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Computed tomography and magnetic resonance imaging in children with rare movement disorders.

Both CT and MR can aid in the differentiation between benign and less favorable pediatric movement disorders. However, MR is the more sensitive imaging modality and thus preferable. This was demonstrated in 18 patients with conditions ranging from idiopathic dystonias to aminoacidopathies, Leigh's disease, and Hallervorden-Spatz disease. In the latter three entities characteristic lesions were shown in the basal ganglia, although not consistently. A specific diagnosis seems possible in Leigh's disease, provided the clinical setting is appropriate.

Adult↗

Therapeutic trial of milacemide in patients with myoclonus and other intractable movement disorders.

We performed a therapeutic trial with the glycine precursor, milacemide, on 10 patients with intractable movement disorders. Six had myoclonus of various etiologies and one each had progressive supranuclear palsy, Filipino X-linked dystonia with parkinsonism, painful legs and moving toes, and stiff-person syndrome. Milacemide was initiated at a dose of 2,400 mg/day, orally, and increased gradually to a maximum of 4,800 mg/day. No clear-cut observable improvement occurred. There were no serious adverse effects.

Acetamides↗

Neuropsychology for movement disorders neurosurgery.

The neuropsychologist plays a crucial role in three phases of the neurosurgical treatment of movement disorder patients, namely screening, outcome evaluation and research. In screening patients, the differential diagnosis of dementia, impact of depression or other psychiatric conditions, and the influence of disease and medication-induced symptoms on cognitive performance must be determined. Postoperatively, systematic evaluations elucidate the cognitive costs or benefits of the procedure. The neuropsychologist is then able to provide feedback and counselling to the professional staff, patient and family to inform management strategies. Neuropsychologists also study alteration of cognitive processing due to lesions or stimulation, which, in tandem with functional imaging, shed light on plasticity in cortical and subcortical processing.

Dementia↗

An FDOPA PET study in patients with periodic limb movement disorder and restless legs syndrome.

The authors investigated nine drug-naive patients with periodic limb movement disorder and restless legs syndrome (PLMD-RLS) and 27 healthy controls with PET using 6-[18F]fluoro-L-dopa (FDOPA). In the patients, the FDOPA uptake (Ki(occ)) in the caudate nucleus was 88% and in the putamen 89% of the control mean values. This equal affection of the caudate and the putamen differs, for example, from the dopaminergic dysfunction in Parkinson's disease, which affects the putamen earlier and more severely than the caudate. The current results indicate mild nigrostriatal presynaptic dopaminergic hypofunction in PLMD-RLS.

Adult↗

Movement disorders in the eosinophilia-myalgia syndrome: tremor, myoclonus, and myokymia.

OBJECTIVE: To describe the development of tremor, myoclonus, and myokymia as chronic sequelae of eosinophilia-myalgia syndrome (EMS). METHODS: Four patients among a cohort of 57 individuals with EMS followed prospectively were identified to have objective movement disorders. Electrophysiologic findings were assessed by recording of electromyographic activity from pairs of upper extremity antagonist muscles using 2 input channels. RESULTS: Clinical and electrophysiologic studies documented the presence of tremor (4 patients), myoclonus (3 patients), and myokymia (1 patient) as late sequelae of EMS. CONCLUSION: The recognition of these movement disorders extends the spectrum of chronic neurologic disease in EMS.

Adult↗

[Possible mechanisms of involvement of the subthalamic nucleus and structures interconnected with this nucleus in movement disorders evoked by dopamine depletion].

A possible mechanism of involvement of the subthalamic nucleus (STN) in movement disorders evoked by dopamine deficit is suggested. Multifunctional role of the STN is based on following reasons. Various STN cells participate in the cortico-basal ganglia-thalamocortical loop and in the basal ganglia-pedunculopontine-basal ganglia loop. Complexity of neural circuits is determined by functional heterogeneity of neurons in the nuclei, reciprocally connected with the STN, as well as by opposite modulation of activity of these neurons by dopamine due to activation of different types of pre- and postsynaptic receptors. Dopamine influences activity of STN neurons directly, through pre- and postsynaptic receptors. It is assumed that high-frequency stimulation of the STN can reduce or eliminate Parkinsonian symptoms not only owing to inhibition of activity of GABAergic neurons in the output basal ganglia nuclei, projected into the thalamus or pedunculopontine nucleus, but also due to excitation of glutamatergic or cholinergic neurons in the output nuclei, and due to potentiation of excitatory inputs to preserved dopaminergic neurons and subsequent rise in dopamine concentration.

Animals↗

Absolutely therapy-resistant depression and mixed movement disorder in an unusual case of polycythemia vera.

This report presents a 64-year-old female patient with unusual psychiatric and neurological complications in polycythemia vera. Six years after onset of the hematologic disorder the patient succumbed to an absolutely therapy-resistant depression, from which she has been suffering for the past four years--intensive and broad--spectrum drug and electroconvulsive treatment has failed to ameliorate the condition. In addition, the patient has developed a mixed movement disorder with rhythmical movements, mainly affecting the trunk, akathisia, hyperventilation, and pleurothotonus ("Pisasyndrome"). The paper discusses a possible link between hypoxic cerebral damage, caused by hyperviscosity of the blood due to polycythemia vera, and the therapy resistance of the depression. The case confirms that organic factors may cause therapy resistance in depressive disorders.

Depressive Disorder↗

Tetrabenazine in the treatment of hyperkinetic movement disorders.

Tetrabenazine, a dopamine-depleting agent first synthesized half a century ago, was initially developed for the treatment of schizophrenia. Although psychotic disorders have since been treated more successfully with other neuroleptic medications, many studies have shown this drug to be effective in the treatment of hyperkinetic movement disorders (hyperkinesias). Hyperkinesias are neurologic disorders characterized by abnormal involuntary movements such as chorea associated with Huntington's disease, tics in Tourette's syndrome and stereotypies in tardive dyskinesia. Recently, clinical trials investigating tetrabenazine for the treatment of chorea associated with Huntington's disease found the drug to be safe and efficacious, making approval by the US Food and Drug Administration for this indication a distinct possibility.

Adrenergic Uptake Inhibitors↗

Movement disorders due to cerebral Toxoplasma gondii infection in patients with the acquired immunodeficiency syndrome (AIDS).

Hemichorea and parkinsonism are unusual manifestations of cerebral toxoplasmosis in patients with AIDS. We here describe two such cases and we reviewed extensively the literature (through computer searches using MEDLINE) for other reported instances. In our patients, unlike the other neurological symptoms, the response of the movement disorders to anti-toxoplasmosis therapy was delayed and only partial. We demonstrate that tetrabenazine is a valuable additional symptomatic treatment for choreic movements in one of our patients. We emphasize that, among patients suffering from AIDS, particularly in countries with high prevalence of toxoplasmosis, the occurrence of movement disorders should first suggest the diagnosis of cerebral toxoplasmosis.

Acquired Immunodeficiency Syndrome↗

Movement disorders in children and adolescents.

Tourette syndrome (TS), Sydenham chorea, and drug-induced dyskinesias are prototypical movement disorders affecting children. Underlying involvement of basal ganglia has been apparent for several decades, but new neuroimaging studies are adding detail to this mechanism. Genetic studies of TS and tardive dyskinesia may further reveal the underlying pathophysiology. Most provocative is the new conceptual model of poststreptococcal autoimmune neuropsychiatric disorder. Although unproven, substantial support for this model comes from immunologic, family, neuroimaging, and treatment studies.

Adolescent↗

CT-guided thalamotomy in the treatment of movement disorders.

CT-guided thalamotomy has been used in Frenchay Hospital since 1985 in the treatment of movement disorders. This technique avoids intraventricular contrast injections and therefore simplifies the placement of thalamic lesions, decreases patient morbidity and shortens inpatient stay. Our experience with this technique is presented.

Humans↗

Movement disorder in never and minimally treated Nigerian schizophrenic patients.

Two hundred and forty-two Nigerian schizophrenic patients (63% male, 37% female), whose mean age was 42 years and length of illness 12 years, were examined for movement disorders using the Abnormal Involuntary Movements Scale, the Simpson and Angus Parkinsonism Scale and the Barnes Akathisia Scale. Twelve patients had never received antipsychotic medication; and none of these had dyskinesia. Dyskinesia was found in 5 of 49 patients (10%) who had taken medication throughout the course of their illness for a total of up to 3 months, 13 of 74 (18%) who had taken medication for 4-12 months, 14 of 41 (34%) for 1-5 years, and 29 of 66 (45%) who had taken medication for more than 5 years. Of 77 patients who were receiving antipsychotic medication at the time of examination, 9 (12%) had Parkinsonism and 12 (15%) akathisia. Examination of the patients' mental state by the Positive and Negative Syndrome Scale showed an association between dyskinesia and positive, but not negative, schizophrenic symptoms. Nigerian patients showed a low level of negative symptoms.

Adolescent↗

Spontaneous paroxysmal circling behavior in the ci2 rat mutant: epilepsy with rotational seizures or hyperkinetic movement disorder?

Circling, turning, rotating, spinning, wheeling, and cursive hyperkinesia are all synonymous terms used to describe the active movement of an animal in a circular direction. Circling behavior can be evoked by unilateral electrical and chemical stimulation or lesions of various brain sites, but can also occur after systemic drug administration or spontaneously in normal animals or mutant rodents. In humans, stereotypic body rotation can occur as a distinctive entity of generalized and focal epilepsy, and may be due to involvement of the striatum. We have previously described a Lewis rat mutant (ci2) with a behavioral phenotype characterized by lateralized circling, hyperactivity, opisthotonus, and ataxia. In these rats, circling occurs in phases or bursts either spontaneously or in response to stress. Neurochemical data indicate that the circling behavior of the ci2 mutants is related to an abnormal asymmetry in dopaminergic activity in the striatum. Because of the similarities to rotational epilepsy, we used video and electroencephalographic recordings to study whether the rotational behavior of the ci2 mutant rat is a result of a partial or generalized epilepsy. Epileptic WAG/Rij rats were used for comparison. Video monitoring of ci2 rats in the absence of any stress or disturbance showed that circling occurs in paroxysmal bursts during active wakefulness, but not during passive wakefulness or sleep. Circling was not preceded or followed by any convulsive motor seizures and was not associated with epileptiform abnormalities in the electroencephalogram, whereas WAG/Rij rats exhibited myoclonic seizures and epileptic spike-wave discharges during passive wakefulness and sleep. As a result of the association of circling with active wakefulness, ci2 rats exhibited many more rotations during the dark (active) phase compared with the light (rest) period. Increase in active wakefulness during the light phase by transfer of the rats to a new environment induced or intensified circling behavior. Most ci2 rats showed a consistent lateral preference during circling, but some rats changed their preference from one session to another. The data indicate that spontaneous paroxysmal circling behavior in the ci2 rat is not a consequence of epilepsy but reflects a hyperkinetic movement disorder with abnormal lateralization of brain function.

Animals↗

Persistent movement disorders induced by buspirone.

Buspirone, an azospirone compound, is a nonsedative anxiolytic that has achieved wide usage since its introduction in 1987. Although relatively free of side-effects, there have been several instances of dyskinesia and dystonia associated with the use of buspirone. We report two patients with persistent movement disorders that developed after prolonged treatment with the drug. One patient developed a lasting problem of cervical-cranial dystonia and tremors after treatment with buspirone at a dosage of 40 mg/day for several weeks. Another, receiving 30 mg/day for 6 weeks, experienced an exacerbation of preexisting spasmodic torticollis and tardive dyskinesia as well as the onset of involuntary phonations. As shown by these and other examples, buspirone poses the risk for inducing or exacerbating several types of movement disorders.

Antipsychotic Agents↗

Cognitive and behavioral anomalies among insomnia patients with mixed restless legs and periodic limb movement disorder.

This study examined whether patients who have periodic limb movement disorder (PLMD), with or without comorbid restless legs syndrome (RLS), display the sleep-disruptive cognitive and behavioral anomalies found among primary insomnia sufferers. Archival data from a Sleep History Questionnaire, home-based polysomnography, and a sleep log were obtained for matched RLS/PLMD, primary insomnia, and noncomplaining volunteer samples. Statistical comparisons showed that the RLS/PLMD and primary insomnia samples differed significantly from the normal sleepers in regard to their propensities for certain sleep disruptive habits, perceived difficulties controlling pre-sleep cognitive activity, and their subjective sleep appraisals. These findings suggest RLS/PLMD patients display many of the cognitive and behavioral anomalies thought to perpetuate primary insomnia. Hence, behavioral interventions may be warranted for RLS/PLMD patients.

Adult↗

The movement disorder of reflex sympathetic dystrophy.

We present 43 patients with reflex sympathetic dystrophy (RSD) who manifested abnormalities of movement. The patients have focal dystonia, weakness, spasms, tremor, difficulty initiating movement, and increased tone and reflexes. These motor signs and symptoms may precede other manifestations of the illness by weeks or months. They most frequently, but not invariably, occur concomitantly with sudomotor or vasomotor changes and pain. Lioresal is effective in reducing spasms. Early in the course of RSD, the motor manifestation may be alleviated by intense sympathetic blockade or sympathectomy. In many patients, the movement disorder becomes independent of sympathetic innervation.

Adolescent↗

Subjective sleep quality and suggested immobilization test in restless leg syndrome and periodic limb movement disorder.

The severity of restless leg syndrome (RLS) and/or periodic limb movement disorder (PLMD) was investigated by using a suggested immobilization test (SIT) and by measuring the influence of these disorders on the subjective sleep quality as assessed by the Pittsburgh Sleep Quality Index (PSQI). Patients with RLS and those with both RLS and PLMD showed remarkably high values for PSQI and SIT, whereas patients with PLMD only showed normal values for PSQI. These findings suggest that there is only a small pathological significance for periodic limb movements, and demonstrate the efficacy of SIT and PSQI for evaluating the severity of these disorders.

Electromyography↗

Interrelations between psychiatric symptoms and drug-induced movement disorder.

After 30 years of clinical research into drug-induced movement disorder (DIMD), we are still facing unresolved issues regarding the interrelations between psychiatric symptoms and DIMD. Recently, I proposed a new classification of DIMD that includes abnormal movements previously labelled extrapyramidal symptoms. DIMD caused by psychotropic drugs is still confused with psychiatric symptoms treated by the same drugs. The results from 2 international multicentre trials, the InterSePT and the Ris-Consta Studies, conducted in the era of both typical and atypical antipsychotic agents, which included over 3,000 patients with schizophrenia and schizoaffective disorder worldwide, still showed a high, but decreasing, incidence of pretreatment DIMD, which varied from 57.5% (1998-1999) to 47.4% (1999-2000), and a decreasing incidence of tardive dyskinesia, which varied from 12% (1998-1999) to 10.2% (1999-2000), reflecting the greater use of atypical antipsychotic drugs. Furthermore, in both studies, psychiatric symptoms as measured by the Positive and Negative Symptom Scale (PANSS) were significantly correlated with DIMD and DIMD subtypes, thus suggesting the need for additional measurement instruments in schizophrenia and related psychoses.

Depressive Disorder↗