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A case of primary hypothyroidism with hemichoreoathetoid movement was presented. After thyroid hormone replacement therapy for a few weeks, the movement disorder decreased dramatically. This abnormal movement may be one of the neurological manifestations of hypothyroidism.
INTRODUCTION: Functional thalamic surgery as a treatment for movement disorders is half a century old. Although the accumulated experience is wide, their precise indications have varied depending on the available alternative therapies. DEVELOPMENT: In this article the historical evolution, anatomofunctional basis and results of the functional surgery on the motor thalamus are reviewed. Considering new advances in this field, the current indications are proposed. CONCLUSIONS: Nowadays, thalamic surgery is a valuable therapeutic option for patients with different tremor syndromes, when the upper limb tremor is disabling, and satisfactory relief with pharmacological treatment cannot be achieved. It can also be useful in selected patients with ballism and with secondary hemidystonia. When unilateral surgery is considered, both thalamotomy and thalamic stimulation can be acceptably safe and effective procedures in experienced surgical teams. The main advantage of stimulation is its irreversibility, making possible bilateral procedures with a lower morbidity. The objective of surgery should be to improve the patients' functional capacities and quality of life, therefore, the indication and selection of the procedure should be individualized.
Abnormal movements occur in many of the neurologic disorders affecting children and in certain conditions are the presenting major manifestations. In children abnormal movements may be transient and benign and do not necessarily indicate a progressive degeneration of the central nervous system. Clinical observations and appropriate laboratory investigation will frequently lead to a neurologic diagnosis that in many instances will respond to specific pharmacologic treatment.
OBJECTIVE: To describe the gamut of movement disorders (MD) seen during the clinical course of kuru. BACKGROUND: Kuru is a subacute spongiform encephalopathy that was confined to several adjacent cultures in the Eastern Highlands of New Guinea and resulted from contamination with brain tissue during the ritual endocannibalism practiced in those societies. This unique neurologic disease was recorded extensively with film between 1957 and 1976, and these comprehensive research documents have been donated to the American Academy of Neurology archives by one of the authors (DCG). METHODS: The comprehensive assembly of film record of kuru, which was collected by one of the authors (DCG) was reviewed. This comprised two parts: The first were films from 1957-1964 and included 17.397 ft of 16-mm film featuring 204 patients (children and adults); the second is assembled from films made from 1967-1976 and includes 9138 ft. of film featuring 47 adult patients. Two MD specialists categorized all MDs observed and a representative videotape was produced. RESULTS: Tremor is the most frequently encountered MD in kuru and is typically of the action/intention type, which appears early in the disease and is soon associated with other clinical signs of cerebellar dysfunction. Widespread clonus is characteristic of advanced disease and can be difficult to differentiate from tremor. Dystonia/athetosis and choreiform jerks also appear as the disease progresses. Dystonia can involve the torso, distal limbs, neck, or jaw. Myoclonic jerks can be superimposed on the cerebellar or dystonic features usually with an enhanced startle response. Parkinsonian symptomatology, other than resting tremor is frequent among the filmed subjects especially in the second stage of the disease. CONCLUSION: The clinical manifestations of kuru involved a wide array of MDs during all three stages of the degenerative illness.
This study describes preliminary laryngeal electromyography (LEMG) data and botulinum toxin treatment in patients with dysphonia due to movement disorders. Twenty-five patients who had been clinically selected for botulinum toxin administration were examined, 19 with suspected laryngeal dystonia or spasmodic dysphonia (SD), 5 with vocal tremor, and 1 with Gilles de la Tourette syndrome (GTS). LEMG evaluations were performed before botulinum toxin administration using monopolar electrodes. Electromyography was consistent with dystonia in 14 patients and normal in 5, and differences in frequency suggesting essential tremor in 3 and Parkinson tremors in 2. The different LEMG patterns and significant improvement in our patients from botulinum toxin therapy has led us to perform laryngeal electromyography as a routine in UNICAMP movement disorders ambulatory.
BACKGROUND: PMD are disabling, but lack any generally accepted treatment strategies. DESIGN/METHODS: Ten patients with PMD received treatment with Psychodynamic Psychotherapy. Patients were assessed with psychiatric rating scales and the movement disorder was rated by a blinded rater with the Psychogenic Movement Disorder Rating Scale (PMDRS). RESULTS: Total mean PMDRS (p = 0.0195), total PMDRS function scores (p = 0.0142), Hamilton depression scores (p = 0.009), Beck anxiety scores (p = 0.002), and GAF (p = 0.0083) all improved with psychotherapeutic intervention. CONCLUSIONS: Psychotherapy and appropriate use of adjunct psychiatric medication can be a successful intervention for PMD.
Our experience of deep brain stimulation (DBS) and motor cortex stimulation (MCS) in patients with post-stroke movement disorders and post-stroke pain is reviewed. DBS of the thalamic nuclei ventralis oralis posterior et intermedius proved to be useful in more than 70% of patients with post-stroke involuntary movements (hemiballismus, hemichoreo-athetosis, distal resting and/or action tremor, and proximal postural tremor). The effect of DBS of the thalamic nucleus ventralis caudalis or internal capsule on post-stroke pain was usually disappointing. Excellent pain control can be achieved by MCS in approximately 50% of patients with post-stroke pain. In the course of clinical trials on MCS for the control of post-stroke pain, it was found that co-existent post-stroke involuntary movements (hemichoreo-athetosis and resting tremor) could also be controlled by MCS. Post-stroke involuntary movements, especially those in thalamic syndrome, are sometimes associated with post-stroke pain. In such disorders, involuntary movements are attenuated, but the pain in the same patients is often exacerbated by DBS of the thalamic nuclei ventralis oralis posterior et intermedius. MCS could be the therapy of choice under such circumstances. Subjective improvement of voluntary motor performance, which had been impaired in association with mild or moderate hemiparesis, was reported during MCS by approximately 20% of patients with post-stroke pain. Such an effect on voluntary motor performance appears to be caused by an inhibition of their rigidity. The reversibility of DBS and MCS makes them an important option for the control of post-stroke movement disorders and post-stroke pain.
Rett syndrome, a progressive neurodegenerative disorder described only in female subjects, is manifested by a wide spectrum of behavioral and motor abnormalities. We studied 32 patients with this disorder, ages 30 months to 28 years old, and characterized their extrapyramidal disturbance. The most common motor abnormalities were stereotyped movements and gait disturbance, seen in all patients. Bruxism, oculogyric crises, parkinsonism, and dystonia were also common, but myoclonus and choreoathetosis were seen only infrequently. The hyperkinetic movement disorders tended to dominate in younger patients, while bradykinetic disorders were more evident in the older patients. This study provides evidence that movement disorders seen in Rett syndrome reflect age-related neurodegenerative changes in the basal ganglia.
Measuring (extrapyramidal) psychotic disorders often is a highly relevant part of clinical research into the course and treatment of movement disorders. Traditionally these disorders have always been assessed on the basis of a well-known set of rating scales. The limitations and shortcomings of these scales are discussed in the article. It is argued that a new standard instrument needs to be developed for the measurement of movement disorders. The SADIMOD can be regarded as a good starting point for the development of such an instrument.
Primary defects of mitochondrial DNA leading to respiratory chain dysfunction have been described in association with dystonia, chorea and parkinsonism. Myoclonus remains the commonest movement disorder associated with such defects. The genetic basis of Leigh's syndrome, which is frequently associated with movement disorders, may be mitochondrial or nuclear. Respiratory chain dysfunction has been identified in Huntington's disease in addition to Parkinson's disease, but the cause and relationship of this dysfunction to the pathogenesis of these common disorders is not yet determined.
The most potent biologic toxin, botulinum toxin (BTX), has become a powerful therapeutic tool in the treatment of a variety of neurologic, ophthalmic, and other disorders manifested by abnormal, excessive, or inappropriate muscle contractions. This review focuses on the use of BTX in the treatment of dystonia and other movement disorders. The therapeutic application of BTX, however, extends beyond movement disorders; chemodenervation with BTX has been found to ameliorate spasticity, rigidity, spastic bladder, achalasia, and even some cosmetic conditions. In addition to describing its therapeutic effects, this article also reviews recent advances in the understanding of the molecular and cellular mechanisms of BTX. Few therapeutic agents have been better understood in terms of their mechanism of action or have had greater impact on patients' functioning than BTX. BTX-A has been used in nearly all clinical trials. Blocking anti-BTX-A antibodies have been detected in about 5% of patients chronically treated with this type of BTX. Patients who develop immunoresistance to BTX-A may benefit from other serotypes of BTX, such as BTX-B and -F, currently undergoing clinical trials.
Recently it has been discovered that defects in neuronal ion channels can result in seizure disorders. The tottering mouse is a genetic animal model carrying a mutation in the alpha1A calcium channel subunit that causes these mice to exhibit generalized petit mal-like epilepsy, cerebellar ataxia, and an intermittent movement disorder that has some characteristics similar to myoclonus or myoclonic epilepsy. We postulate that abnormal cerebellar Purkinje cell output to the deep cerebellar nuclei results in the intermittent movement disorder observed in these mice. The frequency and duration of seizure activity were measured in tottering mice before and 2 weeks after surgical or chemical lesioning of the cerebellum. Surgical lesions in the anterior cerebellar vermis of tottering mice produced significant reductions in seizure duration and frequency. Surgical lesioning of the posterior cerebellar vermis had no significant effect. Chemical lesions of the same cerebellar regions, using a locally applied neurotoxin, NMD-L-A, appear to produce effects similar to the surgical lesions. These data indicate that anterior vermal cerebellar output is important for production of the seizures associated with the intermittent movement disorder observed in tottering mice.
OBJECTIVE: [corrected] To evaluate efficacy and safety of botulinum toxin type B (BTX-B) in treatment of movement disorders including blepharospasm, oromandibular dystonia, hemifacial spasm, tremor, tics, and hypersecretory disorders such as sialorrhea and hyperhidrosis. METHODS: A retrospective study of BTX-B injections in treatment of 58 patients with various neurological disorders was performed. The mean follow-up time was 0.9 +/- 0.8 years. Results of the first and last treatment of patients with at least 3 injection sessions were compared. RESULTS: The response of 58 patients to a total of 157 BTX-B treatment sessions was analyzed. Of the 157 treatment sessions, 120 sessions (76.4%) resulted in moderate or marked improvement while 17 sessions (10.8%) had no response. The clinical benefits after BTX-B treatment lasted an average of 14 weeks. Of the 41 patients with at least 3 injection sessions (mean 10 +/- 8.6), most patients needed increased dosage upon the last session compared to the first session. Nineteen patients (32.8%) with 27 sessions (17.2%) reported adverse effects with BTX-B treatment. CONCLUSIONS: Though most patients require increased dosage to maintain effective response after repeated injections, BTX-B is an effective and safe treatment drug for a variety of movement disorders, as well as drooling and hyperhidrosis.
The results of a community-based study measuring the occurrence of movement disorders in a population of people with learning difficulties treated with antipsychotic medication are presented. This group was compared with an age- and sex-matched group with a similar degree of handicap, who were not treated with antipsychotic medication. When medication was given within British National Formulary Guidelines, no significant increase in movement disorders in the treated group was found. The relevance of this to psychiatric practice is discussed.
In recent years there has been renewed interest in the role of autoimmunity in many neurological disorders such as multiple sclerosis and neurodegenerative diseases. Research advances in this field have led to the discovery of new potential therapeutic strategies. There are, however, only a few neurological disorders in which an autoimmune origin has been adequately documented and definitely confirmed. The most important neurological diseases causing movement disorders in which an autoimmune basis has been demonstrated are summarized in this review. The possible role of immunity with the most frequent movement disorders such as parkinsonism or dystonia is also commented.
Standardized rating scales for Parkinson's disease and dystonia have been validated and are now widely accepted as useful clinical assessment tools. However, the other movement disorders have been more difficult to quantify. The use of a standardized videotape protocol can provide a more precise audiovisual record of the movement disorder patient. With broader use by others and further revisions, these guidelines can be improved in order to provide an accurate assessment and teaching tool. The authors welcome comments.
OBJECTIVE: To describe a case of movement disorder associated with cisapride use. METHOD: Case report.RESULTS: This is the case of a male eight months old child who began to use cisapride, 0,2 mg/kg tid to treat gastroesophageal reflux disease. One month after beginning with the drug, he started to present repetitive movements of the hands characterized by opening and closing hands with flexion and extension of the wrists. According to the mother, these movements became more evident as the dose of the medication was increased, and, thereafter, started to happen also on the feet. When the child was six months old, time of the first neurological evaluation, he presented normal neurological development, except for the Parachute reflex, which was absent. After excluding metabolic, toxic and infectious diseases, the drug was withdrew. The child evolution was benign, with gradual disappearance of the movements, and he was completely normal 30 days after.CONCLUSIONS: The authors stress the need suspending the use of cisapride in any case of neurologic symptoms as seizures, somnolence, malaise or involuntary movements in previously normal patients.
Levodopa-induced dyskinesia (LID) remains a significant motor complication in Parkinson's disease (PD), although opinions differ on its clinical relevance.To explore the current prevalence and impact of LID, we analyzed two cohorts from the Latin American Research Consortium on the Genetics of Parkinson's Disease from movement disorder clinics in the city of São Paulo, Brazil, recruited 10 years apart.The cohorts included 187 individuals diagnosed with PD in phase 1 (2007-2014) and 224 in phase 2 (2021-2022). The presence and functional impact of LID were measured using part IV (items 4.1 and 4.2 respectively) of the Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS).The analysis revealed that LID frequency increased from 34.7 in phase 1 to 54.9% in phase 2 (more recent), with functional impact rising from 25.1 to 38.8%.The findings suggest that LID remains a relevant clinical issue in clinics specialized in movement disorders in Brazil, with no reduction in prevalence throughout the last decade. Further studies from other regions and less specialized neurology centers may help understand this motor complication in Brazil and in other developing countries.