Gene/Protein Disease Symptom Drug Enzyme Compound
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34 patients with focal dystonias (13 with essential blepharospasm, 3 with Meige's syndrome, 2 with hemifacial spasm, 16 with spasmodic torticollis) were treated with botulinum type A toxin. 4 ng of botulinum type A toxin per eye were applied in the M. orbicularis oculi as first injection in the 18 patients without spasmodic torticollis. The 16 patients with idiopathic spasmodic torticollis received 10 ng botulinum toxin A in the contralateral M. sternocleidomastoideus as well as in the ipsilateral M. splenius capitis as first injection. The effect was monitored for a time period of at least 6 weeks by two subjective rating scores, a visual functional score and a global clinical impression score. Patients with blepharospasm showed a distinct improvement already after 4 days which lasted for 6 weeks. 75% of the patients with spasmodic torticollis experienced a moderate to distinct improvement after 4 days which remained stable for 6 weeks. A second injection was performed in 15 (7 blepharospasm, 8 spasmodic torticollis) patients 9-11 weeks later with a similar success. All observed side effects (weakness; stiffness of local muscles; feeling of dryness of eyes, unilateral ptosis) were mild and of transient nature. We suggest therefore botulinum type A toxin as treatment of first choice in focal dystonias.
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PMID:[Botulinum toxin A in therapy of craniocervical dystonias and hemifacial spasm]. 179 25

Injections of botulinum toxin into the oculomotor muscles was used in incorrectible diplopia, ocular torticollis, Duane's syndrome and congenital nystagmus. Favourable results were obtained and the sole complications which could be observed were a transitory ptosis and subconjunctival haemorrhages. Frequently 2 to 3 injections were sufficient for a permanent effect.
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PMID:[Injection of botulinum toxin into the oculomotor muscles in disorders of ocular motility]. 181 62

Botulinum A toxin was injected into the affected muscles in 20 patients with blepharospasm, 8 with torticollis and 12 with hemifacial spasm. In all cases blepharospasm and hemifacial spasm was abolished or markedly reduced. The only side effect was transient ptosis and diplopia. Patients with torticollis had a mild to moderate improvement of the dystonic posture and pain; dysphagia was the most troublesome side effect. Botulinum A toxin is an effective therapy in patients with focal dystonia and spasms.
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PMID:Botulinum A toxin injection in patients with blepharospasm, torticollis and hemifacial spasm. 208 84

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

An 8-year-old boy presented with an ocular torticollis that had appeared many months after a ptosis repair. The three-step test was positive for a superior oblique palsy. However, at the time of surgery the forced duction test showed a marked restriction in depression of the eye. These findings were duplicated before the second procedure normalized the ocular movements. The surgical microscope was of great help for the extensive dissection necessary to correct the condition. The fourth step, the forced duction test, was essential for accurate differential diagnosis between true superior oblique palsy and a mechanical hypertropia.
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PMID:A four-step test for diagnosis of pseudo superior oblique palsy. 316 82

A 5-year-old boy with deletion of the long arm of chromosome 11 presented with trigonocephaly, bilateral ptosis, epicanthus, antimongoloid lid axes, and bilateral iris coloboma. In order to avoid complication by a torticollis a Guyton-Friedenwald fadenoperation was performed. In 1981 Grosse et al. listed among the pediatric symptoms retarded development, ventricular septum defect, mitral stenosis, and skeletal and urogenital anomalies.
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PMID:[Ophthalmologic findings in 11 q-deletion syndrome]. 362 12

A prospective open study of botulinum toxin A treatment for patients with various movement disorders at Siriraj Hospital, Mahidol University was analysed to evaluate its efficacy. The grand total of 900 patients comprised of a) 592 patients (65.78 per cent) with hemifacial spasm; b) 92 patients (10.22 per cent) with occupational cramp; c) 79 patients (8.78 per cent) with blepharospasm and Meige syndrome; d) 72 patients (8.00 per cent) with spasmodic torticollis; e) 19 patients (2.11 per cent) with hemidystonia and generalised dystonia; f) 11 patients (1.22 per cent) with spasmodic dysphonia; g) 10 patients (1.11 per cent) with spastic hemiparesis; and h) 25 patients (2.78 per cent) with miscellaneous group (i.e. tics, Gilles de la Tourette, facial myokimia, benign fasciculation, etc.). The results of treatment for hemifacial spasm were classified as excellent in 486 patients (82.09 per cent), moderate improvement in 60 patients (10.14 per cent), mild improvement in 39 patients (6.59 per cent) and no improvement or worse in 7 patients (1.18 per cent). There were complications of mild transient facial weakness in 50 patients (8.45 per cent) and mild ptosis in 12 patients (2.02 per cent). The effect of botulinum toxin treatment lasted 3-6 months. In occupational cramp and spasmodic torticollis the good response rate was around two-thirds of all patients, whereas, blephalospasm, spasmodic dysphonia, spastic hemiparesis and tics responsed in 79-88 per cent of the patients. Botulinum toxin A injection is thus a simple, safe, and effective out-patient treatment for patients with various kinds of movement disorders but it is a costly therapy.
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PMID:Treatment of various movement disorders with botulinum A toxin injection: an experience of 900 patients. 756 52

Botulinum toxin therapy is safe and effective in the treatment of different movement disorders, especially focal dystonias. We reviewed botulinum toxin treatment of 97 patients: 36 had blepharospasm, 41 had torticollis, and 20 had diverse movement disorders. Patients with blepharospasm and torticollis improved markedly after botulinum toxin injections. The most common side effect in BS patients was ptosis (44.4%); in TC patients, it was dysphagia (29.3%). The mean duration of the improvement in both groups was 3.4 months. Very promising results were obtained also in the heterogeneous group including patients with other focal dystonias and cerebral palsy. On the basis of these results, we concluded that BTA injections must now be considered the mainstay of therapy for focal dystonias and other involuntary movement disorders.
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PMID:Botulinum toxin in the treatment of neurological disorders. 815 63

Fifty-five patients were treated with botulin injections into the muscles showing dystonia, contracture or tremor. Twenty two of them had torticollis, 21 had blepharospasm, 10 had hemifacial spasm, and 2 had tremor. In all, 112 injections were done with good result in 64%, slight effect in 27% and without effect in 9% of the cases. Similar results have been reported from other centers in the world. Adverse effects were not significant and disappeared after several days or weeks. They included ptosis, speech and deglutition disturbances, general weakness and neurotic reactions. These adverse effects developed in 12 cases. In cases of tremor the dose as well as the technique of injections must be individualized. The method is an important therapeutic advance and can be applied in outpatient clinics.
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PMID:[Own experience with botulinum treatment of dystonia]. 854 26

Botulinum toxin has become the initial treatment of choice for the management of essential blepharospasm, hemifacial spasm and other craniocervical dystonias. Numerous studies have confirmed a 90% to 95% response rate. Although a number of common side effects have been reported, the occurrence and incidence of rare local complications remains poorly understood. More importantly, the acute and chronic distant effects of botulinum toxin have not been clearly elucidated. A better understanding of such effects is essential if clinicians are to appropriately advise patients on the use of this therapeutic modality. This article is based on the Duke University experience in the management of over 500 patients with craniocervical spasm disorders, combined with a review of the published literature. These disorders include essential blepharospasm, oromandibular dystonia, hemifacial spasm, and torticollis. The incidence of side effects following more than 6000 treatments with botulinum toxin is presented. Pertinent research relating to the causes of these complications is also reviewed. The most common complications of treatment with botulinum toxin are related to acute local effects resulting from chemodenervation. The most important clinical effect in this group is weakening of the levator muscle resulting in ptosis, and the corneal consequences of lagophthalmos. The latter includes exposure keratitis, dry eyes, blurred vision, and hypersecretion epiphora. Less common local effects include facial numbness, diplopia, and ectropion. Some distant effects are being observed with increasing frequency. These include pruritus, dysphagia, nausea, and a flu-like syndrome. Most significant, however, are the rare reports of generalized weakness and the documentation of EMG abnormalities distant to the site of toxin injection. This has been seen with injections for both blepharospasm and torticollis. Until further studies on the long-term distant complications of botulinum toxin are available, it is recommended that patients receive as few life-time doses of toxin as possible, consistent with adequate management of their spasms. The practice of reinjecting patients routinely every three months, or at the first return of mild spasms should be discouraged.
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PMID:Botulinum-A toxin in the treatment of craniocervical muscle spasms: short- and long-term, local and systemic effects. 882 30


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