Manejo de Distonias
Neurology 2000;55(12 Suppl 5):S2-8
Idiopathic cervical dystonia: an overview.
Barrow Neurological Institute, Div. of Neurology, Phoenix, AZ 85013, USA.
Idiopathic cervical dystonia (CD) is the most common of the adult-onset focal dystonias. It manifests as involuntary muscle contractions that cause twisting or turning of the neck. The resulting abnormal head and neck postures are heterogeneous in their presentation. Because of the variable presentation of the disease and the poor recognition of its clinical spectrum of manifestations, CD is frequently misdiagnosed and accurate diagnosis of the disease is commonly delayed. The pathogenesis of CD remains obscure. However, genetic factors, trauma, the sensory system, and impaired basal ganglia function may all play a role in the development of this disease.
Postgrad Med 2000 Oct;108(5):151-2, 155-6, 159-60 [Texto completo]
Strategies for controlling dystonia. Overview of therapies that may alleviate symptoms.
Department of Neurology, Mayo Clinic Scottsdale, AZ 85259, USA. [email protected]
Dystonia is an involuntary movement disorder characterized by twisting, turning, and posturing. This disorder may affect a single body part or may be more generalized, but the pathophysiology remains unclear. The treatment of choice for most of the focal dystonias is botulinum toxin injections, although oral medications occasionally may be beneficial. Surgical treatment of dystonia may be performed peripherally or centrally but is usually reserved for patients in whom other forms of therapy fail.
J Neuropsychiatry Clin Neurosci 2000 Spring;12(2):219-25
Medication-induced dystonias in nine patients with dementia.
Magnuson TM, Roccaforte WH, Wengel SP, Burke WJ
Division of Geriatric Psychiatry, University of Nebraska Medical Center, Omaha 68198-5580, USA.
Evidence from previous studies of neuroleptic side effects suggests that acute dystonic reactions are rare in elderly patients. The authors report 9 cases of dystonic reactions in patients with dementia following the initiation of antipsychotic medication. The cases are important in documenting that drug-induced dystonias do occur in patients with dementia, that risperidone appears to have contributed to dystonia among elderly patients, and that the categorization of dystonic reactions needs further clarification.
J Clin Neurosci 1999 Jan;6(1):1-8
Dystonia: recent advances.
Department of Clinical Neuroscience, St Vincent’s Hospital, Fitzroy, Victoria 3065, Australia
Dystonia may be primary or symptomatic. Most, if not all, primary torsion dystonias are genetic diseases and manifest as ‘pure dystonia’, without consistent biochemical or neuropathological changes. Symptomatic dystonias may be (a) secondary to drugs or other environmental factors, (b) part of a ‘dystonia plus’ syndrome or (c) part of several heredodegenerative diseases. In the last few years, there have been rapid advances in the genetic classification of primary torsion dystonia. The gene for one form (DYT1dystonia), particularly common in Ashkenazi Jews, has been isolated. In this review, I present a basic clinical overview of dystonia and focus on the recent advances in molecular genetics of primary torsion dystonia (PTD). Treatment of dystonia is a large subject, worthy of a review in itself, and is not covered here. Several of the paroxysmal movement disorders may manifest with dystonia, but these are usually considered separately, as I have done in this review. Copyright 1999 Harcourt
Rev Neurol 1999 Feb;28 Suppl 2:S192-4 [Texto completo en formato PDF]
[Movement disorders: dystonias which are apparently psychosomatic. Torsion
[Article in Spanish] Barbero-Aguirre P
Servicio de Neuropediatria, Hospital Infantil La Fe, Valencia, Espana.
In neuropediatric clinical practice, disorders of movement include a wide diversity of conditions, amongst which the dystonias are uncommon in our practice, although they have to be considered amongst the possible diagnoses in some cases. The great variety of clinical symptoms and age of onset together with the nonspecific, erratic clinical course make diagnosis difficult. Some clinical pictures of genuine torsion disorders may be confused with hysterical conversion disorders, somatizations or Munchausen’s syndrome. Diagnosis requires clinical knowledge of both conditions–torsion dystonia as opposed to hysteria or a conversion reaction–and considerable ability and experience. Genetics and molecular studies have helped to clarify some difficult diagnostic problems and facilitated both diagnosis and treatment. In a diagnostic video session we show the case of a seven year old boy who initially presented with a dystonic disorder. There was some doubt as to the aetiology and different types of treatment were given by different specialists. The true diagnosis was reached after molecular genetic studies.
Presse Med 1999 Feb 13;28(6):312-5
[Treatment of dystonias].
[Article in French] Marion MH
Clinique Turin, Paris.
SYMPTOMATIC TREATMENT: Treatment of idiopathic dystonia depends on the age of onset and the extent of the disease. Symptomatic treatment has made many advances over the last 20 years. CHILDHOOD ONSET GENERALIZED DYSTONIA: In childhood onset cases, it is important first to exclude dopa-responsive dystonia. Most of these patients will respond subsequently to high dose anticholinergics. ADULT ONSET FOCAL DYSTONIA: Treatment of this form has been transformed by botulinum toxin therapy. TREATMENT: The treatment of each focal dystonia (blepharospasm, spasmodic torticollis, oromandibular dystonia, laryngeal dystonia, writer’s cramp, foot dystonia and axial dystonia) are reviewed. Treatments must be titrated carefully. Botulinum toxin injections require precision and must be repeated regularly. A rehabilitation program must be adapted to each individual. Surgery, in very severe cases, requires an extensive well-planned discussion of indications.
Presse Med 1999 Feb 13;28(6):306-11
[Secondary dystonias. Clinical analysis and diagnostic approach].
[Article in French] Gallouedec G, Sangla S, Jedynak CP, Vidailhet M
Service de Neurologie, Hopital Saint-Antoine, Paris.
DEFINITIONS: Dystonia is a muscle contraction disorder marked by sustained involuntary clonic contortions or abnormal posture. Primary dystonias can be divided into familial forms related to genetic anomalies and idiopathic forms. Secondary dystonias are related to an underlying neurological disease. METABOLIC DISEASES: Secondary dystonias related to metabolic diseases generally occur early before puberty although late onset forms have been described. Other signs, in association with the dystonia, include mental retardation, epilepsy,
cerebellous or pyramidal signs, oculomotor disorders, or a neuropathy. Occasionally, extraneurological signs suggest the diagnosis. Biological markers are known for most of these dystonias. EVENT-RELATED DISEASES: In some cases, the dystonia is the only sign and develops as a sequela to an earlier neurological event such as neonatal anoxia, trauma, vascular event or adverse effect of neuroleptics. HEMI-DYSTONIAS: Dystonias limited to one side are generally secondary.
J Neurol 1998 Aug;245(8):511-8
Hartmann A, Pogarell O, Oertel WH
Neurologische Klinik, Philipps-Universitat Marburg, Germany.
Secondary or symptomatic dystonias are (1) often accompanied by other neurological deficits. (2) begin suddenly at rest and occur at rest from the onset, (3) are associated with different hereditary and environmental causes. From an aetiological point of view, secondary dystonias can be caused by focal brain lesions of various origin, neurodegenerative disorders, metabolic disorders of the central nervous system (CNS), and several drugs and chemicals that affect the basal ganglia, thalamus and brain stem. Furthermore, secondary (focal) dystonias can be caused by peripheral injury. In the following review, we will discuss epidemiology, genetics, pathogenesis, neuroimaging, neuropathology, clinical manifestation, clinical course and differential diagnosis of secondary dystonias. Therapeutic options are given depending on the aetiology and the topological type of dystonia.
Neurology 1994 Aug;44(8):1374-8
Dystonia after head trauma.
Lee MS, Rinne JO, Ceballos-Baumann A, Thompson PD, Marsden CD
University Department of Neurology, Institute of Neurology, Queen Square, London, UK.
Dystonia is a rare consequence of head trauma. We describe 10 such cases and review 19 similar patients reported in the literature. Twenty-two of the 29 patients suffered head injury during the first or second decade of life. There was a variable delay between the head trauma and the onset of dystonia. In 18 cases with severe head injury, this interval (median, 18 months; range, 1 month to 9 years) was longer than in 11 cases with mild head injury (median, 14 days; range, 3 days to 5 years). In our series, nine of the 10 cases started as a focal dystonia and one as a hemidystonia. The dystonia progressed and spread over several months or years. Two cases remained as focal dystonias, but the others developed segmental, hemi-, multifocal, or generalized dystonia. On brain imaging studies (CT or MRI), the most frequent lesion site was in the contralateral basal ganglia or thalamus, but two cases had normal brain scans. Dysfunction of the lenticulothalamic neuronal circuit seems to be related to the development of dystonia following head trauma.
Review of reported cases