Dystonias are sustained involuntary muscle contractions of antagonistic muscle groups in the same part of the body that lead to abnormal postures or jerky, twisted, intermittent spasms that may have similarities with tremors, athetosis or choreo. Dystonia may be primary or secondary, generalized, focal or segmental. The diagnosis is made clinically. Botulinum toxin injections used for the treatment of focal or segmental dystonias. The treatment of severe generalized dystonia may require a combination of oral anticholinergics, muscle relaxants and benzodiazepines. Is a severe segmental or generalized dystonia refractory to treatment, surgery may be necessary.
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Dystonias are sustained involuntary muscle contractions of antagonistic muscle groups in the same part of the body that lead to abnormal postures or jerky, twisted, intermittent spasms that may have similarities with tremors, athetosis or choreo. Dystonia may be primary or secondary, generalized, focal or segmental. The diagnosis is made clinically. Botulinum toxin injections used for the treatment of focal or segmental dystonias. The treatment of severe generalized dystonia may require a combination of oral anticholinergics, muscle relaxants and benzodiazepines. Is a severe segmental or generalized dystonia refractory to treatment, surgery may be necessary. Dystonia may be primary (idiopathic) Secondary to CNS disorders or drugs to the CNS disorders that can lead to dystonia include, Wilson’s disease pantothenate kinase-associated neurodegeneration [PKAN] in connection with PANK2 mutations [previously Hallervorden-Spatz- disease] Various lipidoses multiple sclerosis cerebral apoplexy brain hypoxia Among the drugs that cause the most common dystonia include antipsychotics (eg. as phenothiazines, thioxanthenes, butyrophenones) antiemetics (eg. as metoclopramide, prochlorperazine) Disordered movements that a athetosis or choreo seem to be, can be caused by dystonia. Classification dystonias are classified based on etiology Clinical symptoms The etiology is categorized as Inherited: Has a proven genetic origin (formerly known as primary) and includes a disorder with autosomal dominant, autosomal recessive or X-linked inheritance. Idiopathic: Can family or sporadically be Acquired: are in connection with neuroanatomical abnormalities due to other diseases Clinical criteria: Outbreak: Can occur at any age, from infancy to adulthood body distribution: Can focally be (limited to one part of the body) (segmental including ? 2 more contiguous body parts, such as the upper and lower face or the face and neck), multifocal (including ? 2 non-adjacent parts of the body such as the neck and leg), generalized (hull plus 2 different body parts) or hemikorporal (one half the body, also called hemidystonia) temporal pattern: Can static, progressive, paroxysmal or persistent and may have diurnal variations or (through certain tasks task-specific dystonia) are triggered Isolated (No indication e ine other movement disorder) or combined (Ddrch accompanied other involuntary movements [other than tremor], but mostly tips dystonia) and risks Pull antipsychotics and antiemetics as causes of sudden, unexplained dystonia into consideration. Primary generalized dystonia (DYT1 -Dystonie) This rare dystonia is progressive and characterized by persistent, often bizarre postures. It is often inherited as an autosomal dominant disorder with partial penetrance due to mutations in the gene DYT1; for some family members, the gene is minimally expressed. Asymptomatic siblings of patients (carriers) can not have a full-blown form of the disease. Symptoms of primary generalized dystonia usually begin in childhood with inversion and plantar flexion of the foot when walking. First, the dystonia may affect only the torso or a leg, but it proceeds often affects the whole body, it moves generally cranial. Patients with the most severe form can be twisted in grotesque, almost fixed postures and eventually confined to a wheelchair. Symptoms that begin in adulthood, affect usually only on the face or arms. Mental function is usually erhalten.Dopa-responsive dystonia This rare dystonia is hereditary. Symptoms of dopa-responsive dystonia usually begin in childhood. Typically, one leg is affected first. As a result, the children tend to go on tiptoe. Symptoms are worse at night. Walking is increasingly difficult, and arms and legs are affected. However, some children have only mild symptoms such as muscle cramps after exercise. Sometimes the symptoms appear later in life and are similar to those of Parkinson’s disease. The motion may be slow, it may be difficult to keep your balance, and a tremor in the hands may occur at rest. The symptoms are reduced drastically when low doses are used by levodopa. If levodopa relieves symptoms, this confirms the Diagnose.Fokale dystonia dystonia These relate to an individual part of the body. They usually begin in adults after the age of 20-30 years. Initially, the poses may occur intermittently or job related (and thus sometimes described as convulsions). The movements are more prominent at lower activity and at rest; these differences increase with time, however, often leads to a distortion of the affected body part and to severe disability. Pain, however, is uncommon except in focal primary dystonia of the neck (cervical dystonia) and the dystonias that occur in Parkinson’s disease when the response to subside levodopa starts (are most commonly affect the lower extremity, the z. B. an inversion of the foot cause). Motion induced dystonia existence of activity-related focal dystonic spasms triggered by executing learned movements (z. B. writer’s cramp, musician-dystonia, Yip the golfer). The spasmodic dysphonia consists of a strained, hoarse or raspy voice, which is caused by a focal dystonia of the laryngeal muscles. The cervical dystonia manifests itself in involuntary tonic contractions or intermittent spasms of dystonia HalsmuskulaturSegmentale This dystonia affecting ? 2 adjacent body parts. The Meige syndrome (blepharospasm plus oromandibular dystonia) consists of involuntary blinking, gnashing of teeth and grimacing and usually begins in late adulthood. It is distinguished from the buccal-lingual-facial chorea in tardive dyskinesia and tardive dystonia (a variant of tardive dyskinesia). Diagnosis Clinical Investigation The diagnosis of dystonia is clinically. Therapy for generalized dystonia: anticholinergics and / or muscle relaxants in focal or segmental dystonia: botulinum toxin injections to paralysis of the muscles (Chemodenervation) Partial neuro surgery In generalized dystonia is most commonly an anticholinergic (e.g., Trihexyphenidyl 2-10 mg po 3-. times / day benztropine used 3-15 mg pO once daily). It’s getting to the target dose titrated. A muscle relaxant (usually baclofen), a benzodiazepine (eg. As clonazepam), or both may be of additional benefit. Generalized dystonia, which is difficult or does not respond to drug may be with deep brain stimulation of the globus pallidus interna (GPI), a stereotactic neurosurgical procedures treated. In some cases, stereotactic unilateral ablative surgery of the GPi is displayed. In focal or segmental dystonias or generalized dystonia, which relates primarily to a part of the body, the treatment of choice in the injection of purified botulinum type A or B to the affected muscles, with or without electromyographic guide and by an experienced physician. Botulinum toxin reduces excessive muscle contractions by Chemodenervation, but it does not change the abnormal wiring in the brain that causes dystonia. The injection of the toxin is most effective in blepharospasm and torticollis, but they can be very effective with most other forms of focal dystonia. The dosage varies greatly. The treatments have to be repeated every 3-4 months, as the duration of action of the toxin is limited. In a few cases, however, the treatment loses effectiveness on when the toxin is injected repeatedly since the formation of neutralizing antibodies against the toxin protein; Not all antibodies that develop, the toxin neutralizing. Important points dystonia cause abnormal postures and / or crooked, jerky movements. Focal dystonias are common and usually start in adulthood. Generalized dystonia is usually the result of disease or medication, and rarely a primary disease. The diagnosis is made clinically. Treat generalized dystonia with anticholinergic and / or muscle relaxants; treat focal or segmental dystonia and generalized dystonia, the v. a. influencing a body portion with botulinum toxin injections.