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PubMed2004

Historical perspective on movement disorders.

Before atypical antipsychotics were developed, neuroleptics given to patients for the treatment of psychosis often caused movement disorders. Although the role of neuroleptics in the development of tardive dyskinesia was never certain, this adverse effect was of great concern to physicians because its effects could be irreversible and devastating to the patient. It is important to understand whether involuntary movement disorders are an intrinsic part of schizophrenia, because if so, then a certain percentage of patients will develop the dyskinetic syndromes whether they are treated or not. To uncover the role of antipsychotic medications in tardive dyskinesia, it is necessary to examine the descriptions of abnormal movements made by those who were first researching schizophrenia, as well as modern descriptions of neuroleptic-naïve individuals with schizophrenia. The physicians who initially described the syndrome of tardive dyskinesia had observed pre-neuroleptic schizophrenia first hand and saw a difference in the movements of treated and untreated patients. Nevertheless, the idea of a chronic movement disorder caused by treatment with neuroleptics would become controversial for many years. With the development of the atypical antipsychotics, the incidence and prevalence of tardive dyskinesia have dropped remarkably, suggesting that psychosis, its treatment, and dyskinesias are not inextricably linked.

Antipsychotic Agents↗

PubMed2006

Periodic limb movement disorder in children.

Periodic limb movement disorder (PLMD) has been reported in children and is more prevalent in certain conditions such as attention deficit hyperactivity disorder. The International Restless Legs Syndrome Group has recently developed the criteria for diagnosis of PLMD in children. These criteria include both specific clinical features and polysomnographic findings. Exclusion of other causes especially sleep-disordered breathing is essential. Several medications including benzodiazepine and dopaminergic medication have been used in children, although these medications have not been adequately studied in this age group. Some PLMD children have evidence of low iron storage and response favorably to iron therapy.

Child↗

PubMed1997

[Neurosurgical treatment of Parkinson disease and other movement disorders].

The surgical treatment of movement disorders has attracted widespread attention in the past few years. Better understanding of pathophysiological mechanisms, and also the refinement of neurosurgical, neuroradiological and neurophysiological methods and techniques have contributed to the redefinition of surgical treatment. With appropriate selection criteria, symptomatic and functional benefit can be achieved in the majority of patients. Functional neurosurgical procedures nowadays are performed most frequently in patients with Parkinson's disease or cervical dystonia. Functional neurosurgery today has the choice of a variety of different surgical options. The most appropriate operative technique is chosen with regard of the clinical presentation of the individual patient. The different operative procedures and the postoperative results are briefly described.

Humans↗

PubMed2006

Parkinson's disease and other basal ganglia or movement disorders in a large nationwide cohort of Swedish welders.

INTRODUCTION: Although it has been hypothesised that metal welding and flame cutting are associated with an increased risk for Parkinson's disease due to manganese released in the welding fume, few rigorous cohort studies have evaluated this risk. METHODS: The authors examined the relation between employment as a welder and all basal ganglia and movement disorders (ICD-10, G20-26) in Sweden using nationwide and population based registers. All men recorded as welders or flame cutters (n = 49,488) in the 1960 or 1970 Swedish National Census were identified and their rates of specific basal ganglia and movement disorders between 1964 and 2003 were compared with those in an age and geographical area matched general population comparison cohort of gainfully employed men (n = 489,572). RESULTS: The overall rate for basal ganglia and movement disorders combined was similar for the welders and flame cutters compared with the general population (adjusted rate ratio (aRR) = 0.91 (95% CI 0.81 to 1.01). Similarly, the rate ratio for PD was 0.89 (95% CI 0.79 to 0.99). Adjusted rate ratios for other individual basal ganglia and movement disorders were also not significantly increased or decreased. Further analyses of Parkinson's disease by attained age, time period of follow up, geographical area of residency, and educational level revealed no significant differences between the welders and the general population. Rates for Parkinson's disease among welders in shipyards, where exposures to welding fumes are higher, were also similar to the general population (aRR = 0.95; 95% CI 0.70 to 1.28). CONCLUSION: This nationwide record linkage study offers no support for a relation between welding and Parkinson's disease or any other specific basal ganglia and movement disorders.

Adult↗

PubMed1997

Diagnosing rhythmic movement disorder with video-polysomnography.

We evaluated the utility of accurate clinical and electrophysiologic characterization in the diagnosis of the rhythmic movement disorder. Seven children with an age range of 1-12 years, referred for evaluation of relatively violent nocturnal behaviors, were clinically assessed during split-screen, video-polysomnographic monitoring sessions, as they experienced unusual nocturnus movements. Differential diagnoses included self-injurious waking behaviors, seizures, and parasomnias such as somnambulism (sleepwalking), pavor nocturnus (night terrors), and the rhythmic movement disorder (headbanging, bodyrocking, and legbanging). The character of movements, level of responsiveness, and electrophysiologic stage of sleep was determined during typical spells. In all the subjects experienced 37 periods of headbanging, bodyrocking, and legbanging that were strongly associated with stage 2 non-rapid eye movement sleep and K-complexes. The patients were unresponsive during and amnestic for the events. Because the differential for the rhythmic movement disorder includes a large number of disorders associated with abnormal and at times violent nocturnal movements, diagnosis can be greatly enhanced by documenting suspected nocturnal behaviors with thorough clinical assessment during split-screen, video-polysomnographic analysis.

Attention Deficit Disorder with Hyperactivity↗

PubMed2005

Management of referred deep brain stimulation failures: a retrospective analysis from 2 movement disorders centers.

BACKGROUND: Since the Food and Drug Administration approved DBS, there has been a surge in the number of centers providing the procedure. There is currently no consensus regarding appropriate screening procedures, necessary training of individuals providing the therapy, the need for an interdisciplinary team, or guidelines for the management of complications. An increasing number of patients come to experienced DBS centers after unsatisfactory results from DBS surgery. An attempt is made herein to evaluate the reasons for DBS failure in a series of such patients and to make recommendations to improve overall DBS outcomes. OBJECTIVE: To improve outcomes of deep brain stimulation (DBS) surgery by analyzing a series of patients who had suboptimal results from DBS. METHODS: Forty-one consecutive patients complaining of suboptimal results from DBS surgery came to the University of Florida Movement Disorders Center, or to Beth Israel Movement Disorders Center, over a 24-month period. All patients had undergone implantation of DBS devices at outside medical centers. Each patient was evaluated by a movement disorders neurologist, and the complete medical record was reviewed. The DBS device for each patient was interrogated for adverse effects and programmed for maximal benefit. Postoperative imaging studies were evaluated whenever possible. RESULTS: The average age of patients was 63.4 years (range, 49-84 years). The indication for surgery (by record review) included 9 patients with essential tremor, 31 with Parkinson disease, and 1 with dystonia. The diagnoses after referral examination included 5 with essential tremor, 26 with Parkinson disease, 3 with Parkinson disease and dementia, 1 with Parkinson disease and essential tremor, 1 with corticobasal degeneration, 1 with dystonia, 2 with multiple system atrophy, 1 with progressive supranuclear palsy, and 1 with myoclonus. Issues related to inadequate preoperative screening: Thirty (73%) of 41 patients saw a movement disorders specialist prior to DBS implantation. Fourteen (34%) patients had neuropsychological testing, 4 (10%) did not have testing, and in 23 cases (56%), it could not be determined whether or not they were tested. Five (12%) of 41 patients had an inadequate medication trial, and 5 patients (12%) had significant cognitive dysfunction prior to their DBS implantation. Surgical and device-related complications: Nineteen (46%) of 41 patients had suboptimally placed electrodes. Seven electrodes (17%) were replaced with improvement. Three patients' devices had failed due to end of battery life, 2 had infections, and 1 had a fractured lead. Programming and medication adjustments: Seven (17%) of 41 patients had no or poor access to programming. Two patients (5%) moved, and 2 physicians (5%) moved, creating issues with access to care. Eight patients (20%) required local follow-up (they flew to remote centers to have the surgery performed). Fifteen patients (37%) were inadequately programmed and improved significantly with reprogramming. Six patients (15%) experienced partial improvement with reprogramming, and 21 patients (51%) failed to improve despite extensive reprogramming. Thirty patients (73%) benefited from medication changes, 4 (10%) had antidepressants added to their regimens, and 1 (2%) had donepezil hydrochloride added. One patient's carbidopa/levodopa (2%) was restarted after complete discontinuation. OUTCOMES: With the various postoperative interventions described, 21 (51%) of 41 patients had good outcomes, 6 (15%) had modest clinical improvement, and 14 (34%) did not improve. CONCLUSIONS: With appropriate intervention, 51% of patients who complained of "failed" DBS procedures ultimately had good outcomes. Thirty-four percent of these patients had persistently poor outcomes despite maximal intervention. This case series provides important insights into reasons for "DBS failure" and proposes strategies to manage patients with DBS more effectively.

Aged↗

PubMed2005

Computer skills in patients with movement disorders.

BACKGROUND: Electronic communication is important in healthcare, but the level of computer proficiency among patients with neurological disorders is unknown. OBJECTIVE: This study sought to determine the proportion of a movement disorder clinic population that was able to perform basic computer skills, and the effect of specific cognitive and motor features on computer proficiency. METHODS: One hundred and four movement disorder patients participated. Seventy-four completed both paper and computerized questionnaires to evaluate data entry skills and thirty subjects completed paper questionnaires only. Basic e-mail messaging and Internet skills were evaluated. Demographic information, Mini-Mental Status Examination (MMSE) score, and Hoehn and Yahr stage were assessed. RESULTS: Ninety-six percent of subjects successfully completed computerized data entry tasks, and over 70% completed e-mail and Internet tasks. Computer data entry had an average accuracy of nearly 95% when compared to paper data entry. Poorer performance on computer tasks was associated with older age, less education, and cognitive impairment. Computer performance was reduced in subjects with a history of parkinsonism and when both tremor and dyskinesia were present during task performance. Nearly three-quarters of subjects have access to a computer. Subjects who completed the paper questionnaire but refused to complete the computer questionnaire were older, less educated and more cognitively impaired. CONCLUSION: The majority of patients visiting a tertiary movement disorders center were able to perform computer data entry, e-mail messaging and Internet usage. These results reinforce the potential value of electronic communication and information systems in neurology practice.

Aged↗

PubMed2005

A reversible generalized movement disorder in critically ill children with cancer.

INTRODUCTION: Some children treated for cancer become critically ill because of immune suppression and sepsis requiring prolonged intensive care support and assisted ventilation. METHODS: Over a 3-year-period, we have identified six children (four with brain tumors) who developed a generalized movement disorder during a protracted intensive care unit stay. Median age was 2 years (range 1-6 years). Movement disorder developed while receiving multiple medications. RESULTS: Sedation was achieved with midazolam and opioid infusions. Dystonic posturing of limbs, jaw movements, tongue thrusting, and intermittent eye deviations were present in all. Movements increased if the child was stimulated and an electroencephalogram performed in five children excluded seizures. CONCLUSIONS: This movement disorder should be differentiated from seizures to prevent inappropriate treatment. Exacerbation with stimulation is a clinical clue to the correct diagnosis and an electroencephalogram can help differentiate this movement disorder from seizures.

Brain Diseases↗

PubMed2005

Intraoperative monitoring of motor symptoms using surface electromyography during stereotactic surgery for movement disorders.

The authors present practical evidence for the usefulness of intraoperative monitoring with surface electromyograms (sEMGs) from the affected muscles to assist electrode implantation and lesioning in patients with movement disorders. In 22 consecutive patients with various movement disorders, sEMGs were monitored in selected muscles during stereotactic surgery that involved either lesioning or electrode implantation. The electromyograms related to major motor symptoms such as tremor, rigidity, myoclonus, dystonia, and chorea were monitored and characterized on-line by both amplitude and frequency. Major motor symptoms were revealed by sEMGs recorded from the affected muscles. Tremor manifested as highly rhythmic bursts with a narrow frequency band; dyskinesias and chorea appeared as irregularly repeated bursts within a broad frequency range of 1 to 5 Hz; and rigidity and dystonia appeared as sustained high-frequency activity and co-contraction between antagonist muscles. The results suggest that intraoperative monitoring of sEMGs could help to functionally refine and confirm target localization. Surface EMGs could be used (1) as reference signals of the motor symptoms so that other signals, such as the oscillatory local field potentials simultaneously recorded via the implanted electrodes, could be correlated with the sEMGs and used to fine-tune or confirm the target localization; (2) to quantify the effects of acute electrical stimulation on the motor symptoms; and (3) to sensitively detect unwanted capsular responses induced by direct stimulation of the internal capsule. The authors conclude that intraoperative monitoring of sEMGs of the affected muscles of patients with movement disorders during stereotactic surgery provides sensitive and quantitative information that can contribute to improved electrode or lesion placement.

Electric Stimulation↗

PubMed1990

Psychogenic dyskinesias in patients with organic movement disorders.

The association of psychogenic neurological features with organic neurological disease is commonly acknowledged. However, the occurrence of psychogenic dyskinesias in patients with underlying organic movement disorders is not well recognized. Six cases of psychogenic dyskinesias complicating preexisting organic movement disorders are described. This possibility must be carefully considered and excluded before an unusual movement disorder can be considered entirely psychogenic.

Adult↗

PubMedDate not supplied

Psychiatric outcome in patients with a psychogenic movement disorder: a prospective study.

OBJECTIVE: To assess psychiatric outcome in patients with an established diagnosis of a hyperkinetic (i.e. tremor, dystonia, myoclonus) psychogenic movement disorder. BACKGROUND: Longitudinal studies of patients with psychogenic movement disorders (PMD) have to date suffered from small sample size, lack of sample homogeneity (psychogenic movements grouped with other somatoform disorders, mixed hyper and hypokinetic movements), the absence of structured psychiatric interviews, and a failure to adequately address the extent of psychiatric co-morbidity by adopting a hierarchical approach to diagnosis. METHODS: A sample of 88 patients with documented PMD according to the criteria of Fahn and Williams were followed up on average 3.2 years (sd = 2.2; range 1-7 years) after first being assessed at a tertiary referral clinic for patients with movement disorders. The detailed psychiatric assessment included the Structured Clinical Interview for Axis (SCID-I) and Axis II (SCID-II) DSM-IV disorders which generated diagnoses with respect to major mental illness (SCID-I) and personality disorders (SCID-II) respectively. RESULTS: Of 88 subjects initially seen in clinic, three had died (one by suicide), two were in nursing homes (Alzheimers disease, terminal cancer) and three had emigrated. Of the remaining 80 subjects, 42 (52.5%) agreed to be interviewed. There were no demographic or illness-related differences between those who agreed or refused participation. At follow-up, the mean age of subjects was 48.6 (13.3) years, 62.7% were female and 75% were unemployed. An Axis I diagnosis of mental illness was made in 40 (95.3%) subjects. The PMD had remitted in four subjects, but had been replaced in two of them by a different mental disorder. Thirty-eight percent of subjects with PMD had developed additional unexplained medical symptoms at follow-up. Point and lifetime prevalence rates for other Axis I diagnoses were: major depression 19.1% and 42.9%; anxiety disorders 38.2% and 61.9%; co-morbid major depression and anxiety disorders 11.9% and 28.6%. Personality disorders were present in 45% of the sample. No subject viewed their PMD as primarily psychiatric in origin. Poor outcome with respect to the abnormal movements was associated with long duration of symptoms, insidious onset of movements and psychiatric co-morbidity on Axis I diagnoses. CONCLUSIONS: Follow-up data of patients with PMD revealed a persistence in abnormal movements in more than 90% of subjects. Prevalence rates of mental illness in excess of those found in the general population and in neurologic disorders plus an inability to acknowledge the essentially psychologic nature of their condition characterized the outcome picture and carries a poor longer term prognosis.

Female↗

PubMed1997

Concordance of common movement disorders among familial cases.

Multicase families are frequently utilized in studies of movement disorders (MDs). We report families with two or more MD cases seen at the Movement Disorder Clinic, Saskatoon (MDCS). In 30% of the MD probands, there was either a history or documentation of at least one secondary MD case in the family. Only those MD cases that were seen at the MDCS were considered in this study. A total of 56 probands and 77 secondary MD cases were seen at the MDCS between 1968 and 1996. In 24 (31.2%) of the secondary cases, the diagnosis was different from that in the proband. In 46 families (82%), only one secondary case was seen, and the diagnosis was concordant with the proband in 71.7%. In the remaining 10 families with two or more secondary cases, the diagnosis was concordant in 64.5% of cases. The largest subgroup was Parkinson's disease (PD)--40 probands and 53 secondary cases. of these secondary cases, 73.6% had PD. The concordance rate was 91% in essential tremor, but only 12.5% if the proband had essential tremor + parkinsonism. Considering that a large proportion of secondary cases have a diagnosis discordant with the proband, we recommend that, whenever possible, MD diagnosis in secondary cases be based on clinical assessment.

Age of Onset↗

PubMed2000

Physiology of hypokinetic and hyperkinetic movement disorders: model for dyskinesia.

Although the basal ganglia have been implicated in the development of movement disorders since the 1940s, the exact role played by these structures has remained elusive. The development of the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-monkey model of parkinsonism, and the recent resurgence of surgical therapy for the treatment of hypokinetic and hyperkinetic movement disorders has, however, led to an improved understanding of the pathophysiological mechanisms that underlie their development. In this article, we review the functional organization and examine the changes in neuronal activity that occur in the basal ganglia thalamocortical 'motor' circuit in these disorders. An alternative to the classic 'rate' model for Parkinson's disease is presented that incorporates the observed changes in neuronal activity, as well as additional neuronal pathways that contribute to these changes. Based on studies in animal models and humans with hyperkinetic movement disorders, it is postulated that dyskinesias develop as the result of a combination of excessive reductions in the mean discharge rate, altered patterns and increased synchronization of neurons in the internal segment of the globus pallidus. It is further postulated that the particular type of involuntary movement which develops also depends on the relative change in neuronal activity in the direct, indirect and alternative pathways. Support for these postulates is examined, and models for drug-induced dyskinesia, hemiballismus and dystonia are proposed.

Animals↗

PubMed1993

Pathological assessment of movement disorders: requirements for documentation in brain banks.

The general methodological requirements and principal morphologic hallmarks for the post mortem assessment of the major types of movement disorders are critically reviewed. These data may enable Brain Banks to classify movement disorders according to current clinicopathological diagnostic criteria. Comprehensive clinical assessment and accurate neuropathological examination using adequate methods are required for collection of and research on tissues from patients with movement disorders.

Brain Diseases↗

PubMed2000

Restorative neurology in movement disorders.

Cell replacement for restoration of neurological functions in patients with movement disorders has been investigated for more than 15 years. Initial attempts used autologous adrenal medulla grafts implanted into the denervated striatum of patients with Parkinson's disease (PD). This approach was soon abandoned in favor of intrastriatal implantation of human embryonic mesencephalic tissue, rich in dopaminergic neurons. Available data from grafted PD patients show long-term (up to 10 years) graft survival and clinical benefits. The pattern and magnitude of symptomatic relief following transplantation, however, are incomplete and the outcome varies among patients. The need for large amounts of human embryonic tissue has to be circumvented and a better understanding of the relationship between graft placement and symptomatic recovery is necessary before this procedure can be offered to larger groups of patients. Clinical trials in Huntington's disease have so far shown inconclusive results. Neural cell replacement therapy is still an experimental procedure, but has the potential to become a future restorative treatment in PD and other movement disorders.

Adrenal Medulla↗

PubMed2003

A novel quality of life instrument for deep brain stimulation in movement disorders.

OBJECTIVE: To develop a short instrument to examine quality of life (QoL) which specifically addresses patients with movement disorders treated by deep brain stimulation (DBS). DESIGN: The instrument was developed within an existing concept of a modular questionnaire (questions on life satisfaction: "general life satisfaction" QLS(M)-A, and "satisfaction with health" QLS(M)-G), in which each item is weighted according to its relative importance to the individual. METHODS: Items were generated by interviews with 20 DBS patients, followed by item reduction and scale generation, factor analysis to determine relevant and final questionnaire items, estimation of reliability, and validation based on the medical outcome study 36 item short form health survey (SF-36) and the EuroQol (EQ-5D) (data from 152 patients with Parkinson's disease, essential tremor, or idiopathic torsion dystonia, including 75 patients with DBS). RESULTS: Initial questionnaires were reduced to 12 items for a "movement disorder module" (QLS(M)-MD), and five items for a "deep brain stimulation module" (QLS(M)-DBS). Psychometric analysis revealed Cronbach's alpha values of of 0.87 and 0.73, and satisfactory correlation coefficients for convergent validity with SF-36 and EQ-5D. CONCLUSIONS: QLS(M)-MD and QLS(M)-DBS can evaluate quality of life aspects of DBS in movement disorders. Psychometric evaluation showed the questionnaires to be reliable, valid, and well accepted by the patients.

Activities of Daily Living↗

PubMed2003

Paroxysmal movement disorders in severe myoclonic epilepsy in infancy.

We report on the electroclinical findings and the results of a molecular genetic study of a patient with typical severe myoclonic epilepsy in infancy (TSME) and three with borderline SME (BSME) who showed paroxysmal movement disorders, such as choreoathetosis, dystonia and ballismus, during their clinical course. BSME was defined as a clinical entity that shares common characteristics with TSME but lacks myoclonic seizures associated with ictal EEG changes. When the paroxysmal movement disorders were first observed, all the patients in this study were being treated with polytherapy including phenytoin (PHT), and these abnormal movements disappeared when PHT was discontinued or reduced. However, on other occasions, two of our cases also showed the same abnormal movements even when not being treated with PHT. One patient with TSME and two of the three patients with BSME had SCN1A gene mutations that lead to truncation of the associated protein. We conclude that paroxysmal movement disorders seen in SME patients were closely related to their AED therapy, especially the use of PHT. It is thought that patients with both TSME and BSME have some predisposition toward paroxysmal movement disorders, and that this predisposition is partly related to sodium channel dysfunction, although some other factors might influence the occurrence of this phenomenon.

Adolescent↗

PubMed2005

Neuropsychiatric movement disorders following streptococcal infection.

The aim of this study was to describe post-streptococcal movement disorders that form part of the acute rheumatic fever complex. The clinical records of patients diagnosed with Sydenham's chorea were analyzed retrospectively to investigate epidemiology, the significance of socioeconomic deprivation, clinical manifestations, treatments, outcomes, long-term morbidity, and disease evolution. Forty-two patients (21 males, 21 females) were diagnosed with Sydenham's chorea. The median presentation age was 9 years 8 months (range 3y 5mo to 13y 2mo). Nineteen patients were of indigenous African ancestry; 23 were of mixed ancestry. All patients lived in poverty and had poor access to medical care. Twelve of the total group had disabling symptoms for longer than 2 years; six of these patients developed paediatric autoimmune neuropsychiatric disorder associated with Streptococcus (Paediatric autoimmune neuropsychiatric disorder associated with Streptococcus [PANDAS]), five Tourette syndrome (TS), and one learning difficulties. Poor outcome was significantly more prevalent in patients of mixed ancestry, in those with a positive family history, previous behavioural problems, or a failure to complete 10 days of penicillin and 'bed-rest'/hospitalization. Sydenham's chorea is one manifestation of post-streptococcal neuropsychiatric movement disorders. This study demonstrates that patients can present with one diagnosis and evolve other neuropsychiatric conditions such as TS and PANDAS. In the South African context, it is important to delineate neuropsychiatric movement disorders associated with streptococcal infections. The potential genetic susceptibility should be explored.

Adolescent↗