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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Medical treatment of dystonia usually results in an incomplete response and is frequently unsuccessful. Peripheral surgical therapy is available for some focal dystonias, but may only offer temporary relief and may have unacceptable complications. We have used local injections of botulinum toxin into the appropriate muscles for treatment of disabling focal or segmental dystonia in 93 patients with torticollis, blepharospasm, oromandibular dystonia (OMD), limb dystonia, lingual dystonia, and dystonia adductor dysphonia, in addition to four patients with hemifacial spasm. Significant relief of motor symptoms was seen in 69% of the patients with blepharospasm and 64% of patients with torticollis; 74% of the latter group with pain experience relief. Relief of symptoms was noted in most patients with OMD and limb dystonia, and all with lingual dystonia, dystonic adductor spastic dysphonia, and those with hemifacial spasm. Benefit averaged 2 1/2-3 months initially; however some patients experienced longer relief with subsequent injections. Adverse effects were transient, although 2 patients developed antibodies against the toxin, and we documented evidence for distant effects in others. This approach of chemically weakening contracting muscles in focal dystonia offers many advantages over pharmacotherapy and surgical therapy. Additional experience is needed to explore the proper doses, and potential for long term adverse effects.
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PMID:Localized injections of botulinum toxin for the treatment of focal dystonia and hemifacial spasm. 350 53

We studied the effects of botulinum A toxin in 12 patients with blepharospasm and 10 patients with oromandibular-cervical dystonia received in a double-blind manner. All blepharospasm patients improved, 71.6% on a clinical rating score, 60.7% by self-assessment, and 38.9% by video-rating; there was no improvement with placebo. The beneficial effects lasted a mean of 12.5 weeks (range, 5 to 28). Only 37.5% of the patients with oromandibular-cervical dystonia improved. Patients with pharyngeal dystonia and spasmodic dysphonia also improved.
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PMID:Botulinum A toxin for cranial-cervical dystonia: a double-blind, placebo-controlled study. 356 73

We studied five patients with a combination of Meige's syndrome (blepharospasm-oromandibular dystonia) and myasthenia gravis. The coexistence of two disorders impairing eyelid opening led to diagnostic confusion and delayed appropriate therapy. Detailed oculographic monitoring of one patient indicated that eye position drifting due to myasthenic oculomotor fatigue was corrected by eye blinks, and that blinks tended to occur with slower saccades. Our observations suggest that fatigue of extraocular muscles may lead to synkinetic blinking and perhaps eventually to autonomous blepharospasm.
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PMID:Coexistent Meige's syndrome and myasthenia gravis. A relationship between blinking and extraocular muscle fatigue? 363 79

The effectiveness of Botulinum toxin (Oculinum) therapy in 76 patients with the diagnosis of essential blepharospasm was analyzed. Botulinum offers relief to almost all patients suffering from essential blepharospasm, however, this relief is usually temporary. The response time for repeated treatments tended to be longer than the first treatment. Patients with mild blepharospasm responded significantly longer to Botulinum injection, than those with severe spasms. The response to Botulinum was not significantly different in patients with Meige syndrome than in patients with only essential blepharospasm. Patients previously treated surgically for essential blepharospasm did not respond differently than those patients with no previous surgical therapy. The authors believe that Botulinum toxin injection is an effective, although temporary, mode of therapy for the signs and symptoms of this focal dystonia. The authors recognize that there may be psychologic factors affecting the response.
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PMID:Effectiveness of botulinum toxin therapy for essential blepharospasm. 365 74

One hundred and one patients with idiopathic blepharospasm have been treated with injections of botulinum toxin A into the orbicularis oculi. Ten had previously had facial nerve avulsions and responded well, normal visual function being restored in the majority (7/10) for an average of 14 weeks. Without prior surgical treatment the response was more variable, but 71/91 regained normal or near normal vision. Older patients, those with a family history of the condition, and those without oromandibular dystonia responded slightly better. The severity of the blepharospasm, the length of the history, and spontaneous resolution of an episode of focal dystonia in the past had no influence on the outcome. Results were poor in the presence of an associated neurological disorder. Side effects, particularly a temporary partial ptosis, were common but were well tolerated. The average duration of improvement was eight weeks in men, nine in women, and there was no evidence of any increase in duration after multiple injections. Eighty nine patients continued with injections, 11 opted for surgical treatment, and one resumed drugs.
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PMID:Long-term results of treatment of idiopathic blepharospasm with botulinum toxin injections. 366 59

A 76-year-old man is reported with advanced progressive supranuclear palsy (PSP) who developed a persistent, gradually progressive torticollis over a period of several months. Blepharospasm and dysfluency of the extrapyramidal type antedated the torticollis. This first report of torticollis in PSP reinforces previous notions that torticollis is related to pathologic changes in the striatum and brainstem. In addition, the combination of torticollis and blepharospasm in our patient supports the previous concept that these two "focal dystonias" have a common pathophysiologic mechanism. This also suggests that dysfluency in PSP may be an expression of a focal dystonia involving the muscles of articulation.
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PMID:Progressive supranuclear palsy: report of a case with torticollis, blepharospasm, and dysfluency. 379 10

Botulinum toxin type A creates temporary localised flaccid paralysis after injection into skeletal muscle. Thirty four patients with blepharospasm, of whom 28 also had the oromandibular dystonia syndrome, were treated with injections of botulinum toxin type A into the orbicularis oculi, and 28 showed functional improvement after the treatment. A high incidence of local side effects occurred, especially partial ptosis, which was well tolerated. There were no systemic side effects. The average period of relief was 2.5 months, increasing to 2.8 months after a second injection. Functional improvement was limited in patients with severe associated dystonia.
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PMID:Effect of treatment with botulinum toxin on neurogenic blepharospasm. 392 84

We studied a 68-year-old man who died after 13 years of progressive dementia, rigidity, bradykinesia, mild tremor, stooped posture, slow and shuffling gait, dystonia, blepharospasm, apraxia of eyelid opening, anarthria, aphonia, and incontinence. At autopsy, he had generalized brain atrophy with large deposits of iron pigment in the globus pallidus, caudate, and substantia nigra. Axonal spheroids were found in the globus pallidus, substantia nigra, medulla, and spinal cord. The neurochemical analysis of the brain revealed marked loss of dopamine in the nigral-striatal areas, with relative preservation of dopamine in the limbic areas. This is the oldest case of familial Hallervorden-Spatz disease reported and the first with neurochemical analysis of the brain.
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PMID:Late-onset Hallervorden-Spatz disease presenting as familial parkinsonism. 396 11

The pathophysiology of reflexes mediated by the fifth and seventh cranial nerves has been studied in 16 patients with blepharospasm and oromandibular dystonia compared with normal age-matched subjects. The EMG activity of the dystonic spasms in the periocular and jaw muscles was similar to that described in other muscles in patients with generalized torsion dystonia. The latency of the R1 and R2 components of the blink reflex and of the corneal reflex was normal. However, the amplitude and the duration of the R1 and R2 and the duration of the corneal reflex were increased. In some patients the R1 component was also present on the side contralateral to the stimulus, while in normal subjects it was present only on the ipsilateral side. The excitability cycle of recovery of the R2 component of the blink reflex after a prior conditioning shock was enhanced in the patients. There were no EEG potentials preceding blepharospasms in the patients, although a Bereitschaftspotential was seen beginning some 500 ms prior to voluntary blinks in the same individuals. Exteroceptive suppression in the contracting masseter and orbicularis oculi muscles was absent in 40 to 50 per cent of the patients. The jaw jerk was present in all the patients with normal latency. These results indicate that the neuronal arcs of the facial reflexes in blepharospasm and oromandibular dystonia are normal. However, there is an abnormal excitatory drive, perhaps from the basal ganglia, to the facial motoneurons and the interneurons which mediate the facial reflexes in the brainstem.
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PMID:Pathophysiology of blepharospasm and oromandibular dystonia. 404 76

Severe, involuntary, forceful closure of both eyelids, along with dystonia and rigidity, followed hypoxic encephalopathy in a young man whose computed tomographic scan showed symmetric infarcts of the corpus striatum. Symptomatic blepharospasm can result from bilateral damage to the basal ganglia.
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PMID:Blepharospasm with bilateral basal ganglia infarction. 406 20


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