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
Strategies for controlling dystonia. Overview of therapies that may
Department of Neurology, Mayo Clinic Scottsdale, AZ 85259, USA.
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
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]
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]
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,
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