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PubMed1991

The scintigraphic evaluation of Huntington's disease and other movement disorders using single photon emission computed tomography perfusion brain scans.

The increasing availability of single-photon emission computed tomography (SPECT) perfusion brain scans has led to the investigation of a variety of neuropsychiatric conditions including the movement disorders such as Huntington's and Parkinson's disease. In general, observers have noted that Huntington patients have bilaterally decreased uptake of technetium 99m HM-PAO and iodine 123 IMP in the basal ganglia regions involving the heads of the caudate nucleic and adjacent structure, which reflects decreased neuronal function. These functional changes precede the morphological changes due to caudate nucleus atrophy that are observed on computed tomography and magnetic resonance imaging. Cortical changes occur in severely diseased Huntington's patients but are more nonspecific. Prediction of individuals at risk for Huntington's disease using SPECT scans should be done with caution and in association with other clinical data. In contrast, in Parkinson's disease mild diffusely decreased perfusion is commonly noted throughout the cerebral structures, except for the cerebellum. In Parkinson's disease, there is less agreement among observers as to whether the basal ganglia are abnormal. Some observers report that there are no specific basal ganglia perfusion defects in excess of those changes seen elsewhere in the brain. Others report diminished basal ganglia uptake associated with L-dopa therapy in some Parkinson's patients, and in patients with hemi-parkinsonism there have been perfusion deficits reported in the contralateral basal ganglia. In some Parkinson patients, bilateral Alzheimer's-like posterior temporoparietal cortical perfusion defects have been observed in association with progressive dementia. Basal ganglia and cortical perfusion changes also have been reported in a few patients with a variety of other less common movement disorders.

Brain↗

PubMed1991

Cervical dystonia: clinical findings and associated movement disorders.

We studied 300 patients, 61% women, with mean age 49.7 years and mean duration of dystonia 7.8 years, to determine the demographic and clinical characteristics of cervical dystonia (CD) and its relationships to other movement disorders. Torticollis was present in 82%, laterocollis in 42%, retrocollis in 29%, and anterocollis in 25%; however, the majority (66%) had a combination of these abnormal postures. Scoliosis was present in 39%, local pain reported by 68%, and 32% had evidence of secondary cervical radiculopathy. In addition to CD, 16% of patients had oral dystonia, 12% mandibular dystonia, 10% hand/arm dystonia, and 10% had blepharospasm. Tremor was noted in 71% of patients; head-neck tremor was present in 60%, and tremor in other body regions was present in 32%. A family history of a movement disorder was present in 44% of the CD patients. Tardive dystonia was the cause in 6%; 11% had posttraumatic dystonia. Anticholinergic drugs provided moderate improvement in 33% of patients, but local intramuscular botulinum toxin injections relieved CD, local pain, or both in over 90% of all treated patients.

Adolescent↗

PubMed2003

Deep brain stimulation for movement disorders: morbidity and mortality in 109 patients.

OBJECT: Deep brain stimulation (DBS) has been advocated as a more highly effective and less morbidity-producing alternative to ablative stereotactic surgery in the treatment of medically intractable movement disorders. Nevertheless, the exact incidence of morbidity and mortality associated with the procedure is not well known. In this study the authors reviewed the surgical morbidity and mortality rates in a large series of DBS operations. METHODS: The authors retrospectively analyzed surgical complications in their consecutive series of 179 DBS implantations in 109 patients performed by a single surgical team at one center between July 1998 and April 2002. The mean follow-up period was 20 months. There were 16 serious adverse events related to surgery in 14 patients (12.8%). There were two perioperative deaths (1.8%), one caused by pulmonary embolism and the second due to aspiration pneumonia. The other adverse events were two pulmonary embolisms, two subcortical hemorrhages, two chronic subdural hematomas, one venous infarction, one seizure, four infections, one cerebrospinal fluid leak, and one skin erosion. The incidence of permanent sequelae was 4.6% (five of 109 patients). The incidence of device-related complications, such as infection or skin erosion, was also 4.6% (five of 109 patients). CONCLUSIONS: There is a significant incidence of adverse events associated with the DBS procedure. Nevertheless, DBS is clinically effective in well-selected patients and should be seriously considered as a treatment option for patients with medically refractory movement disorders.

Adult↗

PubMed1996

[Movement disorders and AIDS].

We describe the clinical characteristics, causes and response to treatment in 6 patients with AIDS who presented with abnormal movement disorders between January 1987 and July 1993 in our hospital, 3 with hemiballismus-hemichorea, 1 with athetosis, 1 myoclonia and 1 with "rubric" tremor. Brain imaging showed lesions in the corpus striatum in all the patients. Suspected diagnoses were cerebral toxoplasmosis in 4, cerebral lymphoma in 1 and progressive multifocal leukoencephalopathy in 1. The toxoplasmosis patients showed improvement (2 cases) or disappearance (2 cases) of movements with antiparasitic therapy. Treatment provided no benefit to the patients with leukoencephalopathy and lymphoma. Hemiballismus-hemichorea was the most common movement disorder in AIDS patients. The underlying cause is usually lesions in the basal ganglia arising from toxoplasmosis. If the lesions are so caused, movements may improve with antiparasitic therapy.

Acquired Immunodeficiency Syndrome↗

PubMedDate not supplied

The involvement of glutamatergic transmission in the mechanism of movement disorders induced by reversive rotation of white mice.

The ability of the selective non-competitive NMDA receptor blocker MK-801 and a series of new glutamate antagonists --the adamantane derivatives IEM-1754 and IEM-1857 and phencyclidine (IEM-1925)--to prevent movement disorders induced by reversive rotation in mice was studied. l.p. MK-801 at a dose of 0.15 ml and IEM-1754 at a dose of 5.0 mg/kg prevented the development of akinesia in response to reversive rotation as effectively as scopolamine, a known agent which provides effective prophylaxis for movement diseases. IEM-1857, the quaternary analog of IEM-1754, was not effective. IEM-1925 significantly increased the responses of mice to reversive rotation, possibly because of its high activity in relation to other subtypes of glutamate receptors. These data provide evidence for the involvement of glutamatergic transmission in the mechanism of movement disorders of vestibular origin.

Adamantane↗

PubMed2006

Movement disorders associated with encephalitis lethargica: a video compilation.

Encephalitis lethargica (EL; epidemic encephalitis; von Economo's disease) often presented with a movement disorder, and the motor consequences of postencephalitic parkinsonism (PEP) were characteristic of the chronic sequelae of this condition. PEP was similar to Parkinson's disease but was more variable and had some distinct features such as oculogyric crises. Although two previous publications have included video images of the movement disorders associated with EL and PEP, the sequences presented were typically short, showed only a few patients, and did not include the work of several neurologists who had the foresight to preserve filmed images of their patients. We describe the most complete record of EL and PEP moving images that have been preserved and make them available in edited form.

Disease Outbreaks↗

PubMed2004

[Genetics in movement disorders--dystonia, tremor and chorea].

BACKGROUND: Several dystonias, Huntington's disease, essential tremor and various rare conditions are among the hereditary movement disorders, disorders with considerable genetic and clinical heterogeneity. It may be hoped that better understanding of the genetics and pathogenetic mechanisms involved will improve management. MATERIAL AND METHODS: This review is based on personal experience and recent literature. RESULTS: At least 13 types of dystonias are genetically defined. DYT 1 seems to be the most important, and is easily tested. The Huntington gene is readily available for symptomatic as well as presymptomatic testing. Presymptomatic testing gives rise to ethical concerns and require thorough genetic counselling. Also essential tremor seems to show an autosomal dominant inheritance, but the responsible gene(s) has not been identified. INTERPRETATION: As for other movement disorders, involvement of different genes may give similar phenotypes and defects in a single gene give rise to various different phenotypes.

Dystonic Disorders↗

PubMed1982

Chronic cerebellar stimulation (CCS) and deep brain stimulation (DBS) in involuntary movement disorders.

Motor disorders of disinhibition may be modified by prosthetic mobilization of CNS inhibitory mechanisms by chronic electrical stimulation of the cerebellar cortex (CCS) and by deep brain stimulation of the thalamus and internal capsule (DBS). Reduction in spasticity, abnormal movements, intractable epilepsy and aggressive behavior has been reported after CCS, although negative results in human and animal studies have been published. No adverse neurologic, psychologic or intellectual effects of stimulation have occurred after 7 years of CCS, although subclinical histological changes may occur in the cerebellar cortex under the electrodes. CCS has been shown to produce physiological changes in evoked potentials, motoneurone excitability, epileptic discharges in the EEG and quantitative changes in movement. Surface and deep thalamic recordings have shown reduced amplitudes of somatosensory responses after CCS. Over the last 2 years we have employed chronic deep brain stimulation (DBS) in 49 patients with clinically useful results in half the patients. The technique allows reversible modification of movement disorders, and the technique can be used on the second side after a previous thalamectomy. Physiological testing, direct thalamic recordings and quantitative analysis of movement have allowed assessment of optimal rate and voltage of stimulation. For some intractable movement disorders DBS has effected significant therapeutic results when all other therapeutic techniques have failed.

Adolescent↗

PubMed1991

[Spastic movement disorders: non-medicamentous therapies based on neurophysiologic principles].

Lesions of the upper motor neurons cause a spastic syndrome and a central paresis. The consequence of these "plus" and "minus" symptoms on locomotor function is a spastic movement disorder. Strategies of physical therapy for the spastic movement disorders are discussed in relation to research findings in neurophysiology and muscle physiology. The main therapeutic technics are physiotherapy, functional electrical stimulation (FES) and biofeedback training (BFB). Various methodological problems which arise in carrying out and evaluating therapeutic studies are described. Valid prognostic criteria are essential in order to achieve an optimal use of the different therapeutic procedures. This review underlines the need for further research, since some important questions remain open.

Biofeedback, Psychology↗

PubMed1998

Neuropsychiatric assessment of patients with hyperkinetic and hypokinetic movement disorders.

BACKGROUND: The role of the basal ganglia in neuropsychiatric behaviors is not well known. Anatomical, neurophysiological, and neurochemical evidence supports the notion of parallel direct and indirect basal ganglia thalamocortical motor systems, the differential involvement of which accounts for the hypokinesia or hyperkinesia observed in basal ganglia disorders. OBJECTIVES: To evaluate the neuropsychiatric manifestations of patients with a hyperkinetic movement disorder, such as Huntington disease (HD), vs a hypokinetic disease, such as progressive supranuclear palsy (PSP). To verify if patients with HD show a greater frequency of hyperactive behaviors (eg, agitation, irritation, euphoria, or anxiety), while those with PSP exhibit hypoactive behaviors (eg, apathy). PATIENTS AND METHODS: The Neuropsychiatric Inventory, a tool with established validity and reliability, was administered to 29 patients with HD (mean +/- SD age, 43.8 +/- 2 years) and 34 with PSP (mean +/- SD age, 66.6 +/- 1.2 years), matched for education, symptom duration, and overall degree of dementia. RESULTS: There was no difference between the groups in the total Neuropsychiatric Inventory scores. However, there was a double dissociation in behaviors: patients with HD exhibited significantly more agitation (45%), irritability (38%), and anxiety (34%), whereas patients with PSP exhibited more apathy (82%) (P < .01). Euphoria was present only in patients with HD. CONCLUSIONS: We found that patients with HD manifested predominantly hyperactive behaviors, while those with PSP manifested hypoactive behaviors. Based on our findings and the anatomical lesions known to occur in these disorders, we suggest that the hyperactive behaviors in HD are secondary to an excitatory subcortical output through the medial and orbitofrontal cortical circuits, while in PSP the hypoactive behaviors are secondary to hypostimulation.

Aged↗

PubMed2002

The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service.

We have reviewed the clinical and pathological diagnoses of 143 cases of parkinsonism seen by neurologists associated with the movement disorders service at The National Hospital for Neurology and Neurosurgery in London who came to neuropathological examination at the United Kingdom Parkinson's Disease Society Brain Research Centre, over a 10-year period between 1990 and the end of 1999. Seventy-three (47 male, 26 female) cases were diagnosed as having idiopathic Parkinson's disease (IPD) and 70 (42 male, 28 female) as having another parkinsonian syndrome. The positive predictive value of the clinical diagnosis for the whole group was 85.3%, with 122 cases correctly clinically diagnosed. The positive predictive value of the clinical diagnosis of IPD was extremely high, at 98.6% (72 out of 73), while for the other parkinsonian syndromes it was 71.4% (50 out of 70). The positive predictive values of a clinical diagnosis of multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) were 85.7 (30 out of 35) and 80% (16 out of 20), respectively. The sensitivity for IPD was 91.1%, due to seven false-negative cases, with 72 of the 79 pathologically established cases being diagnosed in life. For MSA, the sensitivity was 88.2% (30 out of 34), and for PSP it was 84.2% (16 out of 19). The diagnostic accuracy for IPD, MSA and PSP was higher than most previous prospective clinicopathological series and studies using the retrospective application of clinical diagnostic criteria. The seven false-negative cases of IPD suggest a broader clinical picture of disease than previously thought acceptable. This study implies that neurologists with particular expertise in the field of movement disorders may be using a method of pattern recognition for diagnosis which goes beyond that inherent in any formal set of diagnostic criteria.

Adolescent↗

PubMed2004

Movement Disorder Society Task Force report on the Hoehn and Yahr staging scale: status and recommendations.

The Movement Disorder Society Task Force for Rating Scales for Parkinson's disease (PD) prepared a critique of the Hoehn and Yahr scale (HY). Strengths of the HY scale include its wide utilization and acceptance. Progressively higher stages correlate with neuroimaging studies of dopaminergic loss, and high correlations exist between the HY scale and some standardized scales of motor impairment, disability, and quality of life. Weaknesses include the scale's mixing of impairment and disability and its non-linearity. Because the HY scale is weighted heavily toward postural instability as the primary index of disease severity, it does not capture completely impairments or disability from other motor features of PD and gives no information on nonmotor problems. Direct clinimetric testing of the HY scale has been very limited, but the scale fulfills at least some criteria for reliability and validity, especially for the midranges of the scale (Stages 2-4). Although a "modified HY scale" that includes 0.5 increments has been adopted widely, no clinimetric data are available on this adaptation. The Task Force recommends that: (1) the HY scale be used in its original form for demographic presentation of patient groups; (2) when the HY scale is used for group description, medians and ranges should be reported and analysis of changes should use nonparametric methods; (3) in research settings, the HY scale is useful primarily for defining inclusion/exclusion criteria; (4) to retain simplicity, clinicians should "rate what you see" and therefore incorporate comorbidities when assigning a HY stage; and (5) because of the wide usage of the modified HY scale with 0.5 increments, this adaptation warrants clinimetric testing. Without such testing, however, the original five-point scales should be maintained.

Feasibility Studies↗

PubMed2007

Gait abnormalities in psychogenic movement disorders.

An abnormal gait is not uncommon in patients with medically unexplained neurological symptoms, including those with other psychogenic movement disorders (PMDs). Previous studies have not evaluated the gait characteristics of patients with a variety of PMDs and there are no reports comparing PMDs with and without gait disturbances. We were interested in determining how those with and without additional involvement of gait differed and how PMD patients differed from those with a pure psychogenic gait disorder (PGD) in the absence of another PMD. We investigated gait features in a large series of patients with PMD (n = 279), dividing them into two groups (Group I with a normal gait and Group II with an abnormal gait). Group I included those with PMD with a normal gait and no change in the PMD while walking (I-1), and those with a change in PMD while walking, but not affecting gait (I-2). Group II was divided into those with PMD with additional abnormal gait (II-1) and those with pure psychogenic gait disorder without other abnormal movements (II-2). Excessive slowing of movement was more common in PMD patients with an abnormal gait (Group II) compared to those without (Group I). Slowness of gait was the most common feature in patients with PMD combined with a PGD (II-1) and buckling of the knee pattern was the most common type of pure PGD (II-2), followed by astasia-abasia.

Adolescent↗

PubMed2006

Parkinson's disease and LRRK2: frequency of a common mutation in U.S. movement disorder clinics.

The G2019S mutation in the LRRK2 gene is reportedly a common cause of familial Parkinson's disease (PD) and may also have a significant role in nonfamilial PD. The objective of this study was to assess mutation carrier frequency in PD patients from movement disorder clinics in the United States, stratified by family history, age at onset, and geography; to determine carrier frequency in a large and well-characterized control population; to examine segregation of mutation in families of patients; and to correlate genotype with clinical phenotype. One thousand four hundred twenty-five unrelated PD patients from movement disorder clinics in Oregon, Washington, and New York and 1,647 unrelated controls were studied. The G2019S mutation was detected using a TaqMan assay and verified by sequencing. Eighteen of 1,425 patients and one of 1,647 controls had the mutation. Carrier frequency (+/- 2SE) in patients was 0.013 +/- 0.006 overall, 0.030 +/- 0.019 in familial PD, 0.007 +/- 0.005 in nonfamilial PD, 0.016 +/- 0.013 in early-onset PD, and 0.012 +/- 0.007 in late-onset PD. Geographic differences were insignificant. Age at onset of mutation carriers ranged from 28 to 71 years. Mutation carriers were clinically indistinguishable from idiopathic PD. LRRK2 G2019S is the single most common pathogenic mutation linked to neurodegenerative disease to date.

Adolescent↗

PubMed2002

Restless legs syndrome and periodic limb movement disorder.

Restless leg syndrome and PLMD are nocturnal movement disorders associated with significant adverse effects on the health and well-being of patients and their families [66]. Although the pathophysiological basis remains to be fully described, current research points to abnormalities in CNS function and neurotransmitter systems. The accurate diagnosis of RLS and PMD requires a thorough history, physical examination, diagnostic tests, and often, a referral to a sleep disorders specialist. Considering the prevalence of these conditions and their negative impact, nurses should be well-versed in the assessment and management of these problems as well as in the appropriate education of patients and their families. Nursing research is greatly needed, particularly with regard to the development and testing of biobehavioral interventions designed to decrease associated symptoms and improve clinical outcomes. Finally, because of the complexity of the clinical presentation of RLS and PLMD, this population of patients presents nurse clinicians and researchers alike with an extraordinary opportunity for interdisciplinary collaboration.

Diagnosis, Differential↗

PubMed2001

Movement disorders associated with neuroleptic treatment.

Neuroleptic-induced movement disorders, or extrapyramidal side effects (EPS), can be classified into acute and tardive syndromes. Among the former are parkinsonism, dystonia, and akathisia. Conventional neuroleptics that have traditionally been used to treat psychiatric disorders are often associated with EPS. The newer atypical antipsychotics provide a more promising treatment strategy for psychiatric disorders and have a lower potential for producing EPS than conventional neuroleptics.

Age Factors↗

PubMed2001

Periodic limb movement disorder : a clinical and polysomnographic study.

Periodic limb movement disorder (PLMD) is one of the commonest neurological disorders and causes significant disability, if left untreated. However, it is rarely diagnosed in clinical practice, probably due to lack of awareness and/or lack of necessary diagnostic facilities. Restless leg syndrome (RLS), aging, pregnancy, uraemia, iron deficiency, polyneuropathy are some of the common causes of secondary PLMD. Clinical presentation, polysomnographic findings and management of six patients of PLMD have been discussed in this report.

Adult↗

PubMed1993

Drug-induced movement disorders.

This review covers the major categories of movement disorders secondary to the use of antipsychotic drugs and other pharmacologic agents that interfere with dopaminergic transmission at the basal ganglionic level. In the past year new syndromes were reported, old concepts were either challenged or supported by recent studies, and significant contributions were made in unraveling the complex pathophysiology of these disorders.

Akathisia, Drug-Induced↗