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)

Since the introduction of botulinum toxin (BTX) as a therapeutic tool in the 1970s, the number of uses for this substance has increased exponentially. BTX's mechanism of action involves degrading the SNARE proteins blockading the release of acetylcholine into the neuromuscular junction. In many body systems, decrease of contractility, strength, and tension of certain muscle groups result in improved clinical outcomes. Applications now include cosmetic, gastroenterologic, otolaryngologic, genitourinary, neurologic, and dermatologic uses. In fact, BTX can be considered as a potential treatment in any situation involving inappropriate or exaggerated muscle contraction. Currently, the FDA has approved BTX-A (Botox) for treating glabellar lines, blepharospasm, strabismus, hemifacial spasm, cervical dystonia, and spasticity. With the addition of cosmetic applications to the FDA's approval list, the use of BTX has increased dramatically.
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PMID:Noncosmetic uses of botulinum toxin. 1515 50

Botulinum toxin A (BoNT/A), a neurotoxin, is effective for treating a variety of disorders of involuntary muscle contraction, including cervical dystonia, blepharospasm and hemifacial spasm. It inhibits neurouscular signaling by blocking the release of acetylcholine at the neuromuscular junction. The biological effects of the toxin are transient with normal neuronal signaling returning within approximately 3-6 months post injection. Recently, clinical findings suggest that BoNT/A may inhibit pain associated with migraine and other headache types. The mechanism by which this toxin inhibits pain is under investigation, recent findings suggest that it inhibits the release of neurotransmitters from nociceptive nerve terminals and in this way may exert an analgesic effect. A number of retrospective open-label chart reviews and three placebo-controlled double-blind trials have demonstrated that localized injections of BTX-A significantly reduce migraine frequency, severity, and migraine-associated disability. The majority of patients in these studies experienced no BoNT/A mediated side effects; however, a small percentage of patients did report transient minor side effects including blepharoptosis, dipolpia, and injection-site weakness. Currently there are several large-scale randomized, placebo-controlled clinical trials in progress evaluating the efficacy, optimal dosing and side effect profile of this toxin as a novel treatment for migraine and other headache types. These studies may provide further evidence that BoNT/A is an effective option for the preventive treatment of migraine.
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PMID:Botulinum neurotoxin for the treatment of migraine and other primary headache disorders. 1515 49

Dysphagia is a known adverse effect of botulinum toxin injection into the cervical region for dystonia. We present an unusual case of dysphagia arising from injection into the orbicularis oculi muscle, which has hitherto not been described. We postulate that her dysphagia was caused by distant side effects of botulinum toxin due to repeated injections. We recommend that clinicians should restrict the frequency of injections to as few life-time doses of the toxin as possible for adequate management of spasm. The practice of re-injecting patients routinely every three months, or at the first return of mild spasms should be discouraged.
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PMID:Dysphagia as a side effect of botulinum toxin injection. 1577 92

Idiopathic cervical dystonia (ICD) is the most common form of focal dystonia. A characteristic and unique diagnostic feature is the presence of "sensory tricks", for example, a finger placed on the chin to neutralize the spasm. Although approximately 70% of patients with ICD experience effective sensory tricks, the exact mechanism of these tricks is still unknown. However, recent evidence suggests that higher sensorimotor integration processes are involved. A study using H2(15)O positron emission tomography demonstrated that the application of sensory trick stimulus, resulting in a near-neutral head position, led to an increased activation mainly of the superior and inferior parietal lobules (ipsilateral to head turn) and bilateral occipital cortex and to a decreased activity of the supplementary motor area and the primary sensorimotor cortex (contralateral to head turn). Since transcranial magnetic stimulation (TMS) is an experimental device with the ability to excite or depress the neural circuits, we hypothesize that the use of TMS of specific parameters to specific brain areas (as above) may produce an effect similar to sensory tricks resulting in the relief of spasms and the improvement of cervical dystonia.
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PMID:Improvement of cervical dystonia: possible role of transcranial magnetic stimulation simulating sensory tricks effect. 1578 Apr 89

The authors report a Chinese boy with a DYT1 gene mutation having muscle stiffness, severe painful muscle spasm, myoclonus, and dystonia compatible with stiff child syndrome. Autoantibodies to glutamic acid decarboxylase (anti-GAD) were absent. His asymptomatic mother had a DYT1 mutation. His asymptomatic sister has diabetes mellitus and antibodies to glutamic acid decarboxylase but no DYT1 mutation.
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PMID:Stiff child syndrome with mutation of DYT1 gene. 1668 92

The increasing use of botulinum toxin type-A, especially for focal dystonia and spasticity has highlighted the issue of secondary non-responsiveness. Within the last few years botulinum toxin type-B (Myobloc/Neurobloc) has become commercially available as an alternative to type-A. This paper discusses our initial experience of botulinum toxin type-B in a total of 63 individuals who attended our botulinum clinic. Thirty-six patients had cervical dystonia and a secondary non-response to type-A toxin. Thirteen of these patients (36%) had a reasonable clinical response to Neurobloc and continue to have injections. The other 23 patients either had no response, or a poor response, or had unacceptable side effects and ceased treatment. A small number of people with blepharospasm, hemifacial spasm and foot dystonia also had a disappointing response to injection. Twenty patients with spasticity were also type-A resistant. Seven of these show some continuing response to type-B, without unacceptable side effects. These findings demonstrate that botulinum toxin type-B has a place in the management of patients who have become non-responsive to type-A, but overall the responses to type-B toxin were disappointing.
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PMID:The use of botulinum toxin type-B in the treatment of patients who have become unresponsive to botulinum toxin type-A -- initial experiences. 1632 88

A 58-year-old man with progressive supranuclear palsy (PSP) developed two episodes of respiratory failure associated with laryngeal spasm. It was revealed he had adductor laryngeal breathing dystonia, a relatively unrecognized complication of PSP.
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PMID:Laryngeal dystonia causing upper airway obstruction in progressive supranuclear palsy. 1659 2

Botulinum neurotoxin type B (BT, BT-B) has been used as NeuroBloc/MyoBloc since 1999 for treatment of cervical dystonia, hyperhidrosis, spastic conditions, cerebral palsy, hemifacial spasm, bladder dysfunction, spasmodic dysphonia, sialorrhoea, anal fissures, piriformis syndrome, various pain conditions and cosmetic applications. Generally, its therapeutic effects are comparable to BT type A (BT-A). The adverse effect profiles of BT-B and BT-A, however, differ considerably. BT-B has been found to produce more regional as well as systemic anticholinergic adverse effects, such as dryness of mouth, accommodation difficulties, conjunctival irritation, reduced sweating, dysphagia, heartburn, constipation, bladder voiding difficulties and dryness of nasal mucosa. In BT-B the relationship between autonomic and motor effects known from BT-A is substantially shifted towards autonomic effects. BT-B, therefore, should be used carefully in patients with autonomic disorders and in patients with concomitant anticholinergic therapy. If NeuroBloc/MyoBloc is used to treat cervical dystonia patients with antibody-induced failure of BT-A therapy, 86% of those will develop complete secondary therapy failure after five applications. If NeuroBloc/MyoBloc used to treat cervical dystonia patients without prior exposure to BT, 44% of those will develop complete secondary therapy failure after nine applications. NeuroBloc/MyoBloc, therefore, is associated with substantial antigenicity problems originating from a particular low specific biological potency. Systemic anticholinergic adverse effects and high antigenicity limits the clinical use of NeuroBloc/MyoBloc considerably.
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PMID:Clinical use of non-A botulinum toxins: botulinum toxin type B. 1678 8

Since its development for the use of blepharospasm and strabismus more than 2.5 decades ago, botulinum neurotoxin (BoNT) has become a versatile drug in various fields of medicine. It is the standard of care in different disorders such as cervical dystonia, hemifacial spasm, focal spasticity, hyperhidrosis, ophthalmological and otolaryngeal disorders. It has also found widespread use in cosmetic applications. Many other indications are currently under investigation, including gastroenterologic and urologic indications, analgesic management and migraine. This paper is an extensive review of the spectrum of BoNT clinical applications.
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PMID:Botulinum toxin: clinical use. 1687 Apr 87

Botulinum toxin type-A (BoNT-A) prevents the release of acetylcholine at cholinergic junctions, thereby causing temporary muscle weakness lasting 3-4 months. It is now widely used to treat a broad range of clinical disorders characterized by muscle hyperactivity. BoNT-A has proved effective in the management of several neurological conditions and, in particular, in the management of movement disorders (e.g. blepharospasm, cervical dystonia, laryngeal dystonia, limb dystonia, hemifacial spasm, focal tics, tremor and other hyperkinetic disorders). As a treatment of spasticity, BoNT-A can improve mobility and dexterity as well as preventing the development of distressing and costly secondary complications. In cerebral palsy, BoNT-A is of value, being able to delay or even avoid surgery until motion patterns have become established.
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PMID:Clinical value of botulinum toxin in neurological indications. 1711 46


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