Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Benign essential blepharospasm is a focal dystonia consisting in involuntary closure of the eyelids. Until early 80's, therapeutic modalities included only psychotropic drugs, biofeedback and surgery, which showed limited efficiency. Recently, it has been suggested to inject botulinum toxin in affected palpebral orbicularis muscles. Used in several thousands of patients, it has been found that this procedure provides significant temporary relief from spasms of the eyelids. Local side effects are minimal, and no systemic side effects have been demonstrated in patients with blepharospasm treated with botulinum toxin.
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PMID:[The role of botulinum toxin in the treatment of essential blepharospasm]. 225 35

After cessation of long-term treatment with haloperidol, a 77-year-old man developed severe dyskinetic-dystonic movements involving mainly the tongue and associated also with oromandibular dystonia and blepharospasm that were manifested exclusively during the process of eating and that interfered with feeding. There were no spontaneous involuntary movements nor were they apparent during any other simple or complex activations of the orofacial musculature.
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PMID:Tardive eating dystonia. 225 58

Blepharospasm and hemifacial spasm are involuntary movement disorders that affect the facial muscles. They are classified as cranial dystonias. Their cause is unknown and the underlying pathophysiology is poorly understood. Both dystonias are more common in women than in men. It is the middle-aged group that is most frequently affected. Because of their high visibility, these disorders may cause considerable distress and embarrassment. Affected persons are often mistakenly considered to have psychiatric problems. In addition, both dystonias may result in severe disability. For example, the person with untreated blepharospasm may experience social isolation and functional blindness. Recently, therapy in the form of botulinum toxin became available in larger centers. Repeated injections of the toxin usually relieves symptoms and enable patients to resume a former lifestyle. Neuroscience nurses who are knowledgeable about cranial dystonias and the resources that are currently available can retard progression of disability and help restore the individual's quality of life. Informed neuroscience nurses can also play an important role in case-finding, counselling and referral. Two examples are presented in order to highlight some of the complexities inherent in the diagnosis and treatment of each type of cranial dystonia and to further clarify the nurse's role. These examples are based on the personal and professional experience of the authors.
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PMID:Blepharospasm, hemifacial spasm and the nurse's role. 226 30

Botulinum A toxin injection has great utility in the treatment of essential blepharospasm and other facial spasm disorders. Several investigators have noted the failure of botulinum toxin injections to relieve lid spasm in occasional patients and a decrease in effectiveness or duration of effect following multiple injections in other patients. We reviewed the charts of 30 consecutive patients presenting for the evaluation or treatment of facial dystonia. Of 20 patients who had received multiple injections of botulinum toxin, 10 patients were felt to be treatment failures. A new biomechanical system was developed to investigate the duration and degree of paralysis induced in the gastrocnemius muscle of the rat. Animals were treated with four sequential injections at 6-week intervals to the same muscle, resulting in muscle atrophy and an increase in the duration and degree of muscle paralysis, contrary to clinical findings in humans. The review of patient data confirms that, for many patients, repeated injection of botulinum toxin results in a decrease in duration and degree of effect despite an increased toxin dose. An opposite effect was noted in our experimental model because of progressive muscle atrophy.
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PMID:Botulinum A toxin injection. Failures in clinical practice and a biomechanical system for the study of toxin-induced paralysis. 227 81

Thirty-one subjects affected by different movement disorders underwent polygraphic and videotape monitoring during nocturnal sleep, to assess movement patterns during the night. It was possible to distinguish two categories of disorders according to their pattern of movements. In the largest group (Meige's syndrome, blepharospasm, amyotrophic choreoacanthocytosis, Tourette syndrome, tonic foot, hemiballism) abnormal movements were still present during sleep, but decreased in frequency and amplitude in all stages. The second group presented three syndromes (nocturnal paroxysmal dystonia, nocturnal myoclonus, restless legs syndrome), in which light non-rapid-eye-movement sleep induced a strong activation of abnormal movements, whereas rapid-eye-movement sleep suppressed them.
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PMID:The effect of nocturnal physiological sleep on various movement disorders. 229 64

The blink reflex and its recovery cycle were examined in 57 patients with idiopathic dystonia affecting different parts of the body. The group comprised 9 patients with generalized and 15 with segmental forms, 19 with torticollis, and 14 with focal arm dystonia. None had blepharospasm. The duration and amplitude of the R2 component of the blink reflex showed only minor changes. However, its recovery cycle to paired supraorbital nerve stimuli was abnormal in all groups of patients, except those with focal arm dystonia. These findings may be interpreted as showing abnormal control of the interneuronal networks mediating the blink reflex in patients with dystonia affecting sites other than the facial muscles. The fact that the principal changes were seen in patients with torticollis, and generalized or segmental dystonia, suggests that the extent of dystonia (rather than the severity) and, therefore, the close proximity to the cranial muscles was important in determining the extent of the abnormal interneuron function.
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PMID:The blink reflex in patients with idiopathic torsion dystonia. 232 35

We studied the H-reflex recovery curve and reciprocal inhibition of the H-reflex bilaterally in the upper limb of 5 patients with generalized dystonia, 5 patients with blepharospasm, 10 patients with spasmodic torticollis, and 14 patients with writer's cramp. We compared the results with those obtained from a group of healthy volunteers. The recovery curve of the H-reflex was normal in patients with writer's cramp or blepharospasm, but showed an increase of the physiologic recovery at a 200 msec delay in patients with spasmodic torticollis or generalized dystonia. Reciprocal inhibition of the H-reflex showed a decrease in the amount of inhibition in all the patient groups and a facilitation of the H-reflex during the 3rd period of inhibition in the patients with spasmodic torticollis or generalized dystonia.
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PMID:H-reflex recovery curve and reciprocal inhibition of H-reflex in different kinds of dystonia. 233 Jan 11

Botulinum-A toxin (botAtox) was used in the treatment of blepharospasm (BS), idiopathic hemifacial spasm (HFS), idiopathic spasmodic torticollis (ST) and apraxia of eyelid opening (AEO). The injection of 7.5-30 U botAtox per eye spread over 3 or 4 sites in the palpebral part of orbicularis palpebrae (OP) reduced palpebral spasm in 12/13 cases of BS and in 7/8 cases of HFS. The effect lasted for 14.5 weeks on average (range 4-30 weeks). Palpebral ptosis (lasting 1-3 weeks) was the most frequent side effect (16/107 eyes treated) but was not related to dose of botAtox or number of inoculation sites. Injection of 60-160 U botAtox into the sternocleidomastoid, trapezius and splenius capitis muscles reduced ST objectively in 1/4 patients for about 4 weeks. In the other patients the reduction or abolition of the hypertrophy of the previous hyperactive muscles was accompanied by persistence or rearrangement of the dystonia pattern, suggesting a change in the pattern of activity of the neck muscles after botAtox. 5 U botAtox per eye spread over 4 sites in the OP significantly reduced the frequency of the episodes of involuntary eyelid closure in 2 patients with AEO but not BS. The therapeutic effect lasted for 7 months after the first treatment and for 8 months after the second in a 46 year old woman with a 6 month history while the second patient (72 year old parkinsonian) has now completed her 3rd month of treatment.
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PMID:Botulinum A toxin treatment for eyelid spasm, spasmodic torticollis and apraxia of eyelid opening. 238 98

We report the clinical and laboratory findings in 2 siblings with a syndrome of pigmentary retinopathy, blepharospasm, and dystonia. This entity most resembles Hallervorden-Spatz disease, but appears to be a distinct disorder without identifiable neuroimaging or biochemical abnormalities.
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PMID:A familial syndrome of dystonia, blepharospasm, and pigmentary retinopathy. 239 18

The natural history and response to different treatments were assessed in 31 consecutive patients with blepharospasm (BS) and/or oromandibular dystonia (OMD). The mean age at onset was 52.4 years and there was a female preponderance of 2.5 to 1. Ocular symptoms preceded the onset of blepharospasm in more than 50% of the affected patients, whereas psychiatric and dental problems prior to the onset of focal dystonia were found in 10% and 13% of the cases respectively. Dystonia elsewhere, mainly in the craniocervical area, was found in 23% of patients and appeared to follow a somatotopic progression. The first 2-3 years of history were crucial for the spread of dystonia to other face and body parts. When OMD was the first symptom, a lower tendency of dystonia to progress elsewhere was observed. A putative cause was found in 14% of patients who showed clinical and radiographic evidence of basal ganglia or rostral brainstem-diencephalon lesions. The response to different drugs was inconsistent although transient improvement was induced by haloperidol in 6 patients, by L-Dopa plus deprenyl in 3 patients, by trihexyphenidyl in 2 patients and by clonazepam in 2 patients. One, apparently spontaneous, remission was observed. Botulinum A toxin was injected in the orbicularis oculi of 8 patients affected by BS: moderate to marked improvement lasting 5 to 30 weeks (mean 14.5 weeks) was achieved in all cases; transient ptosis, lasting 1 to 3 weeks, occurred in 3 cases.
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PMID:Facial dystonia: clinical features, prognosis and pharmacology in 31 patients. 251 66


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