Botulinum toxin (BT) therapy is the most effective way of relaxing dystonic muscle hyperactivity. BT produces a strictly local, well controllable and fully reversible blockade of the cholinergic neuromuscular synapse for approximately 3 months. Its application is based upon an injection scheme including the target muscles and their BT doses. The development of appropriate injection schemes greatly depends on the experience of the injector. Most cases of “therapy failure” are actually caused by inappropriate injection schemes and are therefore “therapist failure”. Best results are obtained in focal dystonia. However, recent publications indicate that total BT doses are higher than previously thought; thus, more widespread dystonia can also be targeted [3]. Dystonic muscle activity can also be suppressed by surgical interventions. Deep brain stimulation (DBS) blocks certain basal ganglia pathways by continuous high frequency stimulation through stereotactically implanted electrodes. Target of the stimulation is the globus pallidus internus. For tremor-type dystonia thalamic targets may also be used. DBS works best in idiopathic dystonia. It has a far-reaching effect and is therefore recommended for non-focal dystonia. A combination of posterior ramisectomy, peripheral denervation and myectomy of the sternocleidomastoid muscle is called the Bertrand procedure [3]. Before BT therapy it was commonly used with some success to treat cervical dystonia. Other selective denervations and myectomies may be used in special forms of very localised dystonia. Spinal cord stimulation turned out not to be effective.

A large number of drugs were tried to treat dystonia. In general, drug treatment of dystonia is disappointing as it is usually ineffective and often accompanied by severe adverse effects. Best antidystonic effects are produced by anticholinergic drugs, especially in children tolerating high doses. Antidopaminergic drugs may also be helpful. Dopamine receptor blocking agents, however, bear the risk of inducing tardive dystonia; dopamine-depleting agents do not, but often produce parkinsonism and depression. GABA-ergic compounds have also documented antidystonic efficacy. Continuous intrathecal baclofen administered through an implanted pump was tried with mixed results in non-focal dystonia [5].

Analgesics may be used if there are painful secondary complications and when the painful dystonic muscle activity cannot be sufficiently suppressed otherwise. Anxiolytics can reduce stress as a dystonia facilitating factor. Knowing that there is the option to use them sometimes helps.

Rehabilitation of dystonia includes a number of therapeutic interventions such as physiotherapy, occupational therapy, speech therapy, psychotherapy and the use of orthoses. Additionally, counselling of the patient and his/her family plays an important role. The goals of rehabilitation of dystonia include re-training of normal postures and movement patterns, prevention and therapy of secondary complications and coping with pain and the burden of a chronic disease.

As dystonia is a chronic condition and there is no cure for it, rehabilitation of dystonia needs to be provided continuously. The combination of methods applied and their frequency and intensity depend on the severity of the patient’s individual dystonia. It is best offered on an outpatient basis within the patient’s community. This concept of rehabilitation of dystonia, however, does not match the traditional concept of rehabilitation as a short intense inpatient intervention to correct an acute deficit. Structures to provide appropriate rehabilitation of dystonia are lacking in many health care systems. One strategy to overcome this deficit is the Interdisciplinary Working Group for Movement Disorders (IAB) [6]. It is a network of medical and non-medical and inpatient and outpatient movement disorder therapists dedicated to interdisciplinary collaboration. It is based on regional groups backed up by a central office providing various data bases, educational material and scores and scales to harmonise the evaluation of movement disorders and their therapies. Recently, the IAB started the IAB Academy, providing government-accredited, structuralised education for movement disorder specialists.

Another problem of rehabilitation of dystonia is the lack of academic and scientific structures in many countries. Compared with other health care sectors the disease terminology and the descriptions of interventions are often ambiguous. Coupled with a general lack of robust studies, it is therefore difficult to compare and evaluate the efficacy of the rehabilitative interventions offered [7].

Figure 4.1 shows some typical patients with cervical dystonia. BT therapy is the treatment of choice for cervical dystonia. In patients with special forms of dystonia, such as antecollis, antecaput, tremor forms or alternating forms, and in patients with antibody-induced therapy failure, DBS is an alternative. Antidystonic drugs and adjuvant drugs may be used at the end of the BT treatment cycle. When BT therapy is used, physiotherapy is necessary in most patients to activate antidystonic muscles and to re-learn the normal head position. It also helps to mobilise existing contractures or to prevent them. Based on these principles and on several decades of practical experience, Bleton has developed a physiotherapeutic training programme [8] that is now used in many countries around the world. However, a recent study challenged its efficacy [8]. Other studies demonstrated that physiotherapy may reduce BT doses [10, 11].

Behavioural therapy is mainly based on electromyographic feedback techniques. Nine studies used feedback techniques with some success and sometimes continued improvement [12–19]. However, only two of them [14, 16] used a randomised design. Other behavioural techniques [20–22] are based on changes in the sensory input to the central nervous system [23, 24]. Vestibular stimulation [25] and orthosis [25] were occasionally used in small case series with mixed results.

Figure 4.2 shows some typical patients with blepharospasm and Meige syndrome. The treatment of choice for blepharospasm is BT therapy. It produces robust improvement in most patients treated. In some patients, results are less favourable, usually because of additional eyelid opening apraxia (levator inhibition) preventing appropriate activation of the levator palpebrae muscle. Levator suspension operation [27] connecting the upper eyelid to the frontalis muscle via a Goretex^® string is helpful. Alternatively, a spring attached to a spectacle’s frame may help (Fig. 4.3). Patients starting to use plasters usually report skin irritations after a short period of time. DBS is principally effective in blepharospasm; however, BT therapy is considerably less invasive. Peripheral denervation operations are obsolete because of frequent excessive periorbital pain and uncontrollable effects.

Rehabilitation of blepharospasm is mainly based on electromyographic feedback strategies developed in the 1970s [28–33]. Reducing factors facilitating the normal blink reflex, such as bright light and cold air by wearing sunglasses may be helpful.

Laryngeal dystonia (spasmodic dysphonia) is an extremely rewarding indication for BT therapy. Adductor as well as abductor forms respond well. BT may be applied transcutaneously under electromyographic monitoring or transorally under direct visual control. Pharyngeal dystonia often occurring in patients with tardive dystonia may also be treated with BT therapy.

Rehabilitation of spasmodic dysphonia is usually applied as speech therapy. Speech therapy tries to improve breathing, tongue, lip and mandibular movements and tries to maintain a dystonia-free phonation. The basic idea is to focus attention on these single elements and to train for optimal performance. General recommendations include avoidance of stress, of voice overuse and of unfavourable situations, including talking over the phone and noisy places.

Blitzer and colleagues [35] found in a retrospective review of 110 patients with spasmodic dysphonia that speech therapy, psychotherapy and feedback techniques had limited success, whereas one third of patients benefited from systemic medication and all patients improved with BT therapy. Speech therapy may increase the duration of BT therapy in spasmodic dysphonia [35].

Rehabilitation of pharyngeal dystonia is usually aimed at improving swallowing. It is usually applied by speech therapists as swallowing therapy. Again, it is based on focusing attention on the single elements of the swallowing process and on training their optimised performance. General recommendations include changing the food consistency to either liquid or solid, careful food preparation, thorough chewing, eating under optimal conditions (no distraction, upright body position) and repeated clearing of the throat. Nasogastric tube feeding will only rarely become necessary and should be avoided if possible.

Figure 4.4 shows some typical patients with task-specific dystonia. For writer’s cramp and musician’s cramps, the most common forms of task-specific dystonia, BT is the treatment of choice. However, for writer’s cramp one third to half of the patients are not satisfied with BT therapy. For musician’s cramps, with their excessive functional demands, this percentage is higher. Oral antidystonic drugs are problematic owing to weak and unpredictable effects and potentially severe adverse effects. In musician’s cramps, however, trihexyphenidyl is frequently used [36]. Adjuvant drugs, especially anxiolytics, may be additionally helpful. DBS was tried in single cases with mixed results. In this situation, rehabilitation is in high demand, especially in patients with musician’s dystonia when their ability to perform professionally is at stake.

Originally, it was thought that task-specific dystonia was a functional disorder, a software problem triggered by overuse. Although overuse, especially under stressful circumstances, seems to play a major role in the pathophysiology of task-specific dystonia, it has become increasingly apparent that the pathological basis of task-specific dystonia is not principally different from that of other forms of dystonia. Still, the strong correlation with special motor programmes is intriguing and has stimulated treatment with movement-modifying programmes.

Over many years of practical work with writer’s cramp patients, Bleton developed a physiotherapy programme based on the concept of re-learning normal movements by improving independence and the precision of individual finger and wrist movements and training of antidystonic muscles by drawing loops, curves and arabesques [37]. Occupational therapy is even more widely used and employs a large number of different strategies, including simple ones such as switching writing hands, writing in print, using keyboards and—where possible—speech recognition devices, changing to thick pens and pens with a non-slippery surface, altering the pen-holding position [38–41], attaching the pen to the hand through special devices [42–45], orthoses, using the wrist more than the fingers and optimising the sitting position, general relaxation techniques [46, 47] and general avoidance of stress and anxiety. Writing in private rather than in public may also avoid aggravating stress. Switching the writing hand can be learned within 6–9 months. However, about half of the patients will also develop writer’s cramp in the switched hand. Occupational therapy seems to have an adjuvant effect on BT therapy [48]. Figure 4.5 shows a collection of modified pens to enable the patient to cope with writer’s cramp.

Additionally, there are numerous more complicated training programmes, usually referred to as behavioural therapies. This term is used here to describe re-learning processes and not psychotherapeutic techniques. Behavioural therapies include biofeedback techniques [49, 50], often electromyography-based [51–53], habit reversal training [47, 54], constraint-induced motion therapy or sensory motor retuning [55], general training exercises [41, 56–58] and supinator writing practice [40]. Transcranial magnetic brain stimulation has been used experimentally [59].