parkinson disorder); 3) diagnostic uncertainty (e.g. parasomnias vs nocturnal epilepsy); 4) association with injuries (e.g. RBD, sleepwalking), sleep disruption/daytime sleepiness (e.g. RLS), and psycho-social burden (e.g. enuresis); 5) requirement of specific treatments (e.g. nocturnal epilepsy, stridor, RBD). This article gives an overview on clinical manifestations, pathophysiology, work-up and treatment of sleep-related movement disorders (e.g. RLS, bruxism), parasomnias (e.g. sleepwalking, RBD), sleep-related epilepsies, and on sleep-associated manifestations of movement disorders (e.g. Parkinson disease, multiple system atrophy).

Adult↗

Movement disorders in Friedreich's ataxia.

Since the discovery of the gene mutation causing Friedreich's ataxia (FA), the rich spectrum of clinical manifestations of this autosomal recessive disorder is being increasingly recognized. Movement disorders besides ataxia, however, have not been fully characterized in patients with FA. We describe here two young male patients who, in addition to progressive ataxia, kinetic tremor and other typical features of FA, also manifest axial and limb dystonia. The primary purpose of this report is to draw attention to the broad spectrum of hyperkinetic movement disorders that can present as or be associated with FA.

Adolescent↗

Prevalence of movement disorders in men and women aged 50-89 years (Bruneck Study cohort): a population-based study.

BACKGROUND: There is emerging awareness that movement disorders rank among the most common neurological diseases. However, the overall burden of these disorders in the general community is not well defined. We sought to assess the prevalence of all common categories of movement disorders in a population, accounting for sex differences and age trends. METHODS: As part of an ongoing prospective population-based study of carotid atherosclerosis and stroke risk (the Bruneck Study), a total of 706 men and women aged 50-89 years underwent a thorough neurological assessment. The diagnosis of movement disorders and ratings for disease severity were based on standard criteria and scales. Prevalences were estimated from logistic regression models (regression-smoothed rates) and standardised to the age and sex structure of the general community. FINDINGS: The prevalence of all common categories of movement disorders was 28.0% (95% CI 25.9-30.1). Proportions in men (27.6% [95% CI 24.5-30.7]) and women (28.3% [25.5-31.2]) were closely similar and sharply increased with age (from 18.5% [15.0-22.0] in 50-59-year olds to 51.3% [44.9-57.7] in 80-89-year olds). Almost half of all patients (90/214) had moderate-to-severe disease expression, but only 7.0% (15/214) received standard drug treatment. Prevalence of tremor was 14.5%, followed by restless legs syndrome (10.8%), parkinsonism (7%), primary dystonia and secondary dystonia (1.8%), and chorea and tics (<1% each). A fifth of all movement disorders were diagnosed to be probably drug-induced. INTERPRETATION: There is a high prevalence of and substantial under-recognition and under-treatment of movement disorders in the general community.

Age Factors↗

Profile of patients enrolled in a new movement disorder clinic.

To assess the need for a regional expertise in movement disorders, the numbers of patients, clinic visits, and medication changes for a new movement disorder clinic were recorded. During 3 1/2 years, 355 patients were seen, with 1,329 clinic visits. Idiopathic Parkinson's disease was the most common diagnosis, comprising 36% of the population, followed by dystonia (17%), tremor (12%), parkinsonism (i.e., Parkinson's plus syndromes, drug-induced parkinsonism, etc.) (10%), chorea (10%), Tourette's syndrome (6.5%), and tardive dyskinesia (3.4%). Distribution of follow-up visits was similar, with Parkinson's disease (52%) being most frequent and Tourette's syndrome (3.1%) least frequent. The relative utilization of medical care by each patient group was assessed by determining the number of medication changes and the number of clinic visits per follow-up year. No differences in these measures were found using a one-way analysis of variance. Of the Parkinson's disease patients, 67% had Hoehn and Yahr stages III-IV and 77% of the clinic visits were made by this subgroup. When considered in light of the prevalence of each of the diseases, these data show a need for an expertise in movement disorders for a population base of the size we have served.

Cross-Sectional Studies↗

Prevalence of neuroleptic-induced movement disorders in chronic schizophrenia inpatients.

OBJECTIVE: Since most of the world's schizophrenia patients are treated with conventional antipsychotics, the authors evaluated various methods for establishing the prevalence of neuroleptic-induced movement disorders in these patients. METHOD: DSM-IV criteria and established score thresholds on a movement disorder rating scale were used to identify cases of neuroleptic-induced movement disorder in a representative Estonian patient sample of 99 chronic institutionalized schizophrenia patients, 18-65 years old, treated with conventional neuroleptics (79.8%) or clozapine (20.2%). RESULTS: Neuroleptic-induced movement disorders according to DSM-IV criteria were found in 61.6% of the group: 31.3% had neuroleptic-induced akathisia, 23.2% had neuroleptic-induced parkinsonism, and 32.3% had neuroleptic-induced tardive dyskinesia. Prevalence rates for akathisia and tardive dyskinesia were similar when either DSM-IV criteria or rating scale scores were used, but the prevalence rate for parkinsonism was much lower per DSM-IV criteria than according to rating scale score. CONCLUSIONS: Nearly two-thirds of chronic schizophrenia patients suffered from a neuroleptic-induced movement disorder. Globally, extrapyramidal adverse effects still impose a huge burden on the majority of neuroleptic-treated individuals with schizophrenia. The discrepancy between the standard identification methods for neuroleptic-induced movement disorder indicate the need for further research.

Adolescent↗