Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective study (1995-2000) of reports of cases of metoclopramide poisoning collected at the Lille poison control centre (184 phone calls) shows the frequent occurrence of acute dystonia in children (81 cases). These spectacular extrapyramidal symptoms consist of abnormal movements (31 cases), local hypertonia (30 cases), acute dyskinesia (17 cases), general hypertonia (16 cases) and oculogyric crisis (13 cases). There is no dose-effect correlation and sex has no influence on the occurrence of neurological symptoms. Medical management is simple surveillance or symptomatic therapy (a sedative drug and/or anti-parkinsonian therapy and/or a gastrointestinal adsorbent). Hospitalisation was needed in only 9.1% of cases. Providing better information to physicians about metoclopramide adverse effects and their medical management should allow a reduction in the number of unnecessary hospitalizations.
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PMID:[Acute dystonia from metoclopramide in children]. 1467 77

We present the case of a 19-yr-old woman with a history of generalized dystonia who developed sudden onset of adductor spasms of the vocal cords and increased dystonia after the interruption or intrathecal baclofen therapy. Her symptoms resolved after intrathecal baclofen was restored. In patients with dystonia receiving intrathecal baclofen therapy, the onset of dyspnea associated with increased muscle tone should prompt the investigation of baclofen withdrawal.
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PMID:Respiratory distress after intrathecal baclofen withdrawal. 1528 34

In the United States, the popularity of botulinum toxins as agents to treat muscle hypertonia has grown significantly over the last decade, despite lack of approval from the Food and Drug Administration for the indication of spasticity. Botox (botulinum toxin type A) and Myobloc (botulinum toxin type B) are Food and Drug Administration-approved for other indications, such as cervical dystonia. Another commercial preparation of type A, Dysport, has yet to reach the United States market as of this writing. Although botulinum toxin's efficacy in influencing spastic hypertonia is well accepted, the impact of certain clinical issues, such as dosing and dilution, on treatment outcome is not well established by published studies. This article will review important articles and selected abstracts on the use of botulinum toxin, specifically for spastic hypertonia in adults, with emphasis on current clinical practices as they relate to dosing and dilution.
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PMID:Botulinum toxin: dosing and dilution. 1544 75

Dystonia is a state of continuous contraction of groups of agonist and antagonist muscles resulting in a sustained abnormal posture. Dopa-responsive dystonia was first described in 1976 by Segawa. Patients typically have diurnal variation of their symptoms with worsening at the end of the day and a dramatic response to low-dose L-dopa. This report presents five consecutive children with dopa-responsive dystonia who were misdiagnosed initially as spastic diplegic cerebral palsy, intractable epilepsy, hereditary spastic paraplegia, or a neurodegenerative disorder. There were two males and three females aged 3-13 years (mean 8.6 years). They were monitored for up to 2 years (mean 14.8 months). One had focal, one axial, one segmental, and two generalized dystonia. The dystonia was paroxysmal in two (tiptoe walking and opisthotonus), and all had a progressive course. All children responded dramatically to L-dopa (mean 200 mg/day), including three who were wheelchair-bound for several years. The difficulties in early diagnosis, variability of clinical presentation, and dramatic response to L-dopa will be illustrated. To conclude, dopa-responsive dystonia should be considered in any child who presents with paroxysmal or progressive hypertonia of unknown etiology, because it responds so dramatically to L-dopa.
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PMID:Misdiagnoses in children with dopa-responsive dystonia. 1546 46

Reports describing the neurological features of Lesch-Nyhan disease (LND) vary widely, thereby implying the involvement of different neurological substrates. The movement abnormalities in 20 patients with LND were investigated. Dystonia was the most frequent and severe movement disorder. At rest, hypotonia was more frequent than hypertonia. These findings are compatible with basal ganglia dysfunction in LND.
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PMID:The motor disorder of classic Lesch-Nyhan disease. 1557 Dec 21

Botulinum toxin A (BTX A) has been used for more than 20 years as a safe and effective treatment for numerous diseases characterized by pathological muscle hypertension. In patients suffering from dystonia or spasticity, it has been observed that use of BTX A results not only in muscle relaxation but also frequently relieves associated pain. This pain relief is often seen earlier and to a much greater extent than the muscular relaxation itself. This has led to extending the use of BTX A to treat various focal pain syndromes. The results of initial studies in specific musculoskeletal pain therapy suggest that BTX A infiltrations are effective in the treatment of chronic, therapy-resistant pain of the shoulder and back region. Furthermore, BTX A has been found to be a less invasive option for the treatment of chronic epicondylitis and similar tendonitis conditions. The healing process following rupture of tendons or muscle transfer operations may be improved. In adults with increased muscle tone and endoprostheses, the targeted relaxation of spastic muscles might increase the lifetime of the implant and diminish aseptic loosening. In children with cerebral palsy, prophylactic treatment of hip luxation appears possible. The doses used in pain therapy are low; if correctly applied, the tolerance and safety are high and the effect lasts for a number of weeks.
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PMID:[Botulinum toxin A in orthopedic pain therapy]. 1558 99

Hypertonia in children can be caused by many different diseases. The most common etiology is cerebral palsy. Spasticity and dystonia are the most common types of hypertonia. There are few options for treatment, and usually treatment has an incomplete effect. Therefore, it is necessary to prioritize goals in order to improve overall functional outcome. The use of any intervention will require the ability to verify the magnitude and importance of the outcome in order to ensure that therapy is efficacious. In general, a complex, flexible, and multifaceted approach will be necessary to improve the motor abilities of children with hypertonia.
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PMID:Hypertonia in children: how and when to treat. 1622 66

Hypertonia, which results from motor pathway defects in the central nervous system (CNS), is observed in numerous neurological conditions, including cerebral palsy, stroke, spinal cord injury, stiff-person syndrome, spastic paraplegia, dystonia and Parkinson disease. Mice with mutation in the hypertonic (hyrt) gene exhibit severe hypertonia as their primary symptom. Here we show that hyrt mutant mice have much lower levels of gamma-aminobutyric acid type A (GABA(A)) receptors in their CNS, particularly the lower motor neurons, than do wild-type mice, indicating that the hypertonicity of the mutants is likely to be caused by deficits in GABA-mediated motor neuron inhibition. We cloned the responsible gene, trafficking protein, kinesin binding 1 (Trak1), and showed that its protein product interacts with GABA(A) receptors. Our data implicate Trak1 as a crucial regulator of GABA(A) receptor homeostasis and underscore the importance of hyrt mice as a model for studying the molecular etiology of hypertonia associated with human neurological diseases.
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PMID:Trak1 mutation disrupts GABA(A) receptor homeostasis in hypertonic mice. 1638 Jul 13

Maple syrup urine disease (MSUD) is an inborn error of metabolism resulting from a defect in the oxidation of the branched-chain amino acids leucine, isoleucine, and valine. Patients present in early infancy with brain edema; delay in diagnosis and treatment is common and associated with residual neurologic damage, which includes alternating muscular hypotonia and hypertonia, dystonia, and seizures. These signs can result in trauma, especially to the anterior maxilla, which is the most traumatized region. In patients with MSUD, a fixed prosthesis is recommended because a removable one can be dangerous because of the risk of aspiration. Rehabilitation, using dental implants, is especially challenging in these patients because of the strong muscular forces of the tongue and lips. An implant-supported fixed prosthesis might provide an effective functional, esthetic, and predictable solution for patients with late-treated MSUD. The present report describes a 10-year follow-up of the successful, posttraumatic use of a dental implant to replace an anterior maxillary tooth in a patient with MSUD.
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PMID:Posttraumatic dental implant placement in a patient with maple syrup urine disease. 1676 96

Cerebral palsy is the main cause of immobility in children. This motor dysfunction is caused by several motor components such as weakness, lack of motor control and muscle hypertonia. Drug treatment, delineated in this review, mainly addresses the latter. Recently, new definitions for clinical features of hypertonia in children were published, assisting the distinction between the two common motor symptoms in cerebral palsy, spasticity and dystonia. The main functional symptoms disrupt functional daily life, dictating the overall approach and the specific drug treatment. There are an increasing number of treatments for this distressing disorder. For general spasticity, treatments provided include Baclofen. If symptoms are local, either dystonia, or spasticity, Botulinum toxin is the revolutionary drug used with significant success and relatively few side effects. For generalized dystonia, a trial of both Dopamine and Trihexyphenidyl should be considered. Cerebral palsy, like other complex disorders, requires individualized decision-making and a team approach. Drug therapy is only one aspect of treatment, yet sometimes it may serve as a window of opportunity to facilitate better motor control.
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PMID:[Drug treatment for children with cerebral palsy]. 1690 Jul 42


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