Dystonia is a neurological syndrome characterized by involuntary, stereotyped and repetitive movements of opposing muscles, of a tonic or spasmodic origin, which frequently cause sprains and other abnormal postures and movements.
These dystonic movements are usually continuous and sustained, but their duration and intensity can be modified by several factors:
Emotion, fatigue, relaxation, motor activity, sensory stimuli and sleep. The severity of the movements and dystonic postures varies from confusing or poorly marked symptoms, which are only evident during action, to disabling muscle spasms that prevent the execution of voluntary motor acts, with a typical tendency to recruit adjacent muscles and fix abnormal postures due to contractures segmental.
In correspondence with the location of the muscles involved, dystonias are usually classified as focal, segmental, hemidistonic and generalized dystonia. The latter are also known as muscular dystonias. deforming, have a characteristic evolutionary profile that includes frequent onset by a lower extremity, with postures dystonic lesions that only appear associated with action and that develop axially, until in a period 5-10 years old compromise the trunk and cause scoliosis, kyphosis, lordosis and opisthotonus postures. Its course is inexorably progressive and so far no pharmacological treatments have been found that stop the course of the disease or control it. satisfactorily relieves spasms and dystonic postures.
Although it is still unknown how the Nervous System produces these dramatic motor abnormalities, some advances in the mechanisms The neural mechanisms involved suggest new therapeutic perspectives.
On the other hand, dystonia can coexist with tremor; two basic types of tremor can be observed in patients with dystonia, postural and dystonic. It is possible that the tremor associated with dystonia is actually a form of frustrated with dystonia. In some patients, head and trunk tremors (usually slow at frequencies of 1-5 Hz) may It may precede the onset of dystonia and be the initial manifestation of focal dystonia. Rarely, tremors Cerebellar and parkinsonian disorders can be associated with dystonia and dystonia can be the calling card of Parkinson.
Dystonia may be a symptom of an underlying disorder or entity of a specific disease, in which case it is This is called primary torsion dystonia. Primary torsion dystonia may be sporadic or hereditary and is not associated with cognitive, pyramidal, cerebellar or sensory abnormalities.
In some pathological processes, dystonia can be one of the neurological disorders. For example, dystonia It may be associated with parkinsonism, especially in the early stages of Parkinson's disease or atrophies. multisystem disorders and several secondary forms of parkinsonism.
Dystonic movements are usually exacerbated by voluntary motor activities (action dystonia) such as running, walking, writing, speaking, and performing specific motor tasks, and dystonia frequently increases with age. stress and fatigue. On the other hand, dystonic movements can sometimes be relieved by rest, self-hypnosis, and various sensory tricks (antagonistic gestures) or counterpressure, such as touching the chin or occiput to help combat torticollis.
Dystonic movements usually cease during the lighter stages of sleep; However, the positions Dystonia may persist during all stages of sleep.
Focal dystonia affects isolated muscle groups such as spasmodic torticollis, occupational cramps, foot dystonia, oromandibular dystonia, lingual, pharyngeal, laryngeal (spasmodic dysphonia), some types of bruxism and trismus.
Segmental dystonia affects one or more continuous parts of the body like craniocervical dystonia characterized by a combination of blepharospasm and facial-oromandibular, lingual, pharyngeal, laryngeal and dystonia. cervical. Other categories of segmental dystonia include brachial dystonia (one or both arms, with or without involvement of the the axial or cranial muscles), crural (one leg plus the trunk or both legs) and axial (neck and trunk with or without cranial muscles).
Multifocal dystonia involves one or more non-continuous parts of the body (such as a combination of torticollis and leg dystonia). Hemidystonia, (unilateral dystonia) involves only half of the body and is generally associated with a structural lesion in the contralateral basal ganglia, particularly the putamen.
Generalized dystonia involves the axial and extremity muscles, generally presenting a focal onset with slow progression until compromising the rest of the aforementioned structures, may be due to idiopathic, primary causes and symptomatic.
The therapeutic approach to patients with dystonia is largely determined by the specific diagnosis based on clinical and etiological categorization. The primary goal is to find a potentially curable cause or specifically treatable dystonia. Unfortunately this is only possible in a very small proportion of dystonic patients. Wilson's disease and drug-induced dystonia are potentially causes curable.
The most important progress made in the treatment of dystonia has been local denervation with injections of botulinum toxin (Jankovic and Mallet, 1993). When injected into the affected muscles, said neuromuscular blocker was effective in 95% of patients with blepharospasm, 90% of cases of spasmodic dystonia and hemifacial spasm, 85% of patients with cervical dystonia and the majority of patients with oromandibular and manual dystonia (Brin et al. 1989; Jankovic and Brin, 1991). Although the benefit usually disappears within 3-4 months after the toxin is injected, the treatment is well tolerated and is considered the therapy of choice in most focal dystonias, hemifacial spasm and some forms of tremor (Jankovic, 1991; Jankovic and Schwartz, 1991).
The optimal combination of these therapeutic resources and specialized rehabilitation can improve the general and functional condition of the patient.
In the presence of refractory symptoms or manifestations, severe spasms, disabling postures, pain, etc. or the need to high doses of medications, which cause significant adverse effects to control the symptoms of the disease, can The possibility of surgical treatment using stereotactic functional surgery techniques may be suggested, in which case will proceed first to perform the surgical procedure with prior written informed consent from the patient and then the other therapeutic measures will be carried out.
The Neurological Restoration or Rehabilitation program includes a week of evaluation during which a specialized clinical evaluation of dystonia syndrome, a general clinical evaluation of the condition of the patient's health, a quantification by international scales to measure neurological condition, motor capacity, functional capacity and quality of life, specialized neurophysiological studies in exploration of the motor system and perceptual motor integration, structural and functional imaging studies using Axial Tomography techniques Computerized, Nuclear Magnetic Resonance, Simple Photonic Emission or Molecular Biology techniques, whichThey correlate with clinical findings and the intensity of motor compromise.
This evaluation is complemented by a functional evaluation of the kinetics of movement and gait, studies spirometric, stabilometric, neuropsychological and the application of international instruments to determine the degree of disability or objectify the neurological defect to be modified.
The information obtained by this comprehensive evaluation is analyzed collectively by specialists from various disciplines and is designs a unique program in stages, with specific objectives that are set according to the patient's possibilities and the previously accumulated experience.
The Neurological Restoration or Rehabilitation program is executed in 4-week therapeutic cycles (28 days) which includes hygienic-dietary measures to improve general condition, pharmacokinetic disorders and the nutritional status of the patient, pharmacological adjustment to control spasms and abnormal postures, optimizing the dose and frequency of administration of medications to be prescribed and their periodic adjustment until sufficient control is achieved symptomatic and stability of the motor condition throughout the day. At the same time, disorders of the sleep, appetite, blood pressure and other autonomic or mental alterations that usually coexist with progression of the disease or as a side effect of some drugs.
A comprehensive rehabilitation program is also developed that includes posture, gait and balance training, training of manipulative skills with language training and learning techniques, using methods of feedback, specific motor learning, strengthening and/or stretching techniques and training systems of more efficient specific purposes to stimulate neuroplastic capacities with the comprehensive objective of adapt the pattern of motor behavior and increase the individual's motor capabilities, especially for the execution of tasks everyday.
If the comprehensive Neurological Restoration or Rehabilitation program is applied, will require as many four-week cycles as the evaluation determines, the most common being the application one or two therapeutic cycles annually.
It has been determined in an open clinical trial that the effectiveness of the comprehensive care program is 75% and the ranges of The improvements obtained for motor capacity are approximately 20% and for functional capacity approximately 28%. In In more than 80 patients treated to date, no life-threatening complications, accidents or adverse events have been observed. leave consequences. The rate of adverse effects or transient complications is less than 7%.
Surgical treatment has variable effectiveness and specific indications.
The Vim-Vop Thalamotomy induces more than 90% suppression of the associated tremor or the mobile component of the spasms in the contralateral extremities and approximately 20% overall improvement in dystonic posture. The Posteroventral pallidotomy induces a suppression of spasms in more than 85% of patients and a global improvement of 35 and 40%. The effects of surgery are maintained with little modification until at least 3 years in the most patients.
The training of human resources is guaranteed through the participation of its members in the teaching program of the institution, the training plan for neurology residents, the teaching activities of the subdirectorates of rehabilitation and nursing and internal activities on a weekly basis. International courses are also offered postgraduate degree and training in movement disorders and stereotaxic functional surgery.
The fundamental results observed in these last 5 years of performance include the attention ofmore than 2,000 patients with these alterations, with a demonstrated effectiveness of more than 90% and an efficiency (degree of improvement) that fluctuates between 20-25% (rehabilitation) and 40-60% (surgery), the development of surgical techniques Microrecording-Guided Treatments for the Treatment of Parkinson's Disease, Dystonias, Tremors, and Other Brain Disorders movement and the creation of a new surgical technique (dorsal subthalamototomy) for the most effective treatment of axial disorders in Parkinson's Disease, the improvement of rehabilitation methodology in parkinsonism, ataxia, dystonia and senile gait disorders and the implementation of a combined antioxidant system and other neuroprotective agents.
Personalized Intensive Multifactorial Neuro-Rehabilitation Program
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