Dystonia Definition - What Is Dystonia ?

What is dystonia?

Dystonia is a rare neurological disorder characterized by motor disorders, prolonged contraction of muscles or muscle groups and / or repetitive motion. Sometimes, a part of the body as a result of the condition can take an unnatural position. A difference with tics is that the phenomenon by the patient is not to suppress.

Dystonia may occur in one part of the body (focal), for example, the eyelids, or one of the limbs, but also in multiple parts of the body at the same time (segmental, if these are adjacent to each other; multifocal, if these are not adjacent to each other). An extreme example is generalized idiopathic dystonia, in which all the muscle groups of the body may be involved. Usually, the condition makes it after some years of deterioration show a stabilization. Spontaneous remission is observed in a limited number of cases (10 to 15%), although there still may then be a relapse.

 Dystonia is a rare neurological disorder characterized by motor disorders Dystonia Definition - What Is Dystonia ?

The disease affects both men and women and in all age categories, but more often in the elderly. Dystonia may be due to hereditary predisposition, but also occur spontaneously with no identifiable cause. With regard to the degree of invalidity is to establish a relationship with the age at which the disease manifests itself for the first time. The sooner they act, the more likely that multiple muscle groups will be affected, especially in children (generalized dystonia). The disease can lead to total disability, but in itself is not lethal. The intellectual power remains unaffected.

Primary (idiopathic) and secondary (symptomatic) dystonia

Most patients have a so-called primary dystonia. That is to say that there is no cause for the dystonia is found after medical examination. Such patients have no birth trauma, a normal physical and mental development, no epilepsy, show no signs of damage to pyramidal, cerebellar or visual systems. They do not exhibit the Wilson's disease brains and scans no abnormalities.

In secondary dystonia, there is a condition or other cause which causes the dystonia. It would take too long to detail here to go into all facets of disorders that can cause dystonia. However, three things still deserves special attention.
  • Wilson's disease is to be excluded. To this end, the mirror ceruloplasmine and copper are determined, and examined the eyes and liver.
  • Brain scans show well see if there are structural changes in the brains that could cause dystonia.
  • Antipsychotics and anti-Parkinson agents is very well known that they can cause movement disorders. Especially if symptoms longer than a few weeks his manifesto, to handle these well.
Early onset and late onset dystonia
These terms indicate at what age the dystonia was first manifested in the patient. One makes this distinction Because The age or at is related to the anatomical location where the dystonia occurs. Early onset dystonia Initially Involves Usually an arm or leg, and less comm only the neck, vocal cords or muscles skull. For late onset primary dystonia Approximately holds the reverse: Initially Concerned mostly the skull or neck muscles, and lessoften poor, and only very rarely a leg.

The prevalence of primary dystonia ranges from 50 per million for early onset dystonia, and 30-7320 per million for late onset dystonia. However, the quality of the thesis studies where figures are based on, is not always good. The data from a number of specific groups of people is much more reliable. The prevalence of early onset dystonia in Ashkenazi Jews in New York is 111 per million and 600 per million for late-onset dystonia in northern England, and 3000 per million for late onset dystonia Italians over 50 years.

Dystonia treatment

Physical therapy and braces serve to improve posture and contractures, with continuous movement to avoid possible reduction. Especially by children braces are not well tolerated. In contrast, some patients with cervical dystonia may benefit greatly from. Especially if they themselves have already discovered a sensory trick. That is, that they have discovered in themselves that by neck or head in a certain way to touch, or support the head without too much difficulty keeping late in the desired position. With a neck / head brace can than the effect of the sensory trick be imitated. The patient then has his arm and hand free again. Finger Splints and intense finger exercises may reduce symptoms in musicians with focal handdystonie. The use of a splint or brace for the treatment of a focal dystonia of the arm can be effective, however, the immobilization of a limb dystonia, dystonic may also worsen. An alternative to immobilization constraint induced movement therapy, which is also used for rehabilitation after a stroke.

Botulinum Toxin
With the introduction, in the 80 of botulinum toxin in the medical practice, they achieved tremendous progress in the treatment of dystonia. Botulinum toxin can be used for the treatment of various focal dystonia including blepharospasm, oromandibular dystonia, writer's cramp and other occupational cramps. Most of the experience gained in the treatment of cervical dystonia. A large number of clinical studies have shown that treatment with botulinum toxin is safe and effective, and in addition, a positive effect on the quality of life. Because the product contains protein, resistance can occur. However, this happens rarely, in less than 1 per cent of the cases. Botulinum toxin is now considered as a drug of first choice in the treatment of cervical dystonia. It is administered by intramuscular injection, usually every 3 months.

Other drugs
A small group of patients, 5 percent of children with dystonia, can benefit from the use of a dopaminergicum (levodopa). Because the response to levodopa may well be dramatic, may be considered in all children and adolescents with generalized or segmental dystonia, starting with a trial therapy with levodopa. A typical regime is 100 mg levodopa, together with 25 mg of a decarboxylase inhibitor (e.g., benserazide), in order to increase the effectiveness of levodopa.

Baclofen, a GABA-B agonist, may be effective, particularly in the case oromandibular dystonia, and in children. Dose of 40-180 mg per day.

Antidopaminergic resources should be seen as no longer used because of their lack of efficacy and the frequent occurrence of side effects. An exception is tetrabenazine.

Anticholinergic agents such as trihexyphenidyl are effective in the treatment of dystonia in which larger portions of the body are affected. Start with 2 mg at bedtime, and raise for 4 weeks to 12 mg per day. Some patients may require higher doses, up to 100 mg per day. The risk of side effects such as drowsiness, urinary retention (retention) and a very dry mouth take then. Pyridostigmine, pilocarpine eye drops and artificial saliva can reduce these symptoms.

Often patients need a combination of resources. Muscle relaxants such as benzodiazepines (diazepam, lorazepam, or clonazepam), tizanidine, orphenadrine may be added to anticholinergics, such as that alone are not sufficiently effective. Clonazepam may in particular at blepharospasm and at dystonia with short pulling movements, are effective.

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